Download the full issue PDF - AORTA Journal

Publish Date:
July 2013
Volume 1, Issue 2
A multi-disciplinary journal for clinicians and researchers
with interest in the Aorta and its first-order branches,
intended for cardiac surgeons, cardiologists, vascular
surgeons, interventional radiologists, geneticists, molecular
biologists, engineers, and industry scientists, among others.
Editor-in-Chief:
John A. Elefteriades, MD
Co-Editor-in-Chief:
Michael Jacobs, MD
Editors:
Kim Eagle, MD
Bart Muhs, MD
Sandip Mukherjee, MD
Santi Trimarchi, MD
Associate Editors:
Emily A. Farkas, MD
Bulat A. Ziganshin, MD
Official Journal of the Aortic Institute at Yale-New Haven Hospital
Accepting papers at: http://aorta.scienceinternational.org.
ISSN 2325-4637
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Indications
The Endurant® II Stent Graft System is indicated for the endovascular treatment
of infrarenal abdominal aortic or aorto-iliac aneurysms in patients with the
following characteristics:
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of 19 to 32 mm
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Contraindications
The Endurant II Stent Graft System is contraindicated in:
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Warnings and Precautions
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has not been established. All patients should be advised that endovascular
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the performance of the implanted endovascular stent graft. Patients with
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in the structure or position of the endovascular graft) should receive
enhanced follow-up. Specific follow-up guidelines are described in the
Instructions for Use.
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secondary interventions or surgical procedures.
t 5IF&OEVSBOU**4UFOU(SBGU4ZTUFNJTOPUSFDPNNFOEFEJOQBUJFOUTVOBCMF
to undergo or who will not be compliant with the necessary preoperative
and postoperative imaging and implantation studies as described in the
Instructions for Use.
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'SPNBOFYDFTTVTFPGDPOUSBTUBHFOUT
"TBSFTVMUPGFNCPMJPSBNJTQMBDFETUFOUHSBGU5IFSBEJPQBRVFNBSLFS
along the edge of the stent graft should be aligned immediately below the
lower-most renal arterial origin.
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increased duration of the procedure.
t 5 IFTBGFUZBOEFõFDUJWFOFTTPGUIF&OEVSBOU**4UFOU(SBGU4ZTUFNIBTOPU
been evaluated in some patient populations. Please refer to the product
Instructions for Use for details.
Adverse Events
Potential adverse events include (arranged in alphabetical order): Amputation;
"OFTUIFUJDDPNQMJDBUJPOTBOETVCTFRVFOUBUUFOEBOUQSPCMFNTFHBTQJSBUJPO
Aneurysm enlargement; Aneurysm rupture and death; Aortic damage,
including perforation, dissection, bleeding, rupture and death; Arterial
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Bleeding, hematoma or coagulopathy; Bowel complications (e.g., ileus,
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$BSEJBDDPNQMJDBUJPOTBOETVCTFRVFOU
attendant problems (e.g. arrhythmia, myocardial infarction, congestive heart
failure, hypotension, hypertension);
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%FBUI&EFNB&NCPMJ[BUJPONJDSP
BOENBDSP
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'FWFSBOEMPDBMJ[FEJOnBNNBUJPO(FOJUPVSJOBSZDPNQMJDBUJPOTBOE
TVCTFRVFOUBUUFOEBOUQSPCMFNTFHJTDIFNJBFSPTJPOmTUVMBJODPOUJOFODF
hematuria, infection); Hepatic failure; Impotence; Infection of the aneurysm,
device access site, including abscess formation, transient fever and pain;
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mTUVMB
/FVSPMPHJDMPDBMPSTZTUFNJDDPNQMJDBUJPOTBOETVCTFRVFOUBUUFOEBOU
QSPCMFNTFHDPOGVTJPOTUSPLFUSBOTJFOUJTDIFNJDBUUBDLQBSBQMFHJB
paraparesis, paralysis); Occlusion of device or native vessel; Pulmonary
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BOETVCTFRVFOUBUUFOEBOUQSPCMFNTFHBSUFSZPDDMVTJPODPOUSBTUUPYJDJUZ
insufficiency, failure); Stent graft: improper component placement; incomplete
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JOGFDUJPOTUFOUGSBDUVSFHSBGUUXJTUJOHBOEPSLJOLJOHJOTFSUJPOBOESFNPWBM
difficulties; graft material wear; dilatation; erosion; puncture and perigraft
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including infection, pain, hematoma, pseudoaneurysm, arteriovenous
fistula, dissection; Vascular spasm or vascular trauma (e.g., iliofemoral vessel
dissection, bleeding, rupture, death); Vessel damage; Wound complications and
TVCTFRVFOUBUUFOEBOUQSPCMFNTFHEFIJTDFODFJOGFDUJPOIFNBUPNBTFSPNB
cellulitis)
Please reference product Instructions for Use for more information regarding
indications, warnings, precautions, contraindications and adverse events.
CAUTION: Federal (USA) law restricts this device to sale by or on the order
of a physician.
MRI Safety and Compatibility
Non-clinical testing has demonstrated that the Endurant II Stent Graft is MR
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Editorial Board
Editor-in-Chief
John A. Elefteriades Yale University
Editor Emeritus
Randall B. Griepp
(New Haven, CT)
Co-Editor-in-Chief
Michael Jacobs
Editors
Kim Eagle
Bart Muhs
Santi Trimarchi
Sandip Mukherjee
Editorial Board
Jean Bachet
Steven Bailey
Paul Barash
Roberto Di
Bartolomeo
Joseph Bavaria
Jean-Pierre
Becquemin
Harisios Boudoulas
Alan C. Braverman
Duke Cameron
John Chang
Roberto Chiesa
Michael Coady
Denton A. Cooley
Joseph Coselli
Michael Dake
Maastricht University
Hospital (Maastricht,
Netherlands)
Associate Editors
Emily A. Farkas
Bulat A. Ziganshin
Mount Sinai Medical
Center (New York, NY)
Saint Louis University
(St. Louis, MO)
Yale University
(New Haven, CT)
University of Michigan
(Ann Arbor, MI)
Yale University
(New Haven, CT)
Polilinico San Donato
(Milan, Italy)
Yale University
(New Haven, CT)
Zayed Military Hospital
(Abu Dhabi, United Arab
Emirates)
University of Texas
Health Sciences Center
(San Antonio, TX)
Yale University (New
Haven, CT)
University of Bologna
(Bologna, Italy)
University of
Pennsylvania
(Philadelphia, PA)
Henri Mondor Hospital
(Creteil, France)
Aristolelian University
(Columbus, OH)
Washington University
School of Medicine
(St. Louis, MO)
John Hopkins Hospital
(Baltimore, MD)
Long Island Vascular
Center (Roslyn, NY)
University di Bologna
(Bologna, Italy)
Stamford Hospital
(Stamford, CT)
Texas Heart Institute
(Houston, TX)
Texas Heart
Institute/Baylor College of
Medicine (Houston, TX)
Stanford University
(Stanford, CA)
George Dallas
Tirone E. David
Dimitrios Dougenis
L. (Hank) Edmunds
Anthony Estrera
Rosella Fattori
Anthony Furnary
Valentin Fuster
Leonard Girardi
Gary Grunkemeier
Richard Gusberg
Ala Sami Haddadin
Jay Humphrey
Olga A. Iakoubova
John S. Ikonomidis
Jeffrey Indes
Archimedes Analytical/
Associate Yale Medical
(Hickory, NC)
Toronto General Hospital
(Toronto, ON)
Patras University School
of Medicine (Rio, Greece)
University of Pennsylvania
(Philadelphia, PA)
University of TexasHouston Medical School
(Houston, TX)
S. Orsola University
Hospital (Bologna, Italy)
Starr-Wood Cardiac Group
(Portland, OR)
Mount Sinai Medical
Center (New York, NY)
New York Weill Cornell
Medical Center (New
York, NY)
Providence Health System
(Portland, OR)
Yale New Haven Hospital
(New Haven, CT)
Yale University (New
Haven, CT)
Yale University (New
Haven, CT)
Celera (Alameda, CA)
Medical University of
South Carolina
(Charleston, SC)
Yale University
(New Haven, CT)
Eric Isselbacher
Ion Jovin
Matthias Karck
Nicholas Kouchoukos
George Koullias
Johannes Lammer
Frank A. Lederle
Scott LeMaire
George Letsou
Bart Loeys
Wei-Guo Ma
Jorge Mascaro
George Matalanis
Dianna Milewicz
Raj K. Modak
Hamid Mojibian
Frans Moll
Christoph Nienaber
Dimitris Nikas
Takao Ohki
John Pepper
Massachusetts General
Hospital (Boston, MA)
McGuire VA Medical
Center (Richmond, VA)
University of Heidelberg
(Heidelberg, Germany)
Missouri Baptist Medical
Center (St. Louis, MO)
Stony Brook University
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Medical University
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VA Medical Center
(Minneapolis, MN)
Baylor College of
Medicine (Houston, TX)
University of
Texas-Houston Medical
School (Houston, TX)
Ghent University Hospital
(Ghent, Belgium)
Anzhen Cardiovascular
Surgery (Beijing, China)
Queen Elizabeth Medical
Centre (Birmingham, UK)
Austin Hospital
(Heidelberg, Australia)
University of Texas Medical
School (Houston, TX)
Yale New Haven Hospital
(New Haven, CT)
Yale University School of
Medicine (New Haven,
CT)
University Medical Center
Utrecht (Utrecht,
Netherlands)
University Hospital
Rostock (Rostock,
Germany)
Athens Medical Center
(Athens, Greece)
Jikei University School of
Medicine (Tokyo, Japan)
Imperial College
John A. Rizzo
Flavio Rocha
Natzi Sakalihasan
Hans-Joachim
Schaefers
Marc Schepens
Oz Shapira
Bauer Sumpio
Li-Zhong Sun
Wei Sun
Lars Svensson
Robert Thompson
Britt H. Tonnessen
Ramesh K. Tripathi
Marko Turina
Yuichi Ueda
Paul Urbanski
Hence Verhagen
Stephen Westaby
Christopher White
Simona Zannetti
(London, UK)
Stony Brook University
(Stony Brook, NY)
Virginia Mason Medical
Center (Seattle, WA)
University of Liege
(Liege, Belgium)
University of Saarlandes
(Homburg, Germany)
AZ St. Jan
(Brugge, Belgium)
Hebrew University
(Jerusalem, Israel)
Yale New Haven Hospital
(New Haven, CT)
Capital Medical University
(Beijing, China)
University of Connecticut
(Storrs, CT)
Cleveland Clinic
(Cleveland, OH)
Washington University
School of Medicine
(St. Louis, MO)
Roper Heart and Vascular
Center (Charleston, SC)
Narayana Institute of
Vascular Sciences
(Bangalore, India)
University Hospital
(Zurich, Switzerland)
Tenri Hospital
(Nari, Japan)
Herz and Gefaess Clinic
(Neustadt, Germany)
Erasmus University
Medical Center
(Rotterdam, Netherlands)
The John Radcliffe
Hospital (Oxford, UK)
Ochsner Medical Center
(New Orleans, LA)
Medtronic Cardio Vascular
(Santa Rosa, CA)
Volume 1, Number 2, July 2013
HISTORICAL PERSPECTIVE
89 From Ebers to EVARs: A Historical Perspective on Aortic Surgery
Joseph L. Bobadilla
ORIGINAL RESEARCH ARTICLES
96 Painless Type B Aortic Dissection: Insights From the International Registry of Acute Aortic
Dissection
Jip L. Tolenaar, Stuart J. Hutchison, Dan Montgomery, Patrick O’Gara, Rosella Fattori, Reed E. Pyeritz,
Linda Pape, Toru Suzuki, Arturo Evangelista, Frans L. Moll, Vincenzo Rampoldi, Eric M. Isselbacher,
Cristoph A. Nienaber, Kim A. Eagle, Santi Trimarchi
CME
102 Outcomes of Aortic Arch Replacement Performed Without Circulatory Arrest or Deep
Hypothermia
Nisal K. Perera, William Y. Shi, Rhiannon S. Koirala, Sean D. Galvin, Peter R. McCall, George Matalanis
110 Urgent Carotid Endarterectomy in Patients with Acute Neurological Symptoms: The
Results of a Single Center Prospective Nonrandomized Study
Samuel Bruls, Philippe Desfontaines, Jean-Olivier Defraigne, Natzi Sakalihasan
STATE-OF-THE-ART REVIEWS
117 Acute Traumatic Thoracic Aortic Injury: Considerations and Reflections on the
Endovascular Aneurysm Repair
Luca Di Marco, Davide Pacini, Roberto Di Bartolomeo
CASE REPORTS
123 Dissection of Iliac Artery in a Patient With Autosomal Dominant Polycystic Kidney Disease:
A Case Report
CME
Audrey Courtois, Betty V. Nusgens, Philippe Delvenne, Michel Meurisse, Jean-Olivier Defraigne,
Alain C. Colige, Natzi Sakalihasan
AORTA (ISSN 2325-4637) is an online open-access journal issued bi-monthly (6 issues per year, one volume per year) by Science
International Corporation.
All correspondence should be directed to: John A. Elefteriades, MD, Editor-in-Chief, AORTA Journal, 330 Cedar Street, Boardman
Building #204, New Haven, CT 06510. Tel.: ⫹1-203-785-2551, Fax: ⫹1-203-785-3346, E-Mail: [email protected]
All inquiries regarding copyrighted material from this publication should be directed to Science International Corporation: 31 Sunset Court, Stamford, CT, 06903, USA. Tel.: ⫹1-203-329-8842, Fax: ⫹1-203-329-8846, E-Mail: [email protected]
126 Simultaneous Surgical Treatment of Type B Dissection Complicated With Visceral
Malperfusion and Abdominal Aortic Aneurysm: Role of Aortic Fenestration
Gianfranco Filippone, Gabriele Ferro, Cristiana Duranti, Gaetano La Barbera, Francesco Talarico
131 An Unusual Complication of Surgery for Type A Dissection Treated by Thoracic
Endovascular Aortic Repair (TEVAR)
Giuseppe Petrilli, Giovanni Puppini, Salvo Torre, Daniele Calzaferri, Antonella Bugana, Giuseppe Faggian
BASIC SCIENCE
FOR THE
CLINICIAN
135 Genes in Thoracic Aortic Aneurysms and Dissections – Do they Matter?: Translation and
Integration of Research and Modern Genetic Techniques into Daily Clinical Practice
CME
Julie De Backer, Marjolijn Renard, Laurence Campens, Katrien François, Bert Callewaert, Paul Coucke,
Anne De Paepe
IMAGES
IN
AORTIC DISEASE
146 Imaging Assessment of Periaortic Inflammation in Erdheim-Chester Disease
Thierry Couvreur, Györgyi Lipcsei, Alain Nchimi
POLL
THE
EDITORIAL BOARD
149 How Would You Correct an Aberrant Right Subclavian Artery?
Bulat A. Ziganshin
UPCOMING MEETINGS
152 List of Upcoming Meetings
CME
Historical Perspective
Aorta, July 2013, Volume 1, Issue 2: 89 –95
DOI: http://dx.doi.org/10.12945/j.aorta.2013.13-004
Received: January 15, 2013
Accepted: February 13, 2013
Published online: July 2013
From Ebers to EVARs
A Historical Perspective on Aortic Surgery
Joseph L. Bobadilla, MD
Department of Surgery, Vascular & Endovascular Surgery, University of Kentucky, Lexington, Kentucky
Abstract
Pathology of the aorta has been recognized for nearly
three and a half millennia, dating back to the first
recorded description in the scrolls of Ebers, circa 1550
BC. Since that time, treatment has evolved from magical medicinal remedies and incantations to nearly outpatient percutaneous interventions. From the first attempts at open surgical reconstruction in the 1700s and
1800s, to the latest generations of endovascular devices, innovative pioneers have pushed the envelope of
surgical technique in developing unique and novel
strategies to treat the ever complex pathology of the
aorta. We are just now beginning to understand these
pathologies at the molecular and genetic levels, and
with that expansive extent of investigation enters a
journal, dedicated solely to the aorta. With this article,
we hope to illuminate the rich and deep history of
aortic pathology, and the innovations leading to the
technology of today. A firm understanding of our past
provides a strong foundation for further growth into
the future.
Copyright © 2013 Science International Corp.
Introduction
Aneurysma, aneurysmos . . . the etymologic roots of
Latin and Greek origin, meaning widening or dilation,
form the foundation of the modern day word aneurysm. Today, the term aneurysm applied in its most
strict sense describes a blood vessel one and a half
times the diameter of age matched individuals, with
loss of vessel wall parallelism. Vessels of less dilation
are described as ectatic, derived from the Greek origin
Fax ⫹1 203 785 3346
E-Mail: [email protected]
http://aorta.scienceinternational.org
© 2013 Aorta.
Published by Science International Corp.
ISSN 2325-4637
Accessible online at:
http://aorta.scienceinternational.org
ektasis, or to stretch out. Abdominal aortic aneurysms
(AAAs) account for nearly two-thirds of all diagnosed
aneurysms, with thoracic aneurysms (TAAs) accounting for another 20%. Each year, nearly 200,000 new
AAAs are diagnosed in the United States, and another
15,000 reach size criterion for repair. The SAAAVE Act
of 2007 (Screening Abdominal Aortic Aneurysm Very
Efficiently) has brought aneurysms to a more visible
public and political perspective in recent years, but
the knowledge of aortic aneurysms dates back much
farther in history.
The first documented description of aortic pathology appears circa 1550 BC. Over the following three
and a half millennia, our understanding of aortic
aneurysms has progressed from a mystical and uniformly lethal disease process to one that focuses on
preventative intervention and minimally invasive, even
percutaneous, repair (Fig. 1). In this time, we have progressed from palliative options to nearly outpatient surgical interventions. Our understanding of the disease
processes has zoomed down to the cellular, genetic, and
molecular level with such areas of research including
matrix metalloprotenases, cell signaling pathways, and
collagen gene transcript products. On the other end of
the spectrum, academia and industry have pushed the
envelope in the development of new and novel implantable devices to treat a wider base of patients with increasingly complex anatomy via endovascular techniques. An only natural progression is the birth of a new
specialty journal dedicated solely to this, AORTA. A jour-
Corresponding author:
Joseph L. Bobadilla, MD
Department of Surgery
University of Kentucky
800 Rose Street, Room C219
Lexington, KY 40536
Tel: ⫹1 859 323 6346, Fax: ⫹1 859 323 6840, E-Mail: [email protected]
90
Historical Perspective
Figure 1. Advancements in aortic surgery over three and a half millennia. Anatomic, open surgical, and endovascular advancements
are all illustrated. The first rudimentary description of the cardiovascular system and aneurysms dates back to 1550 BC.
Figure 2. Excerpt from the Ebers Papyrus. Thought to have been based on even more ancient texts, the Ebers Papyrus contains
the first documented description of the human heart, aorta, and aneurysms in general. Courtesy of the online catalogue of the
research archives of the Oriental Institute, University of Chicago. Located at: http://oilib.uchicago.edu/books/
bryan_the_papyrus_ebers_1930.pdf. Originally appears in: [2].
Bobadilla, J.L.
From Ebers to EVARs
Historical Perspective
91
Figure 3. Title plate and anatomic plate from Andreas Vesalius’s “De humani corporis fabrica libri septem.” This text revolutionized the understanding of human anatomy and disease. They were the first documented anatomic charts based on direct human
dissection and observation. Courtesy of the National Library of Medicine: Historical* anatomies on the web. Located at:
http://www.nlm.nih.gov/exhibition/historicalanatomies/home.html.
nal dedicated to the multidisciplinary approach to treatment and research in diseases of the aorta and its firstorder branches. A journal dedicated to the future of this
increasing complex field. However, as Alexis de Tocqueville, a French political thinker and historian once stated:
“when the past no longer illuminates the future, the
spirit walks in darkness.” In the following pages, we hope
to illuminate the history of aortic surgery.
Ancient Anatomic History
The term aorta was first applied by Aristotle in the
4th century BC, used to describe the great vessel of
the heart. Prior to this description, the term had been
used by Hippocrates to describe the bronchial tree,
consistent with the belief that the vital “pneuma”
derived from respiration was delivered to the body by
these vessels. The respiratory and circulatory systems
were seen as one continuous circuit until the early
1600s when separate blood circulation was described
by Harvey. Since Aristotle’s time however, the term
aorta has continued to define the primary arterial
outflow of the left ventricle. Pathology of the great
vessel, however, had been recognized for nearly a
Aorta, July 2013
millennium before the times of Hippocrates and Aristotle. The first preserved written account of the aorta
dates back to 1550BC. The Ebers Papyrus (Fig. 2) was
a hieratic script of Pharaonic Egypt, thought to be a
transcription of an even earlier text. It consisted of a
110 page long papyrus scroll containing more than
700 magical and medicinal remedies for ailments of all
organ systems [1,2]. The book of hearts was the largest
and most highly regarded of these scrolls. It described
the heart and great vessels as the center of all being
and existence. Within this script lies the first recorded
mention of aortic aneurysms, quoted as “. . . only magic
can cure tumors of the major arteries.” [2] The scrolls go
on to describe other conditions of the cardiovascular
system, including peripheral arterial aneurysms:
“When thou meetest a tumor of the vessels in any
part of the body of a person and thou findest it round
in form, growing under thy finger . . . . . . Treat it with
the Knife and burn it with Fire so that it bleeds not too
much. Heal it like the Cautery heals.” [2]
This script likely references the development of
traumatic pseudoaneurysms but remains the earliest
Volume 1, Issue 2: 89 –95
92
recorded description of major vascular pathology.
Nearly 1,000 years pass before the next mention of
these vessels again. Galen, a Greek physician practicing in Rome, served as the physician to the gladiators.
Galen holds the reputation as the first trauma surgeon, caring for those injured in combat. He developed rudimentary anatomic charts which were based
on canine vivisection [3]. While the only anatomic
reference of his time, and for hundreds of years later,
they were somewhat incomplete and inaccurate in
their representation of human anatomy. In his writings, he describes aneurysms on physical examination
as “localized pulsatile swellings.” Furthermore, he goes
on to describe the first documented ruptured aneurysm as when “an aneurysm is wounded, the blood is
spouted out with so much violence that it can scarcely
be arrested.” [4,5] A contemporary to Galen was
Antyllus, another Greek surgeon practicing in Rome.
He is considered the true father of vascular surgery. He
described both true and false aneurysms in his writings and documented the first attempted aneurysm
repair in the year 200AD [6]. The “Antyllus method”
consisted of proximal and distal ligation, central incision of the aneurysm, and evacuation of the thrombotic materials [1]. This remained the standard treatment of aneurysms for over 1,000 years to follow.
A few hundred years later, Aetius of Amida, a 7th
century Byzantine physician and medical writer authored the manuscript De Vasorum Dilatatione, loosely
translated “on the dilation of the vessels.” This was a
detailed manuscript on the development and repair of
abdominal aortic aneurysms utilizing a technique similar
to the Antyllus method [6]. Unfortunately, as many of the
time did, Aetius of Amida believed no wound heals
properly without the formation of pus, and to encourage
this, the aneurysm sac was packed with incense.
Andreas Vesalius lived from 1514 –1564 AD, a Flemish physician, who traveled to Paris to study anatomy,
medicine, and surgery. In 1554, he authored a sevenvolume text of anatomic plates, De humani corporis
fabrica (On the Structure of the Human Body, Fig. 3).
These were the first human anatomic charts of their
time, based on actual human anatomic dissections
[4]. He taught his students by direct observation of
dissection, and thus, he became known as the founding
father of modern human anatomy. His seven-volume
anatomic text provided new, detailed descriptions of the
heart, great vessels, and vascular system. These volumes
provided a level of detail never before seen and helped
Bobadilla, J.L.
Historical Perspective
Original postmortem specimen from Sir Astley Cooper’s aortic ligation. Courtesy of the Gordon Museum of Pathology, King’s College London. Exhibit located online at: http://
www.kcl.ac.uk/gordon/collection/specimens.aspx#APCoopers
LigationAbdominalAorta.
Figure 4.
lay the foundation for future surgical pioneers that
would follow in the centuries to come.
Early Operative History
John Hunter is perhaps best known for his famed
ligation of the popliteal artery; however, his older
brother, William, also studied aneurysms throughout
the vascular system [1]. In 1757, William published the
manuscript “The History of an Aneurysm of the Aorta
with Some Remarks on Aneurysms in General.” He described these aneurysms as dilated and pulsatile vessels. He was also one of the first to describe arteriovenous fistulae, along with the hissing noise heard on
From Ebers to EVARs
Historical Perspective
Figure 5.
93
Wire introduced into aortic aneurysm to promote thrombosis. Originally appears in: [8].
auscultation [6]. One of Hunter’s pupils, Sir Astley
Cooper, went on to further the field of aortic surgery.
He experimented with and developed a retroperitoneal exposure of the aorta in a cadaveric model. In
1817, he was summoned urgently to care for a 38year-old porter with a large external iliac artery aneurysm that had eroded the overlying skin and freely
ruptured [1]. He explored the patient transperitoneally
and ligated the distal aorta with a single heavy silk tie.
The patient survived 48 hours postoperatively. The
post mortem specimen remains on display in the
Gordon Museum of Pathology at King’s College London (Fig. 4) [6]. Around the same period, Jean-Nicolas
Corvisart, personal physician to Emperor Napoleon I,
published his essay on disease of the heart and great
vessels (1806, translated to English by Jacob Gates
1812) [7]. He is now known as the father of cardiology,
with the first detailed description of dilative cardiomyopathy, congestive failure, and other valvular
heart disorders. In addition to these diseases, he
also provided a detailed evolution of aneurysms of
the aorta [7].
In 1865, the first attempts at percutaneous endovascular aneurysm repairs were made (Fig. 5). Moore
and Murchison attempted aneurysm sac thrombosis
Aorta, July 2013
by direct needle cannulation and wire packing. Via
direct aneurysm puncture, 26 yards of wire coils were
introduced into a large thoracic aneurysm [8]. The
patient ultimately expired, but the aneurysm had partially thrombosed. Sepsis and distal embolism were
obvious complication. In 1879, the addition of electricity was included, and the Moore–Corradi Method
was born. This electrothrombosis entailed the coiling
with silver and copper wire, and before complete
packing, the passage of current through this wire to
encourage thrombosis [8,9].
Rudolf Matas (1860 –1957) attempted to use electrothrombosis in 1900 for the treatment of a large
abdominal aneurysm. Prior to this, he had described
the use of endoaneurysmorraphy in the treatment of
peripheral aneurysms (Fig. 6) [10]. He described three
forms of aneurysmorraphy: obliterative, restorative, and
reconstructive [6]. In 1923, he used these techniques to
successfully treat an infrarenal AAA [11]. The patient
survived for 18 months postoperatively, eventually succumbing to pulmonary tuberculosis complications [1].
Rea suggested an alternative form of plication,
utilizing cellophane wrapping around abdominal
aneurysms [12]. This technique gained wide exposure
after Rudolf Nissen used it to wrap an abdominal
Volume 1, Issue 2: 89 –95
94
Historical Perspective
Before and after images of the initial Parodi–Palmaz
endovascular aneurysm repair stent and graft system. Originally appears in: [17].
Figure 6.
Technique for Matas endoaneursymorraphy; the
lumen was preserved by plicating the aneurysm over an inner
silastic tube, which was removed prior to complete endoaneursymorraphy. Originally appears in: [10].
Figure 7.
aneurysm of Albert Einstein in 1949. He survived for
more than 5 years after this intervention but ultimately succumb to a ruptured aneurysm April 18,
1955. Frustrated by the poor results with wraps, coiling, and ligation, Charles Dubost performed the first
successful aneurysm resection and interposition graft
using cadaveric aortic allograft on March 29, 1951
[1,13]. This technique included the complete resection
of the aneurysm sac, an often bloody, morbid, and
dangerous procedure. The next major advancement in
aortic surgery came with the combination of Dubost’s
interposition grafting and Matas’s endoaneursymorraphy. In 1966, Oscar Creech described this combined
procedure that remains the operation of choice nearly
50 years later [14]. This simple synthesis greatly simplified open aortic operations and removed much or
the associated morbidity from complete aneurysm sac
resection.
Contemporary Surgical History
The use of cadaveric allografts was, however, of
limited wide-scale utility. These conduits were of obvious limited supply and were also wrought with the
complications of alloimmunity and late aneurysmal
degeneration. Because of this, the search for a more
stable, long-term, synthetic conduit material was undertaken. Through an intense combined effort of investigation, including surgeons, textile engineers, and
mechanical engineers, the Dacron vascular graft was
born, including a completely new manufacturing process and knitting machine that allowed for the seamless construction of these novel branching vascular
grafts. This new arterial substitute was officially announced to the medical community in 1958 with the
landmark paper authored by our contemporary cardiovascular surgical giants: Michael E. DeBakey and
colleagues [15]. DeBakey and Cooley, however, had
Bobadilla, J.L.
From Ebers to EVARs
Historical Perspective
95
been developing techniques for complex aneurysm
repair and spinal cord protection during thoracic aortic surgery for some years prior, with their first successful resection of a fusiform thoracic aneurysm on
January 5, 1953. They went on to expand their experience, with a series of 245 successful aneurysm repairs
by July 1955. Now, with the addition of a stable and
suitable synthetic conduit, the Dacron interposition
graft became the mainstay of modern vascular surgery, allowing complex operations for thoracic, abdominal, and thoracoabdominal aortic aneurysms. In
1974, Stanley Crawford reported his experience of
thoracoamdominal aneurysm repairs [16]. Initially involving side arm branches to the renovisceral vessels,
and ultimately evolving to incorporate small visceral
patches sewn directly to the graft, these open techniques have only recently been challenged by the
newest generation of endovascular technologies.
While others had dabbled in “endovascular techniques” in aortic surgery for nearly 125 years prior, including attempts at wire coiling and electrothrombosis,
the true breakthrough came with the work of Parodi and
Palmaz. They initially experimented with stainless steel
stents hand sewn to thin-walled Dacron tube grafts (Fig.
7). Their handmade endografts were used in canine
models before initial human trials. On September 6,
1990, the first successful human endovascular aneurysm
repair was performed. They went on to describe their
first five patients in the landmark paper “Transfemoral
intraluminal graft implantation for abdominal aortic aneurysms.” [17] Since then, endovascular devices have
progressed to be more readily available, lower profile,
and more complex in configuration.
Conclusions
From the Ebers Papyrus to endografts, over three
and a half millennia, pathology of the aorta has
plagued mankind and been an area for bold and
progressive innovations. From a disease once treated
with magical medicinal remedies to a pathology that
can now be treated percutaneously, pathology of the
aorta continues to provide challenging opportunities
for innovative and landmark research. It is with this
understanding that the journal AORTA has come to
fruition. A forum dedicated solely to advancing the
knowledge in this specific niche. We hope that this
article has helped to illuminate the deep, rich history
of this field and that this journal will help to elucidate the future of it.
Comment on this Article or Ask a Question
References
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Ebers to EVARs: A Historical Perspecof an aneurysm of the abdominal aorta: reestive on Aortic Surgery. Aorta 2013;1(2):
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treatment of aortic aneurysm. Ann Surg.
Volume 1, Issue 2: 89 –95
Original Research Article
Aorta, July 2013, Volume 1, Issue 2: 96 –101
DOI: http://dx.doi.org/10.12945/j.aorta.2013.13-014
Received: March 6, 2013
Accepted: June 3, 2013
Published online: July 2013
Painless Type B Aortic Dissection
Insights From the International Registry of Acute Aortic
Dissection
Jip L. Tolenaar, MD1, Stuart J. Hutchison, MD2, Dan Montgomery, MS3, Patrick O’Gara, MD4,
Rosella Fattori, MD5, Reed E. Pyeritz, MD, PhD6, Linda Pape, MD7, Toru Suzuki, MD8,
Arturo Evangelista, MD9, Frans L. Moll, MD, PhD10, Vincenzo Rampoldi, MD1,
Eric M. Isselbacher, MD11, Cristoph A. Nienaber, MD, FACC12, Kim A. Eagle, MD, FACC3,
Santi Trimarchi, MD, PhD1*
1
Department of Cardiovascular Surgery, Policlinico San Donato IRCCS, Milan, Italy; 2St. Michael’s Hospital, Toronto, Ontario, Canada;
University of Michigan Health System, Ann Arbor, Michigan; 4Brigham and Women’s Hospital, Boston, Massachusetts; 5S. OrsolaMalpighi Hospital, Bologna, Italy; 6Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania; 7University of
Massachusetts Hospital, Worcester, Massachusetts; 8Department of Cardiology, University of Tokyo, Tokyo, Japan; 9Hospital General
Universitari Vall d’Hebron, Barcelona, Spain; 10University Medical Center Utrecht, Utrecht, The Netherlands; 11Massachusetts General
Hospital, Boston, Massachusetts; 12Thoracic Aortic Center, University of Rostock, Rostock, Germany
3
Abstract
Introduction: The classical presentation of a patient
with Type B acute aortic dissection (TBAAD) is characterized by severe chest, back, or abdominal pain, ripping or tearing in nature. However, some patients present with painless acute aortic dissection, which can lead
to a delay in diagnosis and treatment. We utilized the
International Registry on Acute Aortic Dissections
(IRAD) database to study these patients. Methods: We
analyzed 43 painless TBAAD patients enrolled in the
database between January 1996 and July 2012. The
differences in presentation, diagnostics, management,
and outcome were compared with patients presenting
with painful TBAAD. Results: Among the 1162 TBAAD
patients enrolled in IRAD, 43 patients presented with
painless TBAAD (3.7%). The mean age of patients with
painless TBAAD was significantly higher than normal
TBAAD patients (69.2 versus 63.3 years, P ⴝ 0.020).
The presence of atherosclerosis (46.4% versus 30.1%,
P ⴝ 0.022), diabetes (17.9% versus 7.5%; P ⴝ 0.018),
and other aortic diseases (8.6% versus 2.3%, Pⴝ
0.051), such as prior aortic aneurysm (31% versus
18.8% P ⴝ 0.049) was more common in these pa-
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© 2013 Aorta.
Published by Science International Corp.
ISSN 2325-4637
Accessible online at:
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tients. Median delay time between presentation and
diagnosis was longer in painless patients (median
34.0 versus 19.0 hours; P ⴝ 0.006). Dissection of
iatrogenic origin (19.5% versus 1.3%; P < 0.001) was
significantly more frequent in the painless group. The
in-hospital mortality was 18.6% in the painless
group, compared with an in-hospital mortality of
9.9% in the control group (P ⴝ 0.063). Conclusion:
Painless TBAAD is a relatively rare presentation (3.7%) of
aortic dissection, and is often associated with a history of
atherosclerosis, diabetes, prior aortic disease including
aortic aneurysm, and an iatrogenic origin. We observed a
trend for increased in-hospital mortality in painless
TBAAD patients, which may be the result of a delay in
diagnosis and management. Therefore, physicians should
be aware of this relative rare presentation of
TBAAD.
Copyright © 2013 Science International Corp.
Key Words
Aortic dissection · Painless
*Corresponding author:
Santi Trimarchi, MD, PhD
Department of Cardiovascular Surgery
Policlinico San Donato IRCCS
University of Milano
Thoracic Aorta Research Center
Piazza Malan 2
20097 San Donato Milanese MI, Italy
Tel: ⫹39 02 52774339, Fax: ⫹39 02 52774415, E-Mail: [email protected]
Original Research Article
97
Table 1. Demographics and Patient History
Category
Demographics
Patients (n)
Age (mean⫾SD)
Gender – male
Race – non-white
Hypertension
Diabetes
Marfan syndrome
Atherosclerosis
Known aortic
aneurysm
Prior AAD
Aortic valve disease
Other aortic disease
Family history of
aortic disease
Prior
cath/angiography
Prior CABG
Prior surgery for
aortic aneurysm/
dissection
Intramural
hematoma
Iatrogenic AAD
Hours presentation
to diagnosis
(median)
Table 2. Presenting Symptoms/Signs of Aortic Dissection
Type B
Type B
Not Painless
(%)
Painless
(%)
1119
63.3⫾14.1
773 (66.9)
913 (82.7)
918 (80.2)
85 (7.5)
41 (3.6)
339 (30.1)
43
69.2⫾10.8
25 (58.1)
35 (85.4)
37 (86.0)
7 (17.9)
0 (0.0)
20 (46.5)
0.020
0.234
0.657
0.348
0.018
0.396
0.022
212 (18.8)
90 (8.0)
69 (6.1)
23 (2.3)
13 (31.0)
3 (7.3)
4 (10.3)
3 (8.6)
0.049
1.000
0.302
0.051
48 (11.6)
1 (10.0)
1.000
98 (10.3)
49 (4.4)
6 (21.4)
1 (2.6)
0.061
1.000
145 (13.1)
6 (14.6)
0.775
131 (11.7)
14 (1.3)
6 (14.0)
8 (19.5)
0.654
⬍0.001
p-value
Presenting hypertensive
Presenting hypotensive
Mean systolic blood
pressure
Mean systolic blood
pressure
Presented with pulse
deficits
Shock
Syncope
Cerebrovascular
accident
Ischemic peripheral
neuropathy
Spinal cord ischemia
Limb ischemia
Acute renal failure
Not Painless
(%)
Painless
(%)
p-value
763 (68.8)
80 (7.3)
17 (45.9)
4 (10.0)
0.003
0.532
166.8
147.2
0.003
85.0
0.095
173 (18.6)
11 (1.0)
28 (2.5)
2 (8.3)
1 (2.7)
4 (10.3)
0.286
0.327
0.020
14 (1.3)
2 (5.1)
0.100
31 (2.8)
31 (2.8)
101 (9.3)
161 (14.8)
0 (0)
0 (0)
0 (0)
8 (20.0)
0.622
0.622
0.043
0.364
91.44
possible stage. The aim of the current study was to
assess the clinical characteristics, diagnostics, treatment,
and outcomes of patients with painless TBAAD.
Methods
19.0 (12.7–25.3) 34.0 (22.8–72)
0.006
AAD indicates acute aortic dissection; CABG, coronary artery bypass grafting.
Introduction
The classical presentation of a patient with acute
aortic dissection (AAD) is characterized by severe
chest, back, or abdominal pain. However, previous
reports showed that between 5 and 17% of all dissection patients present with painless acute aortic dissections [1,2]. As expected, atypical presentation can lead
to a delay in diagnosis, which is associated with higher
mortality [3,4]. Painless Type B acute aortic dissection
(TBAAD) does not mean that these patients have uncomplicated dissections, as they still can develop malperfusion and aortic rupture [1,2]. Immediate adequate medical treatment is essential and has to include optimal
blood pressure control in order to reduce shear stress
and limit the propagation of the dissection. Therefore, it
is important to recognize these patients at the earliest
Aorta, July 2013
Category
Patient Selection
The International Registry of Acute Aortic Dissection (IRAD)
is an ongoing multinational registry designed to provide a representative population of patients with acute aortic dissection. The
rationale, design, and methods of IRAD have been previously
published [5]. The diagnosis of TBAAD was based on clinical
symptoms, diagnostic imaging, direct visualization during surgery, and/or postmortem examination. Patients were enrolled at
diagnosis or retrospectively. We analyzed all TBAAD patients enrolled in IRAD from January 1996 to July 2012 and selected those
patients presenting without any pain symptoms. Demographics,
medical history, presenting symptoms, management, and outcomes were compared between patients presenting with and
without pain.
Statistical Analysis
Categorical variables were compared for both groups utilizing the chi-squared tests and Fisher’s exact tests. Student’s
t-test was used to analyze continuous variables and the nonparametric test of medians to analyze non-normally distributed
variables. A p-value ⬍0.05 was considered significant. Kaplan-Meier
survival curves were plotted to estimate survival. Data analysis was
performed with the use of SPSS statistical analysis software (SPSS Inc,
Chicago, Ill).
Volume 1, Issue 2: 96 –101
98
Original Research Article
Table 3. Imaging
Table 4. Management
Type B
Category
CXR done
CXR normal
CXR showed
abnormal
aortic contour
CXR showed
abnormal
cardiac contour
ECG done
ECG normal
TEE done
CT done
CT Normal
Angiography
MRI done
Periaortic
hematoma
identified on
any imaging
study
Most proximal
extension:
Aortic arch
Left subclavian
artery
Descending
Largest diameter of
descending
aorta (median)
Type B
Not Painless
(%)
Painless
(%)
1002 (86.7)
275 (28.1)
37 (86.0)
8 (21.6)
0.905
0.389
397 (43.9)
16 (47.1)
0.713
141 (15.8)
1087 (94.0)
409 (38.2)
574 (50.4)
1110 (96.2)
6 (0.6)
207 (18.2)
185 (16.7)
2 (6.2)
36 (83.7)
10 (28.6)
20 (47.6)
34 (82.9)
1 (3.2)
9 (24.3)
10 (27.8)
0.211
0.006
0.250
0.724
⬍0.001
0.184
0.345
0.081
150 (14.5)
6 (16.7)
0.718
263 (22.8)
7 (16.3)
0.318
577 (49.9)
280 (24.2)
19 (44.2)
13 (30.2)
0.461
0.368
4.0 (3.5–5.0) 3.6 (3.0–4.7)
p-value
0.137
CXR indicates chest X-ray; ECG, electrocardiograph; TEE, transesophageal echocardiography; CT, computed tomography; MRI, magnetic resonance imaging.
Results
Among the 1162 TBAAD patients enrolled in IRAD,
43 patients presented with painless TBAAD (3.7%). The
mean age of patients with painless TBAAD was significantly higher than normal TBAAD patients (69.2 versus 63.3 y; p-value ⫽ 0.020, Tables 1–3). Painless patients presented more often with a history of diabetes,
(17.9% versus 7.5%; P ⫽ 0.018), atherosclerosis (46.4%
versus 30.1%; P ⫽ 0.022), and were more often diagnosed with a known prior aortic aneurysm (31% versus
18.8%; P ⫽ 0.049) Painless patients presented less
frequently with hypertension (45.9% versus 68.8%;
P ⫽ 0.003) and with a lower mean systolic blood
Tolenaar, J.L. et al.
Category
Not Painless Painless
(%)
(%)
p-value
Medical management
Surgical management
Endovascular management
In-hospital mortality
Medical management
Surgery
Endovascular
754 (65.2)
137 (11.9)
251 (21.7)
114 (9.9)
57 (7.8)
25 (18.2)
31 (12.4)
0.988
0.676
0.903
0.063
0.468
0.089
0.320
28 (65.1)
6 (14.0)
9 (20.9)
8 (18.6)
3 (10.7)
3 (50.0)
2 (22.2)
pressure (mean 147.28 mm/Hg versus 166.8.2 mm/Hg;
P ⫽ 0.003). Syncope was more represented in the
painless group (10.3% versus 2.5%; P ⫽ 0.020).
Diagnostics
As might be expected, the mean time interval between admission and diagnosis of aortic dissection
was 34.0 hours among painless patients, as compared
to 19.0 hours in the control group (P ⫽ 0.006). Computed Tomographic Angiography (CTA) was more often
used as the primary diagnostic modality in the painful
group (96.2 versus 82.9%, ⬍ 0.001). Previous angiography was more frequently performed in the painless
group and these patients also had significantly more
iatrogenic dissections (19.5 versus 1.3%; P ⬍ 0.001). The
iatrogenic cause in the painless group was: Percutaneous
transluminal coronary angioplasty (PCTA) in three patients (37.5%), cardiac surgery in three patients (37.5%),
and unknown cause in two patients (25%).
Management and Outcome
Almost two-thirds of the patients were treated
medically, which did not differ between groups. (65.2
versus 65.1%; P ⫽ 0.988; Table 4.) Surgical and endovascular therapies were equally used in approximately
35% of each group. In-hospital mortality was 18.6% in
the painless group, compared with an in-hospital mortality of 9.9% in the control group (P ⫽ 0.063). There
were no statistically significant differences in complications between both groups. Kaplan-Meier survival
curves did not demonstrate a significant difference in
mortality during five-year follow-up (P ⫽ 0.960; Fig. 1).
Discussion
The most common characteristic of TBAAD presentation is acute pain localized to the chest, abdomen,
Painless Type B Aortic Dissection
Original Research Article
Figure 1.
99
Kaplan Meier survival curve.
and back. Previous IRAD reports showed that 95.5% of
all AAD patients presented with pain [5]. However, in
rare instances the presentation of dissection can be
atypical and our study showed that 3.7% of all TBAAD
patients were painless, in contrast with previous experiences which reported an incidence up to 17% in
AAD [1,2]. The lower incidence that we observed could
be explained by the fact that, while this study focused
only on TBAAD, previous studies focused on painless
dissections in general, including a majority of patients
with ascending aorta involvement (Type A acute aortic
dissection), which makes up for more than 75% of the
patient population [1,2]. In addition, IRAD consists of
cardiovascular referral centers, specialized in the treatment of aortic dissection, where patients are referred
for surgical/endovascular treatment, whereas patients
who are thought to be unfit for invasive management
will not be transferred to these centers. Typically,
transferred patients have more complications, resulting in a relative low incidence in the IRAD database.
The true incidence in the population is probably even
higher, as an atypical presentation will likely result in
a higher risk of death prior to the diagnosis.
Aorta, July 2013
The clinical presentation of dissection patients may
be diverse, and sudden collapse or an altered state of
consciousness have been reported to be the presenting symptom in up to 30% of all patients [6]. This
report also included TAAAD patients, which are more
prone to develop complications like syncope or alteration in consciousness [7]. Painless AAD, especially
concerning the ascending aorta, presents more often
with neurological deficits, syncope, and disturbances
in consciousness. These complications influence the
perception of pain, resulting in a relative high prevalence of Type A dissection in this patient category. As
expected, painless Type B dissection patients did not
show this clinical pattern since involvement of the
head and neck vessels did not occur.
Our study showed that TBAAD painless patients are
older at presentation and more often had a history of
atherosclerosis. With increasing age, the incidence of
painless dissections might rise, as previously reported
[1]. In that study, patients who presented at significantly older age, more frequently had a history of
cerebrovascular accidents, and some patients had
only atypical symptoms such as dyspnea, nausea, and
Volume 1, Issue 2: 96 –101
100
abdominal fullness. These three atypical clinical signs
were not recorded within the IRAD registry, so we
can’t make any comparison.
The pathological mechanism of painless TBAAD is not
well understood and multiple explanations for this phenomenon have been proposed. Our study showed that
painless patients present with less hypertension. Due to
low blood pressure, the propagation of the dissection
might develop relatively slow, thereby reducing the wall
stress, which could result in reduction of pain. Alternatively, pain will act as an acute stressor, determining an
increased blood pressure. Furthermore, the perception
of pain can be modulated as the adventitial layer, the
site for aortic innervation, is involved by the dissection
or affected by previous interventions. In addition, it is
thought that other pathologies, like aneurysmatic enlargement, may influence the ability to sense pain.
This possibility is supported by the higher incidence of
other aortic disease and previous aortic aneurysms in
our study population. Most interestingly, significantly
more painless patients had a dissection of iatrogenic
origin. Iatrogenic dissections are thought to occur very
rarely, with Type A dissections reported in 0.04% of
the patients during percutaneous coronary interventions and in 0.12 to 0.16% after cardiac surgery procedures [8–11]. The incidence of TBAAD in these patients is thought to be even lower. During such
procedures, analgesics and sedation may alter the
patient’s perception of pain, increasing the incidence
of painless TBAAD in this subset of patients [12].
The in-hospital mortality was 18.9% in the painless
group, compared with an in-hospital mortality of
10.3% in the control group (P ⫽ 0.096). The explanation for this trend is probably 2-fold. First, the painless
group tended to present at older age, which is a
Original Research Article
condition associated with a higher mortality [13]. Second, the extended delay to diagnosis and treatment,
due to the difficulty in diagnosis, may have resulted in
a higher mortality.
Although this study represents the first report focusing specifically on TBAAD with absence of pain at
presentation, it has some limitations. Previous studies
reported a higher incidence and registry data might
not reflect the true incidence, since the centers are
specialized in aortic dissection and therefore receive
many referred patients. Furthermore, many patients
may have died from a painless dissection before they
were diagnosed and therefore are not registered.
Conclusion
Painless TBAAD is a relatively rare presentation of
aortic dissection and is associated with a history of atherosclerosis, diabetes, iatrogenic origin, and aortic disease like aneurysm. We observed a trend in increased
in-hospital mortality rate among painless TBAAD patients, which may be the result of a delay in diagnosis
and any type of management due to the absence of
classical symptoms. Therefore, physicians should be
aware of this relatively rare presentation of TBAAD.
Conflict of Interest
Possible Conflict of Interest: IRAD is supported by
grants from the University of Michigan Health System,
Varbedian Fund for Aortic Research, Mardigian Foundation, and Gore Medical Inc (Flagstaff, Ariz).
Comment on this Article or Ask a Question
References
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SJ, Montgomery D, O’Gara P, Fattori R,
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International Registry of Acute Aortic Dissection. Aorta 2013;1(2):96–101. DOI: http://
dx.doi.org/10.12945/j.aorta.2013.13-014
Volume 1, Issue 2: 96 –101
Original Research Article
Aorta, July 2013, Volume 1, Issue 2: 102–109
DOI: http://dx.doi.org/10.12945/j.aorta.2013.12.007
Received: November 9, 2012
Accepted: March 8, 2013
Published online: July 2013
Outcomes of Aortic Arch Replacement
Performed Without Circulatory Arrest or Deep
Hypothermia
Nisal K. Perera, MBBS1, William Y. Shi, MBBS1, Rhiannon S. Koirala, MBBS1,
Sean D. Galvin, FRACS1, Peter R. McCall, FRANZCA2, George Matalanis, FRACS1*
1
Department of Cardiac Surgery, Austin Hospital, University of Melbourne, Victoria, Australia; 2Department of Anaesthesia, Austin
Hospital, University of Melbourne, Victoria, Australia
Abstract
Background: Aortic arch replacement using standard techniques, including deep hypothermic circulatory arrest and
selective antegrade cerebral perfusion, is still associated
with significant mortality and cerebral morbidity. We
have previously described the “branch-first” technique
that avoids circulatory arrest or profound hypothermia
with excellent outcomes. We now describe our clinical
experience with a larger cohort of patients as well as
follow-up of our earlier results. We also describe a further
technical simplification to this technique. Methods: From
2005 to 2010, 43 patients underwent a “branch-first continuous perfusion” technique for aortic arch replacement.
In this technique, arterial perfusion is peripheral, usually
by femoral inflow. Disconnection of each arch branch and
anastomosis to a perfused trifurcation graft proceeds sequentially from the innominate to the left subclavian
artery, with uninterrupted perfusion of the heart and
viscera by the peripheral cannula. In the first cohort perfusion to the trifurcation graft was by right axillary cannulation. Since 2009, a modification was introduced such
that perfusion is supplied directly by a sidearm on the
trifurcation graft. This was used in the last 18 patients of
this series. After reconstruction of the debranched arch
and ascending aorta, the common stem of the trifurcation
graft is anastomosed to the arch graft. In this series, there
were 27 males, and mean age was 63 ⴞ 13 years. Fifteen
cases (35%) were performed with urgent/emergent priority. Nineteen patients (44%) were operated for aortic dis-
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E-Mail: [email protected]
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Published by Science International Corp.
ISSN 2325-4637
Accessible online at:
http://aorta.scienceinternational.org
section, and the remainder for aneurysms. Seven patients
(16%) had previously undergone a cardiac surgical procedure. Results: There were two (4.7%) early mortalities
while one patient (2.3%) experienced a permanent stroke.
One patient (2%) required mechanical support while
three (7%) required hemofiltration for renal support. Extubation was achieved within 24 hours in 21 patients
(49%) while 19 (42%) were discharged from the Intensive
Care Unit (ICU) within two days. Eight patients (19%) did
not require any transfusion of red cells or platelets. Mean
follow-up duration was 21 ⴞ 19 months and was 100%
complete. At three years, survival was 95 ⴞ 3.2%. No
patients required subsequent aortic reoperation during
this early follow-up period. Conclusions: This modified
branch-first continuous perfusion technique brings us
closer to the goal of arch surgery without cerebral or
visceral circulatory arrest and the morbidity of deep hypothermia. Our early experience is encouraging although
greater numbers and longer follow-up will reveal the full
potential of this approach. Copyright © 2013 Science International Corp.
Key Words
Aortic arch · Aortic surgery · Cerebral protection
Introduction
Deep hypothermic circulatory arrest with or without selective antegrade cerebral perfusion is currently
*Corresponding author:
George Matalanis, FRACS
Department of Cardiac Surgery
University of Melbourne
PO Box 5555
Heidelberg, Victoria, 3084, Australia
Tel: ⫹ 61 3 9496 5453, Fax: ⫹ 61 3 9459 6220, E-Mail: [email protected]
Original Research Article
widely utilized for cerebral protection during aortic
arch surgery. Nonetheless this technique is still associated with significant risks. Even short periods of
cerebral circulatory arrest have been shown to be
deleterious for higher cognitive function [1]. While
deep hypothermia is frequently used to compensate
for the unpredictable duration of cerebral circulatory
arrest, its associated morbidities such as prolonged
bypass times for cooling/rewarming and coagulopathy are well documented. Selective antegrade cerebral
perfusion while providing nutritive cerebral flow introduces the risk of atheromatous and/or air embolism
from direct manipulation of the arch branches. It also
relies on deep hypothermia alone to provide distal
organ protection.
Since 2005, in an attempt to minimize the risks and
morbidity associated with aortic arch replacement, our
center has adopted a branch-first continuous perfusion technique, in which there are no periods of global
cerebral circulatory arrest or deep hypothermia, with
encouraging early results [2]. More recently, modifications of this technique have been made to enhance
applicability and reduce technical complexity.
We hereby describe our technical modifications and
report our early experience with this branch first continuous perfusion technique for replacement of the
aortic arch in both elective and emergent settings.
Methods
Patients and Methods
Between, 2005 and 2010, 43 patients have undergone this
technique, selectively in the first year and as a routine from
2006 onwards. Of these, 27 were male and 16 were female. The
average age was 64 years (range 29 – 85 years). Preoperative
demographic data are shown in Table 1. Of note, 15 were
operated as urgent/emergent cases for aortic dissection.
Eighteen patients underwent reimplantation of all three
arch branches. Twenty-three patients did not require reimplantation of the left subclavian. Two patients underwent reimplantation of only the innominate. Arch branch reconstruction
ceased at the point where the aorta became free of disease.
Concomitant aortic root surgery was performed in 19 patients in whom six patients underwent root replacement via
the David reimplantation technique, while other valve-sparing
techniques (Yacoub remodeling or reconstruction of the noncoronary sinus and sino-tubular junction) were applied in four
patients. Nine patients underwent a Bentall’s procedure, with
three and six patients receiving mechanical and tissue valves,
respectively. Six patients underwent concomitant coronary artery bypass grafting.
Aorta, July 2013
103
Table 1. Preoperative Clinical and Operative Data
Patients (n ⫽ 43)
Clinical
Age
Male
Nonelective cases
Current smoker
Hypertension
Cerebrovascular disease
Diabetes
Coronary artery disease
Previous cardiac surgery
Previous Type A dissection
AVR/ASD
CABG
Coarctation
Bentall’s
Type A aortic dissection
Acute dissection
Chronic
Operative
Arch branches reimplanted
Trifurcation
Bifurcation
Innominate only
Side-arm inflow modification
Coronary artery bypass grafting
Bentall’s
Mechanical valve
Bioprosthesis
David reimplantation
Other valve-sparing
Elephant trunk
Frozen
Regular
Miscellaneous
Cardiopulmonary bypass time (min)
Minimum temperature (degrees C)
Cerebral exclusion time
Cerebral perfusion flow rate (L/min)
Distil circulatory arrest
64 (55–74)
27 (63)
15 (35)
12 (28)
30 (70)
3 (7)
1 (2)
13 (30)
7 (16)
3 (7)
1 (2)
1 (2)
1 (2)
1 (2)
19 (44)
15 (35)
4 (9)
18 (42)
23 (53)
2 (5)
18 (42)
6 (14)
9 (21)
3 (7)
6 (14)
6 (14)
4 (9)
6 (14)
4 (9)
2 (5)
1 (2)
285 (219–329)
27 (22–31)
165 (133–222)
1.0 (0.8–1.4)
20 (47)
Continuous variables expressed as median (interquartile range).
Categorical values expressed as absolute values (percentages). AVR/ASD indicates
aortic valve replacement, atrial septal defect; CABG, coronary artery bypass grafting.
Operative Technique
Preoperative investigations include axial computerized tomographic angiography (CTA) of the thoracic and abdominal
aorta and transesophageal echocardiography (TEE). Intraoperative cerebral monitoring is performed by a combination of
electroencephalogram bispectral index (BIS) monitoring, cerebral oximetry (INVOS 3100, Somanetics Corp, Troy, MI) and
transcranial Doppler (TCD).
Volume 1, Issue 2: 102–109
104
Figure 1. Arterial cannulation is femoral. In cases of severe
aortoiliac atheroma, axillary or direct ascending aortic cannulation may be used.
The chest is opened via a median sternotomy. Cardiopulmonary bypass is instituted via femoral arterial inflow and
central right atrial drainage (Fig. 1). Left axillary or direct ascending aortic [3,4] cannulation could be used in cases of
severe aortoiliac occlusive or iliofemoral dissection or severe
descending aortic atheroma, although this was only necessary
in a couple of cases.
In the initial experience, left axillary cannulation was added to
the femoral inflow to act as a source for antegrade perfusion to
the arch branches reimplanted into the trifurcation graft [2]. In the
last 18 patients, we totally replaced the need for axillary cannulation by the use of a modified trifurcation graft with an added
perfusion side arm (Vascutek Ltd., Renfrewshire, Scotland, UK).
The arch branches are exposed for a length of 3– 4 cm
using a “no touch” technique. To facilitate this, the thymus is
divided in the midline and the innominate vein is mobilized by
dividing all its tributaries. This allows complete mobility of the
latter structure without having to divide it and potentially
impede left cerebral venous drainage.
The innominate artery is clamped just proximal to its bifurcation and about 1 cm distal to its origin from the arch (Fig.
2A). The innominate artery is then divided between the clamps
and proximal stump ligated, allowing removal of the proximal
clamp and excellent access to the distal innominate stump,
which is anastomosed to the first limb of the three-branched
Perera, N.K. et al.
Original Research Article
graft (Fig. 2B). After the innominate artery anastomosis is
completed and deairing maneuvers performed, the side arm of
the trifurcation graft is used for antegrade flow. Median cerebral
perfusion flow was 1.0 (0.8 –1.4) liters per minute with an aim to
achieve a right radial pressure of 50 –70 mm Hg.
Completion of the innominate anastomosis removes tension on the convexity of the arch, increases its mobility, and
enhances access to and exposure of the left carotid artery and
in turn the left subclavian artery. A similar process is followed for
the anastomosis and reperfusion of the second and third limbs of
the branched Dacron graft to the left carotid and left subclavian
arteries respectively (Fig. 2C and 2D).
Note that in roughly half the cases the nature of arch pathology allowed retention of the subclavian on the distal aorta, avoiding the need for this step. Where a large arch aneurysm interferes
with access to the left subclavian artery, we utilize a number of
maneuvers to facilitate its reconstruction. These include (1) a short
(1–2 cm) extension of the neck incision along the anterior border
of the left sternocleidomastoid muscle can greatly improve exposure; (2) temporarily decreasing the distal perfusion pressure,
which reduces the turgidity of the arch and avails more space;
and (3) delaying the left subclavian reconstruction until the descending aorta is clamped and the arch resected, thus leaving
ample room for left subclavian anastomosis.
At this stage, the perfused trifurcation graft can be laid
easily out of the field over the patient’s neck. It is important to
note that during this whole process the circulation was not
interrupted to either the heart or the distal organs. Also of note
is that all arch branch anastomoses are readily in view and complete hemostasis from these sites can be ensured with ease.
The proximal descending aorta is now readily mobilized. This
can be assisted by temporary reduction in distal perfusion to
increase its maneuverability. Also, division of the ligamentum
arteriosum is key to allowing the recurrent laryngeal nerve to
“drop away” from the aortic wall. Complete distal control with a
clamp is readily achieved in over half of the cases. In the remainder where this is difficult because of adhesions or fragility, use of
intraluminal balloon occlusion together with reduced distal flow
(so as to not dislodge the balloon) allows distal perfusion to
continue. Once the distal anastomosis is completed (20 –30 minutes), a clamp is applied to the graft, allowing resumption of full
distal flows. If an elephant trunk procedure is needed, then a brief
period of distal arrest is used to allow insertion of the prosthesis
into the descending aorta, then the composite descending and
(soft graft component of) the elephant trunk is controlled as
above to allow resumption of distal flows.
Distal anastomosis is performed between the distal arch/
descending aorta and an appropriate size tube Dacron graft with
a preattached single side arm graft (Ante-Flo Prosthesis, Vascutek
Ltd., Renfrewshire, Scotland, UK) (Fig. 2E), or in elephant trunk
cases the preexisting Dacron graft is anastomosed to the descending aorta. After completion of this anastomosis, distal body
flow is changed from femoral to the sidearm graft (or directly into
the graft in the case of frozen elephant trunk) and a clamp applied
to the main arch graft immediately proximal to the perfusion port.
Aortic root reconstruction can now proceed if required,
and anastomosis between the arch graft and root is com-
Outcomes of Aortic Arch Replacement
Original Research Article
105
Figure 2. (A) The innominate artery is clamped proximal to its bifurcation and distal to its origin from the arch and divided
between the clamps. (B) The innominate artery’s proximal stump is ligated and the distal anastomosis to the first limb of the
three-branched graft is performed. (C) The second limb of the branched graft is anastomosed to the left common carotid artery.
(D) The third limb is anastomosed to the left subclavian artery. (E) Distal anastomosis of the arch graft to the distal arch. (F) The
trunk of the trifurcation graft is passed under the innominate vein and anastomosed to the arch graft.
pleted. Finally, the trunk of the branched graft is passed deep
to the innominate vein and anastomosed to the ascending
graft, in end-to-side fashion, again without the need to interrupt cerebral perfusion (Fig. 2F).
using Predictive Analytics SoftWare Statistics Package 17.0 (SPSS
Inc., Chicago). Continuous variables are expressed as median (first
to third quartile) to account for their skewed distribution.
Data Collection and Analysis
Results
Clinical, investigative, operative, perfusion, and early postoperative data were prospectively collected in a departmental database, with additional data extracted from operation reports, perfusion reports, and intraoperative computerized records.
Follow-up data obtained from patients’ records was collected up
to August 1, 2011. Kaplan Meier survival analysis was performed
Intraoperative
Intraoperative data are summarized in Table 1. Median cardiopulmonary bypass time was 285 (219 to 339)
minutes. The median minimum temperature was 27 (22–
Aorta, July 2013
Volume 1, Issue 2: 102–109
106
Original Research Article
Table 2. Early Postoperative Outcomes
Patients (n ⫽ 43)
In-hospital mortality
Neurological dysfunction
Stroke
Visual loss
Hemiparesis
Residual deficit
Return for bleeding
Tracheostomy
Mechanical support
Renal support
Ischemic gut
Ischemic limb
Transfusion
Red cells (units)
Platelets (units)
No transfusion (of both)
No transfusion (either)
Ventilation time ⬍24 h
ICU time ⬍48 h
Hospital stay ⬍7 d
2 (5)
3 (7)
1 (2)
1 (2)
1 (2)
1 (2)
5 (12)
3 (7)
1 (2)
3 (7)
0 (0)
1 (2)
2 (1–5)
2 (0–4)
8 (19)
20 (47)
21 (49)
20 (47)
14 (33)
Continuous variables expressed as median (inter-quartile range).
Categorical values expressed as absolute values (percentages).
31) degrees Celsius. The lower range temperatures represent extra caution exercised early in our experience.
Median cerebral perfusion flow was 1.0 (0.8 –1.4) liters
per minute with an aim to achieve a right radial pressure
of 50 –70 mm Hg. Cerebral perfusion was maintained on
a separate antegrade circuit for a median duration of 165
(133–222) minutes. In 20 patients with adhesions or
difficult access where distal clamping proved difficult,
distal low flow combined with antegrade perfusion via a
balloon occlusion catheter was used with moderate hypothermia (26 –28 degrees Celsius).
Early Postoperative Outcomes
Postoperative outcomes are detailed in Table 2.
There were two mortalities in the early post operative
period. The first was due to right ventricular failure in
an 85-year-old female patient. She had undergone
emergency arch and root replacement in combination
with coronary artery bypass grafting (CABG) for a
delayed presentation of an acute Type A dissection
with a preoperative right ventricular infarct and dysfunction. The second early mortality was in a 61-yearold male patient with acute Type A dissection associated with preoperative malperfusion of the lower
Perera, N.K. et al.
limbs and gut. He underwent emergency ascending,
arch, and frozen elephant trunk replacement. The procedure was completed uneventfully, however, he continued to suffer the consequences of preexisting gut
malperfusion and died of multiorgan failure.
Three patients (7%) experienced neurological dysfunction. The first patient experienced amaurosis fugax,
while the second patient experienced left hemiparesis.
Both of these conditions resolved completely. These almost certainly occurred secondary to embolic events
rather than hypoperfusion. Both early and delayed computed tomography of the brain in both patients did not
show any infarction or hemorrhage. The third patient
experienced short-term memory loss and expressive dysphasia that did not completely resolve. This occurred on
the background of preexisting cerebrovascular disease
and an acute Type A dissection with cerebral malperfusion (Table 2). There were no cases of global dysfunction
or watershed infarcts to suggest inadequacy of collateral
circulation during arch branch clamping. There was one
case of transitory left hand hypoperfusion after ligation
of an atheromatous left subclavian artery, which recovered spontaneously and did not require a carotidsubclavian bypass.
Thirty-two patients (74%) did not experience any
complications. In eight patients (19%), neither red
blood cells nor any other blood product was required.
Twenty-one patients were extubated within 24 hours
and 20 were discharged from the ICU within 48 hours.
Three patients (7%) required a tracheostomy while
five patients (12%) returned to theater for bleeding.
Follow-up
Median follow-up duration was 21 ⫾ 19 months and
100% complete. There was one late death, occurring in a
patient with nonsmall cell lung cancer 58 months after
arch replacement. At three years, survival was 95 ⫾ 3.2%.
No patients required reoperation for residual or recurrent aortic pathology. There were no cases of aortic
rupture or acute dissection. At last follow-up 31 (72%)
patients were in New York Heart Association (NYHA) class 1.
The Kaplan Meier survival curve is displayed in Table 3.
Discussion
The combination of deep hypothermia and antegrade cerebral perfusion remains the mainstay of organ protection during circulatory arrest for arch surgery [5,6], yet the reported outcomes are still less
Outcomes of Aortic Arch Replacement
Original Research Article
107
Table 3. Follow-up
Follow-up 100%
Patients (n ⫽ 43)
At last follow-up (months)
New York Heart Association (NYHA) level
I
II
III/IV
21⫾19
31
8
1
favorable compared to procedures on the more proximal aorta especially in terms of cerebral events. In
addition, deep hypothermia carries its own spectrum
of complications [1,6], which may include coagulopathy. Periods of global circulatory arrest of as short as
20 minutes have been shown to be deleterious to
higher mental function and fine motor skills [1].
The advantages of the branch-first continuous perfusion technique used in our center have been discussed in detail previously [2]. The essential advantage
is that there are no periods of global circulatory arrest,
thus possibly minimizing cerebral morbidity. Cardiac
perfusion is maintained throughout the whole of the
arch branch reconstruction phase, significantly reducing the period of time of reliance on cardioplegia and
the risk of myocardial dysfunction. Maintenance of
distal organ and especially liver and kidney perfusion
during arch reconstruction reduces the risk of postoperative vital organ dysfunction and postoperative
bleeding and may shorten ICU stays.
The two early postoperative mortalities represent a
4.7% in-hospital mortality rate. Both of these occurred in
patients presenting with acute Type A dissection with
malperfusion syndromes, which is known to have a high
in-hospital mortality rate [7]. Nonetheless our results are
in line with contemporary studies reporting 30-day inhospital mortalities ranging between 3.4% to 13% [8–12].
We also continued to observe a low incidence of renal,
gastrointestinal, hepatic, and ventilatory impairment.
Reported rates of permanent stroke in contemporary aortic surgery range from 2.0% to 4.8% [1,5,13–
15]. The two transient and one permanent neurological deficit sustained in our series gives an incidence in
line with these. Importantly, these deficits most likely
occurred secondary to embolic events and not hypoperfusion infarcts, thus supporting the safety of individual arch branch clamping.
Early survival at 3 years in this series was 95%. Although longer follow up is required, these results are
Aorta, July 2013
comparable to larger studies that have reported 3- to
5-year survival between 71% and 87% [8,10–12]. Importantly, no patient has required a reoperation for aortic
pathology. If this persists into the long-term follow-up, it
may be a testimony to the benefit that the maintenance
of cerebral, cardiac, and distal body perfusion in this
technique allows even the most complex reconstructions to be completed meticulously in an unhurried
fashion, thus providing complete correction of pathology and eliminating imperfections that may have otherwise been tolerated in view of time pressures. This may
also be a reflection of the excellent hemostasis achieved,
as all anastomoses’ suture lines remain visible and accessible at each stage of the procedure.
The absence of abnormalities in cerebral monitoring
during the reconstruction of the left common carotid
artery in cases leading up to 2009 encouraged us to
apply the same principle “in reverse” to innominate artery clamping. Again this was supported by no abnormalities being detected on cerebral monitoring during
the relatively short periods of innominate clamping required. This eliminated the need for axillary artery cannulation and its low but definite risks of axillary artery
injury, dissection, or brachial plexus injury [16,17] as well
as increased operative time. The latter is especially undesirable during emergency cases. This is particularly the
case in obese patients and those with fragile or smallcaliber axillary arteries. This technique has evolved to
include an added side-arm to the trifurcation graft to
provide direct cerebral perfusion. This modified technique has simplified the procedure, lessened technical
demand, and has the potential to bring aortic arch replacement into the armamentarium of the nonaortic
subspecialized cardiac surgeon.
There may be a number of potential disadvantages
of this technique, which have also been previously
discussed [2]. Specifically, a drawback of the modified
technique described here is that direct right common
carotid inflow is interrupted for the anastomosis of the
first limb of the branched graft to the innominate
artery. This is, however, analogous to interruption of
direct left common carotid inflow during anastomosis
of the second limb of the branched graft in the previous technique. We have not encountered any abnormality in intraoperative cerebral monitoring during
this phase of the procedure thus far. This is most likely
due to the extremely rich collateral network in the
head, neck, and body wall connecting the three arch
branches’ distribution in addition to the typically short
Volume 1, Issue 2: 102–109
108
artery clamp times (typically 10 –12 minutes). This collateral system significantly supplements the capacity
of the Circle of Willis. Despite this theoretical disadvantage, our early experience has supported the ongoing use of this modification.
We acknowledge that cardiopulmonary bypass times
are not significantly reduced by our technique and some
might argue that the use of deep hypothermic circulatory arrest (DHCA) (along with the associated periods of
cooling and rewarming) would result in similar operative
and cardiopulmonary bypass times to those that we
report. While we agree that DHCA is a well-established
technique for arch reconstruction and that it provides
the surgeon with a bloodless and uncluttered operative
field we feel that its use is associated with a number of
clinically significant disadvantages that are not solely
associated with the periods required for cooling and
rewarming. It is well established that even short periods
of DHCA are associated with subtle higher cerebral dysfunction [1,18,19], cerebral reperfusion injury [20], impairment of normal cerebrovascular regulatory mechanisms [21–23], and the generation of excessive cerebral
temperature gradients [24,25]. Although cerebral injury
can be reduced by the use of ancillary methods of
cerebral protection such as antegrade or retrograde cerebral perfusion [26–28], many of those techniques impose various periods of total circulatory arrest. Furthermore, while much of the emphasis during periods of
circulatory arrest is focused on avoidance of cerebral
injury, preservation of other organs such as the liver,
kidneys, and spinal cord is often not specifically addressed, their protection relying on deep hypothermia
alone. It is this global hypoperfusion of other organs that
occurs during prolonged periods of DHCA with or without cerebral perfusion that we feel leads to much of the
morbidity associated with arch surgery. Although the
clinical impact of this organ ischemia may be clinically
significant as acute specific organ failure, more often it
masquerades as more subtle end organ dysfunction culminating in sepsis, gastrointestinal bleeding, and multi-
Original Research Article
organ failure. Thus, while our cardiopulmonary bypass
times are not shorter than DHCA techniques, it is our
opinion that the avoidance of deep hypothermia and,
more particularly, global circulatory arrest results in
lower morbidity and mortality [29].
We acknowledge the limitations of this series, primarily its small size, institutional bias, and evolution of
technique over time. As expected, we have noticed
shorter bypass and ischemic times with increasing
experience that may translate to improved outcomes
toward the latter stages of the learning curve. Care is
still required to handle the aorta with a no touch
technique so as to avoid the risks of embolic events
caused by atheromatous disease.
Conclusion
This branch-first continuous perfusion technique
brings us closer to the goal of arch surgery without
cerebral or visceral circulatory arrest and the morbidity
of deep hypothermia. This technique presents another
alternative to established techniques in aortic arch
surgery. The modification described here technically
partially simplifies a demanding procedure while our
early experience remains encouraging. Greater numbers and follow-up are anticipated.
Acknowledgments
The authors would like to acknowledge the assistance of Dr. John McKay in addition to Ms. Margaret
Shaw and Beverley Toone. We also thank Ms. Beth
Croce for her excellent illustrations.
Conflict of interest
None
Comment on this Article or Ask a Question
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EDITOR’S COMMENTS AND QUESTIONS
Matalanis shows us his technique for an ingenious
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Aorta, July 2013
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Cite this article as: Perera NK, Shi WY,
Koirala RS, Galvin SD, McCall PR,
Matalanis G. Outcomes of Aortic Arch
Replacement Performed Without Circulatory Arrest or Deep Hypothermia.
Aorta 2013;1(2):102–109. DOI: http://dx.
doi.org/10.12945/j.aorta.2013.12.007
arrest, albeit at the “expense” of short durations of deprivation of blood flow to individual arch branches. He
has accumulated a considerable–and very favorable–
experience with this alternative technique.
Volume 1, Issue 2: 102–109
Original Research Article
Aorta, July 2013, Volume 1, Issue 2: 110 –116
DOI: http://dx.doi.org/10.12945/j.aorta.2013.13-008
Received: January 29, 2013
Accepted: May 10, 2013
Published online: July 2013
Urgent Carotid Endarterectomy in Patients with
Acute Neurological Symptoms
The Results of a Single Center Prospective Nonrandomized Study
Samuel Bruls, MD1, Philippe Desfontaines, MD2, Jean-Olivier Defraigne, MD, PhD1,
Natzi Sakalihasan, MD, PhD1,3*
1
Department of Cardiovascular and Thoracic Surgery, University Hospital of Liege, Liege, Belgium; 2Department of Neurology and Stroke
Unit, CHC, Liege, Belgium; and 3Department of Vascular and Thoracic Surgery, CHC, Liege, Belgium
Abstract
Background: To evaluate the feasibility and the safety
of performing urgent (within 24 hours) carotid endarterectomy in patients with carotid stenosis presenting
with repetitive transient ischemic attacks or progressing stroke. Methods: Thirty consecutive patients underwent urgent carotid endarterectomy for repetitive transient ischemic attacks (N ⴝ 12) or progressing stroke
(N ⴝ 18) according to the following criteria: two or
more transient ischemic attacks or a fluctuating neurological deficit over a period of less than 24 hours (progressing stroke), no impairment of consciousness, no
cerebral infarct larger than 1.5 cm in diameter on computed tomography and a carotid artery stenosis of 70%
or more on the appropriate side, diagnosed by
echodoppler ultrasonography and/or arteriography.
Patients with cerebral hemorrhage were excluded. All
patients were examined pre- and postoperatively by
the same neurologist and surgery was performed by
the same vascular surgeon. All the patients underwent
a cerebral CT scan within 5 days after surgery. Results:
There were 19 men and 11 women. The mean age was
71 ⴞ 7.6 years. The time delay of surgery after the
onset of transient ischemic attacks or progressing
stroke averaged 19.4 ⴞ 11.5 hours. For patients suffering progressive stroke, one developed a fatal ischemic
stroke 24 hours after surgery, five showed no improvement of their neurological status after surgery, but
none worsened. Twelve patients experienced signifi-
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Published by Science International Corp.
ISSN 2325-4637
Accessible online at:
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cant improvement of their neurological status with an
European Stroke Scale of 77.9 ⴞ 25.2 at admission and
95.8 ⴞ 4.6 at discharge, and all but one of those patients had a Barthel’s index value over 85/100 at discharge. The 12 patients with repetitive transient ischemic attacks had an uneventful postoperative outcome.
The mean duration of follow-up was 3.4 ⴞ 1.2 years. No
patient developed another transient ischemic attack or
ischemic stroke during the follow-up period. Conclusions: The results of our series documented the feasibility and the safety of performing urgent (within
24 hours) carotid endarterectomy in patients presenting with repetitive transient ischemic attacks or
progressing stroke. This procedure seems to us to be
justified by the fact that waiting for surgery may lead
to the development of a more profound deficit or
another stroke in these neurologically unstable patients whose only chance for neurological recovery is
in the early phase.
Copyright © 2013 Science International Corp.
Key Words
Carotid endarterectomy · Transient ischemic attacks ·
Stroke in evolution
Introduction
Carotid endarterectomy (CEA), first performed in
1953 by DeBakey [1], is an effective and recognized
*Corresponding author:
Natzi Sakalihasan MD, PhD
Department of Cardiovascular and Thoracic Surgery
University Hospital of Liege
CHU Sart-Tilman, 4000
Liege, Belgium
Tel: ⫹32 4366 7163, Fax: ⫹32 4366 7164, E-Mail: [email protected]
Original Research Article
vascular elective procedure for symptomatic patients
with moderate or severe (ⱖ70%) carotid stenosis and in
patients with severe asymptomatic stenosis [2,3]. But the
best timing to perform CEA in patients with acute neurological symptoms (repetitive transient ischemic attacks, minor stroke or stroke-in-evolution) has for a long
time been subject to controversy and is still a source of
debate. In fact, to our knowledge, there are no prospective randomized trials to determine which neurologically
unstable patient (presenting repetitive transient ischemic attacks or stroke-in-evolution), might safely undergo urgent or delayed CEA.
In the past, the increased risks of reperfusion injury
and conversion to hemorrhagic infarction have led to
the historical recommendation of delayed CEA in patients with acute neurological symptoms. But, in recent years, most published data demonstrated that
the risk of recurrent stroke in the first few days after a
transient ischemic attack (TIA) or minor stroke appears
to be much higher than previously estimated. Rothwell et al. [4], assessed the risk of stroke at 7, 30, and
90 days first after TIA as 8, 11.5, and 17.3% respectively
and after a minor stroke as 11.5, 15, and 18.5%. On the
other hand, some centers report the safety and efficacy of urgent CEA (before two weeks) after acute
minor stroke, repetitive TIAs, or stroke-in-evolution
(SIE) [5–13]. A subanalysis of NASCET results [14,15]
illustrates a rapid decline of the benefit of CEA over
time in terms of stroke prevention after the index focal
neurological deficit (TIA or minor stroke). Recent
guidelines document that early surgery is associated
with increased benefits compared with delayed surgery for secondary stroke prevention and recommend
CEA within two weeks for patients presenting with a
TIA or minor stroke [16].
We performed a prospective nonrandomized protocol for urgent (within 24 h) CEA in neurologically
unstable patients (presenting with repetitive TIA or
progressing stroke) with a symptomatic carotid stenosis of more than 70% in order to assess the safety of
this therapeutic approach.
Methods
During a five year period, we performed a single center,
prospective, nonrandomized consecutive series of urgent CEA.
In accordance with the NASCET and ECST studies [3,2] and the
Charing Cross series results [17], the following inclusion criteria
have been used: symptomatic carotid stenosis of 70% or more,
Aorta, July 2013
111
Table 1. Inclusion Criteria for Patient Enrollment
Inclusion criteria
Symptomatic carotid stenosis of 70% or more
Unstable neurological status consisting in repetitive TIA or
progressive stroke evolving no longer than 24 h
No impairment of consciousness
No cerebral infarct larger than 1.5 cm in diameter on
preoperative CT-scan
with unstable neurological status consisting in repetitive TIA or
progressive stroke evolving no longer than 24 hour, no impairment of consciousness, and no cerebral infarct larger than 1.5
cm in diameter on preoperative CT scan (Table 1). There were
no exclusion criteria except for age over 80 years. Hemorrhage
seen on the initial CT-scan eliminated the patient from the
study. The term “progressing stroke” is applied to patients with
a neurological deficit that has progressed or fluctuated over a
period of at least 24 hours. The diagnosis of carotid stenosis
was based on echodoppler ultrasonography and/or selective
carotid angiography. The degree of stenosis was determined
by means of the North American Symptomatic Carotid Endarterectomy Trial (NASCET) method. All patients were examined
by the same neurologist pre- and postoperatively (PD). Neurological evaluation of the patients was blinded from the surgeon’s clinical examination to avoid under or overestimation in
the patient’s clinical status.
All patients were scored by the European Stroke Scale (ESS)
[18] at admission and at discharge (maximum ESS score is 100
and indicates a patient without any neurological deficit). Barthel’s index [19] was only evaluated at hospital discharge
because it is impossible to determine preoperatively patient’s
autonomies. It is considered that a patient is independent at
home if his score (Barthel’s index) exceeds 85. The preoperative
investigation included in all cases: blood sample analysis, ECG
and/or cardiac echography, chest X-ray, carotid echo color
Doppler ultrasonography, selective angiography of the carotid
arteries, and cerebral CT-scan. No MRI was performed because
MRI was not accessible on an emergency basis. From the day
of the admission to the discharge from hospital, all patients
received heparin at a prophylactic dose, along with statin
therapy. As CEA was performed on an emergency basis (within
the first 24 hours), no aspirin was administred preoperatively.
A standard surgical open endarterectomy, with Javid shunt
(to maintain cerebral circulation during surgery) and prosthetic
patching, was performed under general anesthesia by the
same vascular surgeon (NS) in all cases. The postoperative care
was performed in a stroke unit, with ECG, noninvasive arterial
blood pressure monitoring, and transcutaneous oxygen saturation monitoring for at least 48 hours. All the patients underwent a cerebral CT scan before discharge, within the five days
after surgery. In the postoperative period, patients were maintained on a low dose of heparin (4000 IU) and statin therapy
together with their scheduled medications. At discharge from
the hospital, antiplatelet therapy (acetylsalicylic acid 100 mg
daily) was started. During regular follow-up, all patients were
Volume 1, Issue 2: 110 –116
112
Original Research Article
Table 2. Patient’s Characteristics and Medical History
No. of patients (%)
Demography
Mean age (y)
Men: Women
Neurologic clinical presentation
Repetitive TIA (ⱖ 2/24h)
Progressive stroke
Comorbidities
Hypertension
Diabetes
Hypercholesterolemia
Smoking history
Previous myocardial infarction
Previous TIA and/or stroke
71
19 (63): 11 (37)
12 (40)
18 (60)
6
3
19
14
3
0
reviewed by the same neurologist (PD) and the same surgeon
(NS) independently from each other at six weeks after surgery
and every six months during the first year, and every 12
months thereafter. Assessment of outcome was based on follow-up control examination.
Statistical Analysis
Patient details, including age,
gender, and comorbidities were collected in an Excel database
(Microsoft Ltd). Categorical data were presented as absolute
frequencies and percent values. Quantitative measurements
were expressed as mean ⫾ SD. Data on survival, neurological
events, and patency was studied directly.
Results
Patients Characteristics
The study concerns
30 consecutive patients included out of a series of 638
patients admitted to the emergency department for
acute TIA or progressive stroke during a five year period.
In these 30 patients, CEA was performed within 24 hours
following the neurological event (repetitive TIA or progressive stroke). Of these, there were 12 patients presenting with repetitive TIA and 18 progressive strokes.
There were 19 men and 11 women with a mean age of
71 ⫾ 7.6 years. No patient had any neurological deficit
before the onset of repetitive TIA or progressing stroke.
Baseline patient characteristics and medical history are
presented in Table 2.
Perioperative Characteristics
All patients had
documented internal carotid artery stenosis of 70% or
more. For patients suffering progressive stroke (n ⫽
18), the degree of carotid artery stenosis was 85% or
more. The mean delay of surgery after onset of the
first TIA or progressive stroke was 19.4 (⫾11.5) hours
Bruls, S. et al.
(range, 6 – 48 hours). At operation, the macroscopic
examination of the internal carotid artery in all 30
patients showed a complex ulcerated plaque and/or
an intraplaque hemorrhage.
Outcomes
One patient (5%) with initial progressive stroke developed a fatal ischemic stroke
within 24 hours after the operation, and Doppler ultrasonography performed immediately showed very
good patency of the operated carotid artery. Five
(28%) of the 18 patients with progressive stroke had
an incomplete recovery with limited residual neurological deficit and experienced no clinical improvement but none of them worsened after the operation,
whereas the 12 other patients (67%) with residual
neurological deficit (as a result of their progressive
stroke) showed significant improvement of their clinical status. The 12 patients with repetitive TIA remained free of neurological deficit after the operation.
All but one of the 18 patients with progressive stroke
had a Barthel’s index over 85 at hospital discharge.
The mean ESS of the 18 patients with progressive
stroke was 77.9 ⫾ 25 at admission, and was 95.8 ⫾ 4.6
at discharge. All the patients underwent a cerebral CT
scan within five days after surgery. No hemorrhagic
transformation of cerebral infarcts was detected. No
new lesion on postoperative CT scan was found in the
12 cases of TIA. All of the 18 progressive stroke patients had a lacunar size lesion (⬍15 mm); there had
been no enlargement of the lesion postoperatively
except in one case in which there was a large infarction (⬎2 cm). There was no reoperation for cervical
hemorrhage or wound infection. No patients developed vocal cord paralysis due to nerve injury. Patients
were discharged after a median of four days (range,
4 –10 days). The mean duration of follow-up was 3.4
years (⫾1.2) and was 100% complete in all patients.
No residual or recurrent stenosis was documented on
echo color Doppler ultrasonography follow-up. No
recurrent stroke and/or TIA, no cardiac event, and no
death occurred in this series during follow-up.
Discussion
Timing of CEA in patients with acute neurological
symptoms still remains a challenging but unresolved
problem [5–7,20]. The management uncertainty can be
explained by the inability to predict who is at higher
early risk of a recurrent stroke after a cerebrovascular
Urgent Carotid Surgery in Symptomatic Patients
Original Research Article
113
Table 3. Results of Urgent CEA (within 15 d) Reported Recently in the Literature
Author and year
No. of
patients
Mean interval between
symptom and CEA
In-Hospital
mortality rate
Gertler et al., 1994 [21]ⴱ
Schneider et al., 1999 [22]a
Brandl et al., 2001 [23]a
Gay et al., 2002 [11]ⴱ
Huber et al., 2003 [24]ⴱ
Sbariga et al., 2006 [10]a
Karkos et al., 2007 [8]b
Bazan et al., 2008 [6]ⴱ
Ballotta et al., 2008 [25]a
Gorlitzer et al., 2009 [9]ⴱ
Leseche et al., 2011 [12, 13]a,b
Dorigo et al., 2011 [26]ⴱ
Capoccia et al., 2012 [27]ⴱ
Present studyⴱ
52
43
16
21
67
96
42
764
102
28
91
75
48
30
⬍24 h
ⱕ72 h
⬍24 h
⬍24 h
2d
1.5 d
3d
–
8d
4d
5d
⬍24 h
⬍24 h
⬍24 h
0%
0%
0%
9.5%
3.0%
2%
4.8%
2.0%
0%
0%
0%
2.7%
2.0%
3.3%
Complications
(stroke rate)
2.0%
0%
–
–
13%
0%
4.8%/19%
2.88%/3.1%
0%
0%
0%
–
2.0%
3.3%
ⴱMixed study including patients with acute stroke and TIA.
a
Study concerns patients with acute ischemic stroke.
b
Study concerns patients with crescendo TIA.
event (TIA or stroke). Interestingly, a subanalysis of the
NASCET results has revealed that the benefit of CEA
versus medical treatment is greatest if the symptomatic
carotid artery stenosis is operated within two weeks
following the index neurological event [14,15]. Among
the medically treated patients, the risk of ipsilateral
stroke is highest immediately after the initial ischemic
event and subsequently drops dramatically [17].
In recent years, several studies (Table 3) have shown
very good results and outcomes for urgent CEA procedures. In the Charing Cross series [17], 19 patients
suffering from progressing stroke and 14 patients presenting with repetitive TIA underwent urgent CEA (all
patients were operated within 48 hours after the onset
of symptoms). There was a good evolution in all but
three cases. All the patients had a small infarct size
(⬍2.0 cm) on preoperative CT scan, were conscious,
and had a mild neurological deficit. The criteria for the
selection of our patients have been chosen in the light
of the results of the Charing Cross series. The choice of
these criteria was based on the assumption that a
severe neurological deficit or impaired consciousness
often implies a large infarction in progress, eventually
but not yet visualized on early cerebral CT scan, leading to a higher risk of postoperative bleeding because
of hyperperfusion in a large ischemic brain area. In our
study, using these criteria, all but one patient had a
Aorta, July 2013
good outcome. One patient suffered a fatal stroke due
to postoperative enlargement of the existing small
cerebral infarction. Intraoperative embolization was
probably the cause because Doppler ultrasonography
performed immediately showed very good patency of
the operated carotid artery.
Gertler et al. [21] reported their experience in neurologically unstable patients with carotid stenosis presenting with crescendo TIA and SIE, of whom only one
patient (2.7%) worsened his neurological deficit after
CEA within 24 hours. Based on these good results, they
recommend urgent CEA. Most recently, Leseche et al.
[12,13] reported excellent outcome of urgent CEA in the
acute phase of SIE and crescendo TIA, with no perioperative stroke or death. The mean delay to surgery from
initial examination was five days. For patients operated
for SIE, a complete neurological recovery was observed
in 81% of patients, while 19% maintained a residual
deficit. No patient presented a worsening of his deficit
following urgent CEA. In a meta-analysis of 47 studies on
carotid surgery published between 1980 and 2008, Rerkasem et al. [20] found no excess operative risk for early
(urgent) CEA versus delayed CEA.
However, less favorable outcome after urgent CEA
in neurologically unstable patients has been reported
by other investigators. These studies demonstrated
a higher rate of perioperative complications after
Volume 1, Issue 2: 110 –116
114
urgent CEA in neurologically unstable patients (presenting crescendo TIA and SIE) compared with delayed CEA. Karkos et al., [7] in a meta-analysis, reported a 16.9% perioperative stroke rate and a 20%
combined stroke/death rate for urgent CEA after
stroke-in-evolution. Considering crescendo TIA, the
analysis revealed a more favorable outcome (6.5%
perioperative stroke rate and a 9% combined
stroke/death). In a meta-analysis done by Bond et al.
[28] and Halm et al. [29], the operative risk of CEA
increases when it is performed after stroke-inevolution (a 14.0% 30-day stroke/death rate), compared to a 2.8% 30-day stroke/death rate after CEA
for asymptomatic carotid artery stenosis.
This rather elevated morbidity-mortality should be
balanced against the stroke risk in these neurologically
unstable patients if surgery had not been performed.
Actually, no randomized controlled trial has been done
comparing the outcome of crescendo TIA and stroke-inevolution treated medically versus urgently operated.
Early CEA for symptomatic severe carotid stenosis
(ⱖ70%) in neurologically unstable patients may be
justified by the instability of the lesion, in order to
prevent a subsequent complete or more severe stroke.
In all of our patients, an ulcerative or hemorrhagic
plaque was discovered intraoperatively. Urgent removal of this unstable embolic source has some logic.
An arbitrary 2-week delay for CEA probably exceeds
the risk of urgent CEA and may expose the neurologically unstable patient to a risk of recurrent or more
disabling stroke, or to an occlusion of the internal
carotid artery. Before starting our prospective study, in
some cases with a small cerebral lesion, we had chosen medical treatment before performing urgent surgery. Unfortunately in some cases, we observed fatal
neurological events a few days later, after the first
neurological events (unpublished data).
In contemporary literature, there exists consensus
that a patient presenting with an acute, nondisabling
neurological deficit with complete or partial recovery
should benefit from a carotid endarterectomy without
delay. For the high-risk group of neurologically unstable patients (crescendo TIA and SIE), who often present with subocclusive stenosis with friable ulcerated
plaque, the current available literature data are less
conclusive. Some series reporting a rather high perioperative morbidity-mortality seem to discourage urgent CEA in this setting. However, in our small expe-
Bruls, S. et al.
Original Research Article
rience, and in some centers of excellence, the
operative outcome of urgent CEA in neurologically
unstable patients was favorable. The creation of a
“Stroke Unit” could favor the management and development of urgent CEA while allowing better selection
and management of these unstable patients.
Limitations of the Study
Our results should
be interpreted in light of several limitations. First, the
number of patients enrolled in this single center prospective study is too small to give definite conclusions.
This was the reason why a formal statistical analysis
was not performed. However, due to the heterogeneity and paucity of data in the literature, subject to
controversy and still a source of debate, our experience may add to the management of these unstable
neurological patients. Second, it is important to note
that our study is not randomized. Although randomized trials are certainly the gold standard in clinical
study, in neurologically unstable patients, such a trial
is difficult for ethical reasons.
Conclusion
Our consecutive series shows that urgent CEA
can be performed safely in selected patients with an
evolving or unstable neurological deficit. It also
confirms the relevance of some previously noticed
criteria for the prognosis of urgent CEA, such as a
normal level of consciousness, the absence of large
cerebral infarction on preoperative cerebral CT scan,
and the limited severity of the neurological deficit
before the operation. We may recommend surgery
within 24 hours for all symptomatic patients with unstable plaques diagnosed by imaging tools. Urgent CEA
seems to us to be justified by the fact that a symptomatic
carotid stenosis is an unstable lesion and waiting may
lead to the development of another stroke that is more
disabling for the patient. The perioperative risk can be
reduced with better diagnostic strategies and must be
balanced against the natural history if surgery is not
performed. Only a large randomized multicenter prospective trial will be able to conclusively assess the effectiveness and outcome of urgent CEA in neurologically
unstable patients.
Comment on this Article or Ask a Question
Urgent Carotid Surgery in Symptomatic Patients
Original Research Article
115
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Cite this article as: Bruls S, Desfontaines
P, Defraigne J-O, Sakalihasan N. Urgent
Carotid Endarterectomy in Patients
with Acute Neurological Symptoms:
The Results of a Single Center Prospective Nonrandomized Study. Aorta
2013;1(2):110 –116. DOI: http://dx.doi.
org/10.12945/j.aorta.2013.13-008
Volume 1, Issue 2: 110 –116
116
EDITOR’S COMMENTS
Dr. Sakalihasan and colleagues show us that
prompt open surgical therapy for recurrent TIAs or
unstable stroke in evolution can lead to excellent
clinical outcomes and apparent salvage of at-risk
cerebral tissue. They have taken a courageous posture. While not a large-scale randomized study, this
report gives an important “real world” glimpse at
what can be accomplished with aggressive, non-
Bruls, S. et al.
Original Research Article
timid surgical care. We look forward to watching
their experience grow based on these very favorable institutional results. It is important to note that
the operated patients were selected from among
hundreds of patients presenting during the time
interval of this study; clinical judgment in patient
selection, in addition to the stated inclusion and
exclusion criteria, likely was an important factor in
attaining favorable results.
Urgent Carotid Surgery in Symptomatic Patients
State-of-the-Art Review
Aorta, July 2013, Volume 1, Issue 2: 117–122
DOI: http://dx.doi.org/10.12945/j.aorta.2013.12-009
Received: November 20, 2012
Accepted: May 13, 2013
Published online: July 2013
Acute Traumatic Thoracic Aortic Injury
Considerations and Reflections on the Endovascular Aneurysm
Repair
Luca Di Marco, MD, PhD*, Davide Pacini, MD, Roberto Di Bartolomeo, MD
Cardiac Surgery Department, S. Orsola-Malpighi Hospital, University of Bologna, Bologna, Italy
Abstract
Key Words
Traumatic rupture of the thoracic aorta is a lifethreatening lesion and it occurs in 10 to 30% of
fatalities from blunt thoracic trauma and is the second most common cause of death after head injury.
Immediate surgery is often characterized by a high
mortality and morbidity rate. Delayed repair of traumatic aortic injuries has significant survival benefits
and a much lower mortality rate compared with early
open repair. Despite developments in operative techniques, there still remains considerable operative
mortality and morbidity associated with a surgical
approach even if delayed. Endovascular stent grafts
for the thoracic aorta represents an alternative to the
conventional approach for traumatic aortic rupture.
Because of the lower invasivity avoiding thoracotomy and use of heparin, endovascular repair can be
applied in acute patients without the risk of destabilizing pulmonary, head or abdominal traumatic lesions. However, despite the good deal of convincing
evidence for endovascular treatment for thoracic aortic diseases and for traumatic aortic injuries as a valid
and efficacious alternative to surgery, several reports
show a variety of late complications of thoracic endografts especially for first-generation stent-grafts.
In light of this, is the endovascular treatment really
safe, efficacious and free from complications in the
long term? This manuscript aims to offer a moment of
reflection on this important chapter of aortic
pathology.
Copyright © 2013 Science International Corp.
Aortic · Traumatic · Endovascular · Acute injury
Fax ⫹1 203 785 3346
E-Mail: [email protected]
http://aorta.scienceinternational.org
© 2013 Aorta.
Published by Science International Corp.
ISSN 2325-4637
Accessible online at:
http://aorta.scienceinternational.org
Introduction
Traumatic rupture of the thoracic aorta is a lifethreatening lesion and it occurs in 10 to 30% of
deaths due to blunt thoracic trauma and it is the
second most common cause of death after head injuries [1,2]. The highest mortality usually occurs within
the first few hours after injury; almost 90% of patients
die at the scene of the accident and approximately
one third of patients who arrive at the hospital die
before surgical treatment [3,4].
The first references about aortic rupture date back
to Vesalius in 1557, after falling from a horse. Nowadays traumatic aortic rupture is the second most common cause of trauma-related deaths, leading to 8000
deaths per year in USA [5].
The majority of tears or ruptures occur at the aortic
isthmus; at this site, the relatively mobile thoracic
aorta joins the fixed arch and the insertion of the
ligamentum arteriosus. In 1947, Strassman reported in
a cohort of 7000 autopsies only 0.7% of patients with
traumatic aortic rupture. But several recent investigations have shown that traumatic aortic rupture occurs
in 22% of fatal blunt trauma [1,6]. In the era of highspeed motor vehicles, there has been an increased
*Corresponding Author:
Luca Di Marco, MD, PhD
Cardiac Surgery Department
S. Orsola-Malpighi Hospital
University of Bologna
Bologna, Italy
Tel: ⫹390516363361, Fax: ⫹39051345990, E-Mail: [email protected]
118
incidence of traumatic aortic injuries. In fact, injury is
often associated with rapid deceleration in road traffic
accidents or falls. The use of seat belts has partially
modified the characteristics of the trauma impact that
leads to aortic injury. However, air bags and seatbelts
do not protect against this type of impact. On the
other hand, the frequency of lethal injuries in head-on
collisions is lowered by the mandatory use of restraints, which protect the victim from thoracic and
head lesions, but not from the mechanism producing
aortic injury.
The diagnosis and management of traumatic thoracic aortic injuries have undergone some major
changes in the last few years. In fact, the replacement
of chest X-rays by routine Computed Tomography (CT)
scan for screening purposes in high-speed deceleration injuries has resulted in an earlier and more frequent diagnosis of traumatic aortic injuries. Angiography has largely been replaced by CT scan for the
definitive diagnosis of traumatic aortic ruptures.
Nowadays, angio CT-scan represents the gold standard for the diagnosis of traumatic aortic ruptures. It is
widely available in emergency departments and it
allows us to study the total body in a few seconds, also
highlighting minimal aortic lesion.
The best time to intervene in the aortic lesion and
whether surgery should be preceded or followed by
the treatment of associated traumatic lesions have
long been a matter of debate. Immediate surgery has
been characterized by a high mortality and morbidity
rate, ranging between 20 to 40%. In a retrospective
report of 144 patients undergoing surgery within an
average of six hours after arrival in hospital, there was
an intraoperative mortality of 10.2% and postoperative mortality of 18.4% with major postoperative morbidity such as paraplegia reaching 10.5% [7]. In a
recent multicenter trial involving 274 patients collected over 2.5 years, the overall mortality was 31%,
with 14% of operative mortality in stable patients
undergoing planned thoracotomy [8]. In light of this
very high risk for immediate surgical intervention, in
the past the surgical repair of an aortic rupture had
been delayed because of coexisting injuries such as
central nervous system trauma, severe respiratory insufficiency, extended body-burns sepsis, and contaminated open wounds, rendering the surgical risk too
high as reported by Akins in 1981 [9].
Because of the high morbidity and mortality, since
1992 we have delayed aortic repair in all patients who
Di Marco, L. et al.
State-of-the-Art Review
have arrived alive at the hospital unless signs of impending aortic rupture such as hemodynamic instability, massive hemothorax, and/or contrast media extravasation on CT-scan were present [10]. Several
studies have shown that delayed repair of traumatic
aortic injuries has significant survival benefits and a
much lower mortality rate compared with early open
repair [11]. In 2005 we reported an improvement of
patient outcome with traumatic rupture of the thoracic aorta by delaying surgical repair until after management of major associated injuries and in the absence of signs of impending rupture [12].
In light of this, in our institutions, for the management of traumatic aortic lesions, we routinely adopt
the algorithm reported in Figure 1. Surgery or endovascular treatment is delayed in the case of stable
patients, while immediate surgical or endovascular
repair is reserved for unstable patients with signs of
impending rupture.
It is clear that despite developments in operative
techniques, considerable operative mortality and morbidity associated with a surgical approach still remain,
even if delayed. Endovascular stent grafting of the
thoracic aorta provides an exciting new alternative to
the conventional approach for treating traumatic aortic rupture and it is emerging as the preferred technique for elective or emergent treatment of descending thoracic aortic lesions. It is a less invasive approach
compared with open surgery and it is preferable for
stabilization of the aortic lesion in patients with multiple traumatic injuries. Because of the lower invasiveness, avoiding thoracotomy and the use of heparin,
endovascular repair can be applied in the acute patients without the risk of destabilizing pulmonary,
head, or abdominal traumatic lesions. In early clinical
series, endovascular treatment demonstrated lower
morbidity and mortality in comparison with open surgical repair even in high-risk patients [13]. At present,
several reports in the literature have provided data on
comparative results of endovascular therapy with respect to open surgery, supporting the use of stent
graft in traumatic aortic injuries, both in acute and
chronic cases. In a 2008 meta-analysis, Xenos showed
that endovascular treatment of descending thoracic
aortic trauma is a valid alternative to open surgical
repair. It is associated with lower postoperative mortality and ischemic spinal cord complication rates [14].
In 2008 Demetriades, in a multicenter study of the
American Association for the surgery of Trauma re-
Acute Traumatic Thoracic Aortic Injury
State-of-the-Art Review
119
Traumatic aortic lesion
Monitoring and intensive medical treatment
( β -blockers + vasodilators )
UNSTABLE
STABLE
Delayed Surgery
Immediate Surgery
Endovascular Stent Graft
or
surgery
Figure 1.
Bologna’s strategy in case of traumatic aortic rupture.
ported a considerable reduced mortality rate in the
endovascular repair group as compared with open
surgical repair [15].
Even if the majority of traumatic injuries are stable
lesions, in approximately 5% of them, the risk of rupture may be high in the acute phase. Signs of impending rupture such as uncontrolled blood pressure, contrast medium extravasation on CT-scan, repeated
hemothorax, periaortic hematoma, or irregular lesions
are considered signs of instability [16].
Even for endovascular treatment in the acute phase
we deal with the same question: emergency or delayed treatment? Sometimes the aortic tear, acting
with a valve mechanism, may cause a pseudocoarctation syndrome producing a reduction of flow
in the descending aorta with lower extremity ischemia. This complication represents an emergency, accounting for 10% of victims. In these unstable patients, endovascular techniques offer a suitable
alternative to emergency open repair, even if some
limitations of the endovascular procedures exist such
as possible facial bone trauma which contraindicates
transesophageal echo or frequent aortic wall intramural hematoma with consequent high risk of stent-graft
migration.
Because endovascular treatment requires some peculiar anatomic conditions, not all the patients can be
treated. Proper peripheral vascular access (at least 7– 8
mm of diameter of femoral or iliac artery) is necessary,
but this condition is not always available, especially in
Aorta, July 2013
young patients. One of the most important anatomic
characteristics of any lesion allowing endovascular
treatment is the presence of an adequate proximal
neck (at least 5 mm of non-diseased aortic wall from
the origin of the subclavian artery). The aortic isthmus
is usually very close to the left subclavian artery and
sometimes the lesion is in contiguity with, or at a
limited distance from, the origin of the vessel. Stent
grafting of the descending thoracic aorta ideally requires
a proximal landing zone distal to the origin of the left
subclavian artery and proximal to the visceral arteries.
Several studies have reported the artificial creation
of a landing zone by covering the left subclavian
artery with the stent graft, with or without previous
subclavian to carotid transposition or bypass grafting,
increasing the risk of ischemic complications such as
stroke, left arm and spinal cord ischemia, or cerebellar
infarction [17–19]. Revascularization of the left subclavian artery with transposition or bypass from the carotid artery has been shown to prevent these complications.
Sepehripour in a meta-analysis of 2011 showed that
when coverage of the left subclavian artery is anatomically necessary, partial coverage is better than complete coverage in order to avoid these complications.
Revascularization may be considered, but these decisions should consider the individual patient scenario
[20].
But is the endovascular treatment really safe, efficacious, and free from complications in the long term?
Volume 1, Issue 2: 117–122
120
In general, despite the fact that there is a good
deal of convincing evidence for endovascular treatment for thoracic aorta diseases and for traumatic
aortic injuries as a valid and effective alternative to
surgery, several reports show a variety of late complications, especially for first-generation thoracic
endografts [21].
Both short- and midterm outcomes after endografting thoracic aortic lesions are encouraging,
with significantly lower morbidity and early mortality compared with open surgery. However, despite
emerging popularity and growing interest as an
alternative to surgery, endograft design and manufacturing have not kept pace with growing clinical
ambition. Major challenges associated with endovascular procedures using the current generation of
endografts range from the relative rigidity and size
of the delivery system to the failure of thoracic
endografts to conform snugly with the anatomy of
the aortic arch [22].
Various structural and positional changes in older
first-generation endografts have been reported. The
time-related changes in shape, physical structure and
position of the stent-grafts have, as a consequence,
secondary endoleaks, graft thrombosis, aneurysm rupture, and reperfusion from collaterals [23]. The aorta of
patients with traumatic injuries differs from atherosclerotic diseased vessels for which these grafts were
designed. Usually these patients are younger and have
normal, smaller proximal aortic diameters, such that
grafts are oversized by 10% to 20%. Since smaller
endografts are not available, large mismatches between the diameter of the aorta and the endograft
may occur, thus increasing the risk for endograft collapse. In case of bigger oversizing, the endograft undergoes significant compression forces and torque at
the proximal descending aortic angle. Excessive oversizing, especially if by greater than 25%, may cause
wrinkling of the stent graft and make it subject to
collapse [24]. The collapse may occur within 24
hours after stent implantation or after some
months. Graft collapse may cause total aortic closure and distal malperfusion [25–27].
Usually, it is common to use more than one component to treat a thoracic aortic aneurysm, which
causes some risk of device separation as the aortic wall
remodels, especially if there is insufficient component
overlap or coupling [28]. As a consequence, type I and
type III endoleaks are more likely to occur due to
Di Marco, L. et al.
State-of-the-Art Review
challenging seal zones and device migration increasing the likelihood of developing systemic pressures
within the aneurysm sac.
Physiological coarctation of the aorta from protrusion of a thoracic stent graft into the arch is a complication of thoracic stent grafting distinct from the
more commonly described graft collapse. The protrusion of the stent graft into the arch causes the obstruction of the aorta. Patients may present with
symptoms of aortic arch coarctation such as proximal
hypertension, left upper extremity ischemia, or left
carotid or vertebral insufficiency despite having a patent endograft [29]. Perforation of the aortic wall by a
stent graft is an infrequent complication of thoracic
stent-graft implantation. This complication is usually
due to the metallic component of the stent causing
friction against the aortic wall because of continuous
pulsatility of the aorta. This complication underlines
the importance of completely examining the longterm durability and compatibility of prosthetic materials [30].
Aortoesophageal fistula secondary to thoracic aortic stent-graft placement is an unusual but catastrophic complication of endovascular repair of the
thoracic aorta with very limited therapeutic options.
The fistulae may arise secondary to the development
of pseudoaneurysm, endoleak into the residual aneurysm sac, or erosion of the stent graft through the
aorta from graft infection [31–34].
In light of the above and in consideration of the
complications which endoprostheses may undergo,
continuous and meticulous follow-up of these devices
with regular imaging is advised [35].
It is clear that frequent and sustained surveillance is
essential for safe management of patients. The primary motivation for close surveillance includes the
evaluation of residual aneurysm sac size, presence of
endoleak, and device migration allowing an early
identification of potential adverse events. In fact a
reintervention rate of 10% per year has been reported
for treatment of problems identified on follow-up surveillance [36].
The CT-scan is usually the method of choice for
periodic assessments during follow-up protocols after
endovascular aneurysm repair (EVAR). The routine use
of contrast-enhanced CT-scans has become more controversial since repeated scans with their inherent
ionizing radiation have been suggested to have carcinogenic potential. This evidence suggests that less-
Acute Traumatic Thoracic Aortic Injury
State-of-the-Art Review
121
frequent CT scans may simplify the follow up protocol,
reduce radiation exposure and the total cost of EVAR
[37,38].
In spite of the fact that endovascular techniques can
now be considered an effective alternative to open surgery in the treatment of traumatic thoracic aortic injuries,
the long-term durability of a stent graft for traumatic
aortic lesions is still unknown. Techniques and technologies continue to improve and the results obtained
should be viewed as work in progress.
Early outcomes appear successful, but these results
may be deceiving especially for those patients with
compromised anatomy and the risk of late stent-graft
migration, loss of device integrity and local erosion or
rupture of the aortic wall. At present, the lack of
long-term data and the evolving technology of stentgraft design should be an incentive for exercising
great care in patient selection [39,40].
Comment on this Article or Ask a Question
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10.1056/NEJMra072149
Acute Traumatic Thoracic Aortic Injury
Case Report
Aorta, July 2013, Volume 1, Issue 2: 123–125
DOI: http://dx.doi.org/10.12945/j.aorta.2013.12.012
Received: December 28, 2012
Accepted: February 15, 2013
Published online: July 2013
Dissection of Iliac Artery in a Patient With
Autosomal Dominant Polycystic Kidney Disease
A Case Report
Audrey Courtois, PhD1*, Betty V. Nusgens, PhD1, Philippe Delvenne, MD, PhD2,
Michel Meurisse, MD, PhD3, Jean-Olivier Defraigne, MD, PhD4, Alain C. Colige, PhD1,
Natzi Sakalihasan, MD, PhD4
1
Laboratory of Connective Tissues Biology, GIGA, University of Liège, Sart-Tilman, Belgium; 2Department of Anatomopathology, CHU
Liège, University of Liège, Liège, Belgium; 3Department of Abdominal, Endocrine and Transplantation Surgery, CHU Liège, University of
Liège, Liège, Belgium; 4Department of Cardiovascular and Thoracic Surgery, CHU Liège, University of Liège, Liège, Belgium
Abstract
Autosomal dominant polycystic kidney disease
(ADPKD) is a risk factor for several cardiovascular
disorders such as intracranial aneurysm or aortic dissection, preferentially occurring at the thoracic or
abdominal level. A 47-year-old man suffering from
ADPKD had renal transplantation. Sixteen hours after
surgery, he presented with left leg pain. Clinical and
ultrasound examination revealed thrombosis of the
external left iliac artery. Therefore, we decided to
perform intra-arterial angiography to evaluate the
possibility of an endovascular treatment. Aortofemorography showed an obstruction of the external
left iliac artery that was found during emergency
surgery, consecutive to a dissection, which occurred
following the surgery for kidney transplantation. The
resected segment of the dissected vessel was analyzed by histology. Collagen fibers organization and
density in the adventitia and smooth muscle cells
density in the media were similar in the dissected and
a normal artery from a healthy donor. By contrast, an
almost complete disappearance and fragmentation
of elastic lamellae were observed in the media of
the dissected artery, most likely responsible for the
weakening of the arterial wall and its dissection.
Association between ADPKD and single dissection of
the iliac artery has been rarely reported. Relationship
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E-Mail: [email protected]
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© 2013 Aorta.
Published by Science International Corp.
ISSN 2325-4637
Accessible online at:
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between inactivation of polycystin/PKD genes and elastic fibers degradation through elevated TGF␤ signaling
and matrix metalloproteinase 2 (MMP2) elastolytic activity, as recently reported in ADPKD, would be worth
investigating.
Copyright © 2013 Science International Corp.
Key Words
Autosomal dominant polycystic kidney disease · Arterial dissection · Elastic lamellae
Introduction
Autosomal dominant polycystic kidney disease
(ADPKD) is the most frequent genetic renal disorder
with an incidence of 1/1000. It is characterized by the
progressive formation of fluid-filled cysts in the kidney
leading to early onset renal failure. Several cardiovascular disorders have been associated with ADPKD,
hypertension being the most common problem [1],
while other major complications include intracranial
and aortic aneurysms and dissections (for review, see
[2]). Here, we report a case of left iliac artery dissection
in a patient with ADPKD.
*Corresponding author:
Audrey Courtois, MSc, PhD Student
Laboratory of Connective Tissues Biology, GIGA
University of Liège
Tour de Pathologie, B23/⫹3, B-4000
Sart-Tilman, Belgium
Tel: ⫹3243662459, Fax: ⫹3243662457, E-Mail: [email protected]
124
Figure 1. Imaging of the ADPKD patient. (A) CT-scan showing
the polycystic kidney (arrow) and the transplanted kidney
(arrowhead). (B) and (C) Aorto-femorography showing the occlusion of the left external iliac artery (arrow).
Case Report
Case Report
Figure 2. General organization of the wall of the dissected
iliac artery. (A) and (B): hematoxylin/eosin staining. (C) and (D):
Masson trichrome staining. (E) and (F): ␣-smooth muscle actin
immunohistochemistry performed on a healthy iliac artery (A,
C, E) and the dissected segment of the iliac artery of the ADPKD
patient (B, D, F). Bar ⫽ 100 ␮m.
A 47-year-old man presenting with familial polycystic kidney disease (ADPKD) underwent surgery for
kidney transplantation. During surgery, any macroscopic anomalies were observed at the level of close
iliac artery. However, approximately 16 hours after
surgery, the patient presented pain at the level of the
left leg. Clinical examination revealed absence of left
femoral pulses. Ultrasound examination confirmed
thrombosis of the aorto-iliac access. Computed tomography (CT) angiography revealed the absence of
perfusion at the level of the transplanted kidney and
confirmed the thrombosis of the left iliac artery (Fig.
1A). Therefore, intra-arterial angiography was performed to decide which treatment could be performed. An endovascular treatment was not possible
because of the complete thrombosis of the external
left iliac artery with suspected dissection (Fig. 1B and
1C). The patient underwent emergency surgery, during which a localized hematoma was observed at the
level of the arterial anastomosis with extension to the
external left iliac artery. The diagnosis of dissection
was made after reopening of the anastomosis. Be-
cause of the fragile aspect of the arterial tissues, we
performed a large resection of the dissected segment
of the iliac artery that was replaced by an arterial
prosthesis. The donor renal artery was reimplanted on
the prosthesis. Pieces of the dissected segment were
fixed for histological analysis. This study was approved
by the hospital-university ethics committee.
The general organization of the iliac artery was
analyzed by hematoxylin/eosin (H/E; Fig. 2A and 2B)
while a Masson trichrome staining allowed to evaluate
the fibrillar collagen framework (Fig. 2C and 2D) and
an immunostaining with anti-␣-smooth muscle actin
(␣-SMA) showed smooth muscle cells (Fig. 2E and 2F).
The H/E staining clearly showed a large blood infiltrate
between the adventitia and the media (Fig. 2B). As
compared to a healthy iliac artery, no difference was
observed in the collagen framework or in the smooth
muscle cells organization at the level of the dissected
wall. However, an orcein staining showed a striking
rarefaction and disruption of the elastic lamellae in the
media of the dissected iliac artery (Fig. 3B and at
Courtois, A. et al.
Iliac Artery Dissection and ADPKD
Case Report
125
Figure 3. Visualization of elastic fibers by orcein staining of a
healthy iliac artery (A) and (C) and the dissected segment of the
iliac artery of the ADPKD patient (B) and (D). Bar ⫽ 100 ␮m in
(A) and (B) and 25 ␮m in (C) and (D).
higher magnification Fig. 3D) as compared to a
healthy artery (Fig. 3A and 3C). The dissection occurred at the junction of the external elastic lamella
and the media. No inflammatory cells were found in
the media or in the adventitia.
Discussion
Spontaneous dissection of the iliac artery is an
extremely rare event and may occur as a complication
of traumatic injuries or systemic disorders such as
Marfan syndrome and ␣-1 antitrypsin deficiency [3,4].
ADPKD has been associated with a large number of
cardiovascular disorders. Intracranial aneurysms occur
with a 10% incidence in ADPKD patients [5]. Aortic
dissection, which usually occurs at the thoracic level, is
a rare complication of ADPKD. In this report, we describe the first case of a spontaneous iliac artery dissection associated with ADPKD without any other apparently affected blood vessel. This genetic disease is
caused by mutations in the PKD1 or PKD2 genes
encoding for, respectively, polycystin 1 and 2. These
proteins are expressed by vascular smooth muscle cells
(VSMC) and are involved in Ca2⫹ homeostasis and in cell
interactions with their surrounding extracellular matrix
that are critical in maintaining the integrity of the media
and regulating the VSMC phenotype [2,6]. The density of
VSMC and the expression of ␣-SMA in the media of the
dissected wall appeared both normal, which suggests
that elastic fibers rarefaction is not related to VSMC
apoptosis. Mutations in PKD genes could, however, result in defective interactions between VSMC and the
surrounding matrix, which, in turn, would lead to modification of the VSMC pattern of protein expression and
to degradation of the elastic tissue. Recently, elevated
TGF␤ signaling was observed in an advanced stage of
ADPKD, coinciding with increased levels of target genes
of the TGF␤ pathway such as matrix metalloproteinase 2
[7], known for its elastolytic activity and its activation in
abdominal aortic aneurysms [8].
In this case report, we observed a potential association between ADPKD and iliac dissection that would
need to be validated on a large series of patients.
Comment on this Article or Ask a Question
References
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1016/S0140-6736(94)92513-5
Aorta, July 2013
5. Chapman AB, Rubinstein D, Hughes R,
Stears JC, Earnest MP, Johnson AM, et al.
Intracranial aneurysms in autosomal dominant polycystic kidney disease. N Engl J
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6. Griffin MD, Torres VE, Grande JP, Kumar R.
Vascular expression of polycystin. J Am Soc
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7. Hassane S, Leonhard WN, van der Wal A,
Hawinkels LJ, Lantinga-van Leeuwen IS, ten
Dijke P, et al. Elevated TGFbeta-Smad signalling in experimental Pkd1 models and human
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Pathol. 2010;222:21–31. 10.1002/path.2734
8. Sakalihasan N, Delvenne P, Nusgens BV, Limet R, Lapiere CM. Activated forms of
MMP2 and MMP9 in abdominal aortic aneurysms. J Vasc Surg. 1996;24:127–133. 10.1016/
S0741-5214(96)70153-2
Cite this article as: Courtois A, Nusgens
BV, Delvenne P, Meurisse M, Defraigne
J-O, Colige AC, Sakalihasan N. Dissection
of Iliac Artery in a Patient With Autosomal
Dominant Polycystic Kidney Disease: A Case
Report. Aorta 2013;1(2):123–125. DOI: http://
dx.doi.org/j.aorta.2013.12.012
Volume 1, Issue 2: 123–125
Case Report
Aorta, July 2013, Volume 1, Issue 2: 126 –130
DOI: http://dx.doi.org/10.12945/j.aorta.2013.12.008
Received: November 10, 2012
Accepted: March 9, 2013
Published online: July 2013
Simultaneous Surgical Treatment of Type B
Dissection Complicated With Visceral
Malperfusion and Abdominal Aortic Aneurysm
Role of Aortic Fenestration
Gianfranco Filippone, MD1*, Gabriele Ferro, MD1, Cristiana Duranti, MD2,
Gaetano La Barbera, MD1, Francesco Talarico, MD1
1
Division of Vascular and Endovascular Surgery, Palermo, Italy; 2Section of Radiology, Ospedale Civico e Benfratelli, Palermo, Italy
Abstract
Introduction
Aortic dissection occurs in about 5% of patients with
coexistent abdominal aortic aneurysm (AAA); combined
type B dissection complicated with visceral malperfusion and AAA is an uncommon aortic emergency and
patients presenting with complications of thoracic aortic
dissection have a dismal prognosis related to difficulties
in treatment strategies. Despite tremendous improvement of endovascular techniques, surgical aortic fenestration represents a quick, safe, and effective procedure able
to restore flow in an otherwise malperfused aorta. This
procedure has to be kept in mind because subsets of
patients cannot be treated conventionally due to either
prohibitive risk of aortic replacement, anatomic contraindication, or limitations of percutaneous procedures.
Herein we report a case of a patient presenting with type
B aortic dissection complicated by visceral malperfusion
and AAA which was successfully treated simultaneously
by open AAA repair and surgical fenestration. We focus
on the mechanism of malperfusion and on the role of
surgical fenestration.
Copyright © 2013 Science International Corp.
Key Words
Aortic dissection · Visceral malperfusion · Fenestration
Fax ⫹1 203 785 3346
E-Mail: [email protected]
http://aorta.scienceinternational.org
© 2013 Aorta.
Published by Science International Corp.
ISSN 2325-4637
Accessible online at:
http://aorta.scienceinternational.org
Aortic dissection occurs in 5% of patients with
coexistent abdominal aortic aneurysm (AAA) increasing the risk for aortic rupture [1]; about 30% of acute
type B dissections present life- threatening complications with a dismal prognosis due to high mortality
caused by this catastrophic aortic emergency [2].
Moreover, association between AAA and type B dissection complicated by visceral malperfusion represents a clinical rarity but presents a challenge in both
diagnosis and therapeutic strategies [3].
Herein we report a case of a patient presenting
with type B aortic dissection complicated by visceral
malperfusion and concomitant AAA which was successfully treated simultaneously by open AAA repair and
surgical fenestration. We focus on the mechanism of
malperfusion and on the role of surgical fenestration.
A 67-year-old hypertensive man experienced an
acute type A dissection and was operated on one year
earlier with a Bentall procedure and subtotal arch
replacement using two grafts. He was admitted to our
hospital complaining of persistent abdominal pain
without peripheral pulse deficit; laboratory findings
showed abnormal liver function test results and lactate elevation. Aware of the clinical history, a com*Corresponding author:
Gianfranco Filippone, MD
Division of Cardiac Surgery
University of Palermo
Via Giusti,3 90144
Palermo, Italy
Tel: ⫹39 091 6552687, Fax: ⫹39 091 6664831, E-Mail: g.fi[email protected]
Case Report
127
(A) Preoperative CT scan demonstrated extension of the dissection from distal arch to proximal neck of abdominal
aortic aneurysm; (B) CT image showing true lumen compression of celiac trunk; (C) 3Mensio CT image demonstrating visceral
malperfusion due to the false lumen being overpressurized.
Figure 1.
puted tomography (CT) scan was proposed. The scan
showed the two grafts used for extended aortic replacement and a type B dissection with an overpressurized false lumen originating below the subclavian
artery (Fig. 1A), compressing a very thin true lumen
with subocclusion of the celiac trunk and mesenteric
artery (Fig. 1B). The spiralized cul de sac of the dissection stopped at the origin of an infrarenal, 5-cm abdominal aortic aneurysm (Fig. 1C).
The CT images were reconstructed with the 3Mensio medical imaging software program for a better
visualization of the malperfusion mechanism, and the
Aorta, July 2013
patient was prepared for an emergent open AAA repair and surgical fenestration.
The abdomen was penetrated through a midline
laparotomy and gross inspection showed a dark colored, bruised and pulseless long segment of the small
bowel (Fig. 2A). The aorta was cross clamped below
the left renal vein, and upon opening the AAA, the cul
de sac of dissection with a large false lumen and a
virtual true lumen was identified just above the AAA
proximal neck (Fig. 2B). Fenestration of the intimal flap
was performed, excising as much intima as possible in
both a circumferential and longitudinal extent toward
Volume 1, Issue 2: 126 –130
128
Case Report
(A) Intraoperative view showing ischemic small bowel; (B) intraoperative view showing forcep opening true lumen of
the dissected aorta; (C) incision of the intimal flap; (D) recovery of the small bowel after fenestration and AAA repair.
Figure 2.
the proximal clamp (Fig. 2C). At the end of the procedure, a 20mm straight woven Dacron graft was sutured to the proximal aortic stump. Distal anastomosis
above the aortic bifurcation completed the operation.
Inspection of the small bowel revealed rosy colored
loops that, except for a very short segment, was otherwise viable; with recovery of vitality there was no
need for resection (Fig. 2D).
The postoperative course was uneventful; a continuous decrease in biomarkers of end-organ ischemia
was observed and after completion of a CT scan control (Fig. 3A and 3B) the patient was discharged to
home two weeks later. At one year follow up, the
patient is doing well and CT control shows a stable
diameter at the top of the descending aorta.
As reported by Cambria, occurrence of acute dissection in an aorta previously afflicted with atherosclerotic aneurysm is unusual. In a series of 325 patients
with aortic dissection, he identified only 5% of them
with coexistent aneurismal disease. He pointed out
that this association appears to increase the risk of
aortic rupture in both the proximal and distal aorta,
also indicating that the presence of a juxtaposed
atherosclerotic aneurysm greater than 5 cm constitutes a “complicated” dissection and standard antihypertensive therapy fails to prevent aortic rupture.
The fate of the false lumen following primary repair
of an aortic dissection influences the outcome of the
Filippone, G. et al.
patient; remaining patent or partially thrombosed, it
may be a source of complications, like for our patient
where a life-saving operation converted a type A into
a type B dissection.
Type B aortic dissection is generally treated with
medical therapy when uncomplicated, but about 30%
of cases at clinical presentation are complicated either
by hemodynamic instability or by vascular ischemia
with high risk of mortality if untreated [4].
The association between AAA and type B aortic
dissection complicated with visceral malperfusion represents a clinical rarity but presents a challenge in
both diagnosis and operative indications. The majority
of patients with complicated type B dissection have a
spiral aorta with collapse of the true lumen. In our
patient the dissection progressed downward trough
the posterolateral wall of the descending thoracic
aorta and then spiraled anteriorly, ending at the level
of the origin of the abdominal atherosclerotic aneurysm.
The role of atherosclerotic plaque in the natural
history of aortic dissection is uncertain. The analysis
made by Roberts suggests that atherosclerotic plaque
frequently serves to terminate the dissection process
but the situation is quite different when atherosclerotic or degenerative aneurysm is present. In such a
circumstance, rupture of the aneurysm is the more
likely scenario.
Role of Aortic Fenestration
Case Report
129
Figure 3. (A) postoperative CT scan showing reexpansion of the true lumen; (B) 3Mensio CT scan demonstrating blood flow
restored into visceral vessels and the AAA graft replacement.
The incidence of aortic branch vessel involvement
in aortic dissection ranges from 25% to 50% [5]; expansion of the false lumen at the expense of the true
channel is the most common mechanism of vascular
obstruction. Absence of a distal re-entry site in the
dissected aorta or its branches may jeopardize blood
flow in the true channel to the point of total occlusion,
leading to secondary distal thrombosis inside the aortic branch vessel.
According to evolving CT scan criteria, two types of
ischemia mechanisms are depicted: aortic type and
branch type. In the first one there is collapse of the true
lumen inside the aorta while in the latter the dissection
flap narrows the true lumen of a visceral artery [2].
Despite tremendous improvement in surgical and
endovascular techniques, some patients cannot be
treated either conventionally due to the prohibitive
risk of an open procedure, or percutaneously due to
anatomic contraindications or limitations of catheterbased interventions. A percutaneous procedure may
consume time in a patient that needs quick intervention due to impending bowel infarction [7]. These
Aorta, July 2013
circumstances associated with the presence of the
AAA that is a complication “per se” led us to perform
surgical fenestration to restore flow in the true lumen
and AAA repair at the same time.
The fenestration technique was first described in
1935, but the largest series have been reported by the
Harvard and Yale groups [5,6]. Once control of the
aorta is achieved, the vessel is crossclamped, usually
below the renal arteries, and transected. The intimal flap
is identified and a portion of the dissecting membrane is
removed, sliding the scissors up to the level of the
proximal clamp. In conclusion, the aim of the procedure
is to form a re-entry point to decompress the false lumen
and equipoise pressure in both channels of the aorta,
permitting a re-expansion of the true lumen.
The interval between the appearance of complications and surgical treatment is related to the poor
prognosis of complicated type B dissection. Surgical
fenestration as previously described extraperitoneally,
or in our case transperitoneally, in association with AAA
repair can be performed quickly without specialized endovascular or imaging equipment.
Volume 1, Issue 2: 126 –130
130
Case Report
Experience with short- and long-term outcomes following fenestration is scant, however, both the Yale and
Harvard series demonstrated a three-year and five-year
survival rate of 77% and 55%, respectively, with an almost 100% reperfusion rate. Failure of successful reperfusion was noted only in patients with a delay in the
diagnosis of more than 48 hours after onset of dissection. It is of interest to note that no late aneurysmal
development was noted in the survivors [5,6,8].
Combined type B aortic dissection complicated by
visceral malperfusion and AAA represents an uncommon aortic emergency. Despite tremendous improvement of endovascular techniques, this challenging disease still carries high mortality mainly due to
difficulties relieving visceral ischemia. Surgical fenestration represents a safe, quick, and effective procedure; flow is restored above and below the site of
operation. The best results are achieved when it is
performed immediately on presentation with organ
ischemia. In conclusion, this technique should be kept
in the surgeon’s repertoire because subsets of patients
cannot be treated conventionally due to prohibitive
risk of thoracic aortic replacement and/or anatomic
contraindications or limitations of percutaneous procedures.
Comment on this Article or Ask a Question
References
1. Cambria RP, Brewster DC, Moncure AC, Steinberg FL, Abbot WM. Spontaneous aortic dissection in the presence of coexistent or previously repaired atherosclerotic aortic
aneurysm. Ann Surg. 1988;208:619 –624.
2. Uchida N, Shibamura H, Katayama A, Aishin K,
Sutoh M, Kuraoka M. Surgical strategies for
organ malperfusions in acute type B aortic
dissection. Interac Cardiovasc Thorac Surg.
2009;8:7678. 10.1510/icvts.2008.186247
3. Roberts CS, Roberts WC. Combined thoracic
aortic dissection and abdominal aortic fusiform aneurysm. Ann Thorac Surg. 1991;52:
537–540. 10.1016/0003– 4975(91)90920-L
4. Fattori R, Botta L, Lovato L, Biagini E, Russo V,
Casadei A, et al. Malperfusion syndrome in
type B dissection: role of the endovascular
Filippone, G. et al.
procedures. Acta Chir Belg. 2008;108:192–
197.
5. Panneton JM, The SH, Cherry KJ, Hofer JM,
Gloviczki P, Andrews JC, et al. Aortic fenestration for acute or chronic aortic dissection: An
uncommon but effective procedure. J Vasc
Surg. 2000;32:711–727. 10.1067/mva.2000
6. Pradhan S, Elefteriades JA, Sumpio BE. Utility of the aortic fenestration technique in
the management of acute aortic dissections. Ann Thorac Cardiovasc Surg. 2007;13:
296 –300.
7. Hartnell GG, Gates J. Aortic fenestration: A
why, when and how to guide. Radiographics.
2005;25:175–189. 10.1148/rg.251045078
8. Trimarchi S, Jonker FHW, Muhs BE, Grassi V,
Righini P, Upchurch GR, et al. Long- term
outcomes of surgical fenestration for complicated acute type B aortic dissection. J Vasc
Surg. 2010;52:261–266. 10.1016/j.jvs.2010.02.
292
Cite this article as: Filippone G, Ferro G,
Duranti C, La Barbera G, Talarico F. Simultaneous Surgical Treatment of Type
B Dissection Complicated With Visceral
Malperfusion and Abdominal Aortic Aneurysm: Role of Aortic Fenestration.
Aorta 2013;1(2):126 –130. DOI: http://dx.
doi.org/j.aorta.2013.12.008
Role of Aortic Fenestration
Case Report
Aorta, July 2013, Volume 1, Issue 2: 131–134
DOI: http://dx.doi.org/10.12945/j.aorta.2013.13-011
Received: February 14, 2013
Accepted: May 10, 2013
Published online: July 2013
An Unusual Complication of Surgery for Type A
Dissection Treated by Thoracic Endovascular
Aortic Repair
Giuseppe Petrilli, MD1*, Giovanni Puppini, MD2, Salvo Torre, MD1, Daniele Calzaferri, MD1,
Antonella Bugana, MD1, Giuseppe Faggian1
1
Division of Cardiac Surgery, University of Verona, Verona, Italy; 2Department of Radiology, University of Verona, Verona, Italy
Abstract
A 58-year-old man was admitted to our hospital for
massive swelling in an anterior cervical location. Nine
years earlier, he underwent surgical repair of a complex
type A aortic dissection. This procedure was complicated
by a fistula between the anastomosis of the graft and the
descending aorta, resulting in massive presternal swelling. Therefore, we performed thoracic endovascular repair with successful sealing of the prosthetic leak, achieving progressive reduction in the collection of fluid. We
propose thoracic endovascular aortic repair as an alternative to open surgical repair for the treatment of complicated cases.
Copyright © 2013 Science International Corp.
Key Words
Emergency TEVAR · Periprosthetic leak · Presternal
swelling
Introduction
Open thoracic or thoracoabdominal aortic repair
carries a significant risk of mortality and morbidity,
despite recent literature suggesting a significant improvement [1]. Thoracic endovascular aortic repair
(TEVAR) offers the possibility of treating patients who
are not candidates, or those who are at extremely
high risk for conventional surgical procedures because of their existing comorbidities and has gained
increased acceptance across the world, with excellent
Fax ⫹1 203 785 3346
E-Mail: [email protected]
http://aorta.scienceinternational.org
© 2013 Aorta.
Published by Science International Corp.
ISSN 2325-4637
Accessible online at:
http://aorta.scienceinternational.org
short-term results [2]. Utilizing this therapeutic strategy, we report our successful management of a patient who suffered complications after a cardiovascular operation.
Clinical Case
In 2003, a 58-year-old man was admitted to another institution for a type B aortic dissection treated
with medical management. After two days, the patient underwent surgical repair because of retrograde
intramural hematoma extending to the ascending
aorta as shown by computed tomography (CT). In the
theater a 26 mm vascular prosthesis was placed extending from the sinotubular junction (STJ) to the
aortic arch immediately distal to the origin of left
subclavian artery, with direct reimplantion of the epiaortic vessels. This graft was connected to the descending aorta with another 20 mm vascular prosthesis, anastomosed end-to-side from the neo-ascending aorta to
the descending thoracic aorta. This procedure was performed at another surgical institution and is comparable
to a technique described by Griepp et al. [3].
Circulatory arrest duration was two hours with
anterograde cerebral perfusion from the right axillary
artery. This procedure was complicated by right hemiplegia, probably due to cerebral malperfusion. CT of
the brain demonstrated extensive bilateral ischemic
*Corresponding author:
Giuseppe Petrilli, MD
Azienda Ospedaliera Universitaria
Div. Cardiochirurgia
Piazzale Stefani 1 - 37100
Verona, Italy
Tel: ⫹393471161792, Fax: ⫹390458123308, E-Mail: [email protected]
132
Case Report
CT shows the periprosthetic fistula, as indicated by
the red arrow.
Figure 1.
Figure 2.
cerebral lesions and evoked potentials showed a left
decortication pattern.
Last February, the patient was admitted to our hospital due to the appearance of massive presternal swelling. The patient had a medical history that included
hypertension and peripheral vascular disease. He was
hemiplegic with cognitive impairment. A CT scan
showed the presence of a fistula from the end-to-side
anastomosis, connecting the graft to the descending
aorta (Fig. 1). The lesion resulted in a voluminous collection of fluid extending from the periaortic to the subcutaneous presternal regions (Latero-lateral (LL) ⫻ Craniocaudal (CC) ⫻ Postero-anterior (PA) ⫽ 8 cm ⫻ 10 cm ⫻
7 cm), crossing the diastasis of the manubrium (Fig. 2).
Therefore, the patient was transferred to the operating room where endovascular repair was performed
under general anesthesia. A standard cut-down over
the left femoral artery was performed and percutaneous right axillary access was achieved. Systemic heparin (100 mg) was then administered. A 28/200/24 mm
Relay plus (Bolton Medical, Barcelona, Spain) stentgraft was advanced over a stiff wire and navigated in
the true lumen under fluoroscopic guidance. The
stent-graft was successfully placed from the previous
vascular graft to about halfway down the descending
thoracic aorta and the anastomosis originating the
leakage was excluded. At the end of the delivery there
Presternal swelling.
Petrilli, G. et al.
Complication Treated by TEVAR
Case Report
133
was evidence of minimum type 1 endoleak, sealed by
postdilatation of the endoprosthesis with a 27 mm
balloon. Aortography demonstrated a satisfactory result. The patient’s condition improved and he was
discharged 5 days after surgery.
At one month postsurgery a 3D CT scan image
showed the successful sealing of the endoleak (Fig. 3)
and at three months, reduction of the hematoma (Fig. 4)
was evident.
Discussion
Figure 3.
Endoprosthesis in situ.
Figure 4.
Reduction of presternal swelling after three months.
Aorta, July 2013
Acute aortic dissection of the descending aorta is
still a life-threatening condition with high mortality
and morbidity. Currently, there is consensus that acute
uncomplicated type B dissections are managed medically. Conventional resection and graft replacement
of the descending thoracic aorta has been the preferred method of treatment only in cases with complications such as aortic rupture, malperfusion of end
organs, and/or persistent pain despite medical treatment. Open repair carries a 2.9% to 7% risk of paraplegia and an operative mortality rate ranging from
Volume 1, Issue 2: 131–134
134
15% to 23.5%. Nevertheless, the introduction of endovascular stent grafts is revolutionizing the definitive
treatment of these injuries. The potential benefits of
TEVAR over open repair include no need for thoracotomy or single lung ventilation, decreased use of systemic anticoagulation, avoidance of aortic crossclamping, less blood loss, less postoperative pain, and
a lower paraplegia rate [3]. Indeed, since its introduction more than a decade ago, TEVAR has shown promising results for patients with various thoracic aortic
diseases [4]. These include unstable acute type B aortic
dissection, chronic type B aortic dissection, and type B
dissection with retrograde extension into the ascending aorta [5]. The concept of this procedure was directed toward sealing the proximal intimal tear, redirecting the flow into the true lumen, and promoting
depressurization and thrombosis of the false lumen. In
addition, such an approach can effectively treat malp-
Case Report
erfusion syndrome by re-establishing side branch flow
in dynamic obstruction [2].
In accordance with this, we adopted TEVAR to repair
a dehiscence of an anastomosis between the transverse
prosthesis and the descending aorta. The patient’s condition has improved considerably and, after three
months, the severe subcutaneous and intrathoracic collection of fluid has been considerably reduced.
A surgical alternative would have resulted in a complex reoperation with circulatory arrest to repair the
leak, thus exposing the patient to the risk of new
complications, especially neurological.
In conclusion, TEVAR is a reliable and flexible
method that can be extended to complicated patients
where an open surgical option presents a prohibitive
risk of mortality.
Comment on this Article or Ask a Question
References
1. Coady MA, Ikonomidis JS, Cheung AT, Matsu- 3. Griepp B, EM: Aneurysms of aortic arch, in
Edmonds LH, Jr. (ed.): Cardiac Surgery in the
moto AH, Chaikof EL, et al. Surgical manageAdult. New York, McGraw-Hill, 1997; p.
ment of descending thoracic aortic disease:
1209.
open and endovascular approaches: a scientific statement from the American Heart As- 4. Akin I, Kische S, Ince H, Nienaber CA. Indication, timing and results of endovascular
sociation.Circulation.2010;121:2780 –2804.10.
treatment of type B dissection. Eur J Endo1161/CIR.0b013e3181e4d033
vasc Surg. 2009;37:289 –296. 10.1016/j.ejvs.
2. Ehrlich MP, Rousseau H, Heijmen R, Piquet P,
2008.12.004
Beregi JP, Nienaber CA, et al. Midterm results
after endovascular treatment of acute, com- 5. Cao CQ, Bannon PG, Shee R, Yan TD. Thoracic
endovascular aortic repair—indications and
plicated type B dissection: the Talent Thoracic
evidence. Ann Thorac Cardiovasc Surg. 2011;
Registry. J Thorac Cardiovasc Surg. 2012;145:
17:1–6. 10.5761/atcs.ra.10.01612
159 –165. 10.1016/j.jtcvs.2011.10.093
EDITOR’S COMMENTS
This case showcases just how dramatic such false
aneurysms can become, with a truly massive suprasternal pulsatile mass. This case would have been
difficult (although fully possible) to approach by open
Petrilli, G. et al.
Cite this article as: Petrilli G, Puppini G,
Torre S, Calzaferri D, Bugana A, Faggian
G. An Unusual Complication of Surgery
for Type A Dissection Treated by Thoracic Endovascular Aortic Repair. Aorta
2013;1(2):131–134.DOI:http://dx.doi.org/
10.12945/j.aorta.2013.13-011
surgery, making an endovascular approach attractive.
As the offending fistula appears to be a defect in a
graft-to-graft anastomosis, the chosen endovascular
stent grafting may indeed lead to permanent obliteration of the tract and the false aneurysm.
Complication Treated by TEVAR
Basic Science for the Clinician
Aorta, July 2013, Volume 1, Issue 2: 135–145
DOI: http://dx.doi.org/10.12945/j.aorta.2013.13-024
Received: May 10, 2013
Accepted: June 3, 2013
Published online: July 2013
Genes in Thoracic Aortic Aneurysms and
Dissections – Do they Matter?
Translation and Integration of Research and Modern Genetic
Techniques into Daily Clinical Practice
Julie De Backer, MD, PhD1,2*, Marjolijn Renard, PhD1, Laurence Campens, MD1,2,
Katrien François, MD, PhD3, Bert Callewaert, MD, PhD1, Paul Coucke, PhD1,
Anne De Paepe, MD, PhD1
1
Centre for Medical Genetics, University Hospital Ghent, Ghent, Belgium; 2Department of Cardiology, University Hospital Ghent, Ghent,
Belgium; and 3Department of Cardiovascular Surgery, University Hospital Ghent, Ghent, Belgium
Abstract
Introduction
Since the identification of the fibrillin-1 gene as the
causal gene for Marfan syndrome, our knowledge of
molecular genetics and the applicability of genetic testing in clinical practice have expanded dramatically.
Several new syndromes related to thoracic aortic aneurysms and dissections (TAAD) have been described and
the list of underlying genes in syndromal and nonsyndromal TAAD already includes more than 10 different
genes and is rapidly expanding. Based on this knowledge, our insights into the underlying pathophysiology
of TAAD have improved significantly, and new opportunities for targeted treatment have emerged. Clinicians
involved in the care of TAAD patients require a basic
knowledge of the disease entities and need to be informed on the applicability of genetic testing in their
patients and families. Gene-tailored treatment and management is indeed no science fiction anymore and should
now be considered as part of good clinical practice. We
provide a systematic overview of genetic TAAD entities
and practical recommendations for genetic testing and
patient management.
Copyright © 2013 Science International Corp.
Key Words
Thoracic aortic aneurysms and dissections · Molecular
genetic testing · Aneurysm syndrome
Fax ⫹1 203 785 3346
E-Mail: [email protected]
http://aorta.scienceinternational.org
© 2013 Aorta.
Published by Science International Corp.
ISSN 2325-4637
Accessible online at:
http://aorta.scienceinternational.org
Over the past decade, expanding knowledge of the
genetic basis of Thoracic Aortic Aneurysms and Dissections (TAAD) significantly improved our understanding of the pathogenesis of the disease and improved our ability for risk stratification and medical
guidance of patients and their families. Strategies for
molecular genetic testing have reached a hinge point
with the introduction in routine diagnostics of highthroughput next generation sequencing techniques.
It is therefore extremely important that clinicians in
the field know the indications and limitations of molecular genetic testing. These will be reviewed in this
manuscript.
Etiology and Classification
The etiology of TAAD is complex and heterogeneous. Degenerative aortic disease related to classical
cardiovascular risk factors such as smoking, arterial
hypertension, and hyperlipidemia are the main cause
of TAAD in older patients. In younger patients with no
risk factors, other causes, including genetic disease,
should be considered. Genetic aneurysmal disease
*Corresponding author:
Julie De Backer, MD, PhD
Department of Cardiology and Medical Genetics
University Hospital Ghent
De Pintelaan 185
9000 Ghent, Belgium
Tel: ⫹32 9 332 5627, Fax: ⫹32 9 332 4970, E-Mail: [email protected]
136
can be categorized in three main groups: (i) inherited
syndromes predisposing to early onset TAAD (⬍5% of
all TAAD) such as Marfan syndrome (MFS), Loeys-Dietz
syndrome (LDS), and Aneurysm-Osteoarthritis syndrome [1–3]; (ii) familial forms of TAAD (FTAAD - 20%
of all TAAD) including patients with confirmed disease
in first-degree relatives and evidence for an autosomal
dominant inheritance pattern; these patients may
sometimes present with associated cardiovascular lesions such as biscuspid aortic valve (BAV), patent ductus arteriosus (PDA), or cerebrovascular disease [4–6];
and (iii) isolated or sporadic forms of TAAD (80%)
including patients with no family history or clinical
features of a syndromic TAAD disorder. The latter two
categories are the so-called nonsyndromic forms of
TAAD as opposed to the syndromic forms of the first
category.
Table 1 provides an overview of syndromic and
nonsyndromic forms of TAAD with their corresponding genes and clinical features. Discriminative features
are in bold.
The paradigm disease for genetically determined
syndromic TAAD is Marfan syndrome (MFS), caused by
mutations in the fibrillin-1 gene (FBN1). The diagnosis
of MFS is based on the identification of clinical manifestations and may be supplemented with FBN1 gene
sequencing. Cardinal manifestations include dilatation
of the aortic sinus, lens luxation, and a combination of
additional features defined by the “systemic score.”
Dilatation of more distal parts of the aorta occurs in a
minority of MFS patients [7–9]; patients who underwent previous surgery of the ascending aorta seem at
increased risk. A recent study from Mimoun and colleagues demonstrated that dissection in the descending part of the aorta may occur whatever the diameter
of the ascending aorta [10].
In 2004, Mizuguchi et al. identified mutations in the
Transforming Growth Factor Beta Receptor 2 gene
(TGFBR2) in a large family and four additional probands presenting with aortic dilatation and variable
additional clinical features reminiscent of a connective
tissue disorder, referred to as Marfan syndrome type2
[11]. In 2005, Loeys et al. published their findings on a
large series of patients presenting with widespread
aggressive aortic disease with rapid growth and early
dissections. They observed an increased prevalence of
dysmorphic features including hypertelorism and cleft
palate/bifid uvula. Patients harbored mutations in either the TGFBR1 or TGFBR2 gene and the disorder was
De Backer, J. et al.
Basic Science for the Clinician
named after the authors (Loeys-Dietz syndrome, LDS)
[12]. Patients with LDS may also present with arterial
tortuosity/aneurysms/dissections outside the aorta
necessitating extensive vascular imaging at regular
time intervals. With the identification of mutations in
genes involved in the TGF␤ pathway, a new era with
regards to our understanding of the pathophysiology
and treatment of TAAD emerged. Other gene mutations have been identified including the SMAD3 gene
causing Aneurysm-Osteoarthritis syndrome and mutations in the TGF␤ ligand. In view of the important
clinical overlap between these disorders, the term
“TGF␤ associated vasculopathies” may be preferred
over individual syndrome names.
The genetic background of nonsyndromic TAAD is
even more complex and heterogeneous. Genes involved in syndromic forms may also be encountered
in patients with isolated aortic disease, emphasizing
the fact that the clinical spectrum of these disorders is
very broad. Other genes involved in nonsyndromic
TAAD include the ACTA2 gene (encoding smooth
muscle ␣-actin), the MYLK gene, encoding myosin light
chain kinase and the MYH 11 gene encoding the
myosine heavy chain subunit [4,13–15]. The proteins
encoded by these genes are involved in the vascular
smooth muscle cell apparatus. Patients present with
TAAD, sometimes in association with other features
such as livedo reticualris, iris flocculi, and cardiovascular disease in the case of ACTA2, patent ductus arteriosus in the case of MYH11, and gastro-intestinal
disease in the case of MYLK.
Establishing a correct diagnosis of TAAD in an
individual patient primarily requires detailed clinical
evaluation of the proband and family members (see
below). It should be noted, however, that substantial
clinical overlap exists between these subgroups.
Therefore, additional molecular genetic testing may
be helpful and sometimes even required for confirmation of the specific diagnosis.
Strategy for Clinical Evaluation and Genetic Testing
The absolute prerequisite for further clinical/
genetic investigations in TAAD patients is a correct
diagnosis of the aneurysm itself, based on careful
measurement of the diameter of the aorta according
to appropriate guidelines [35]. The measurements obtained need to be correlated to values in normal
subjects matched for age, body surface area, and gender [36]. To correlate with normal values, nomograms
Genes in Thoracic Aortic Aneurysms and Dissections
Aorta, July 2013
NOTCH1
MYH11
Highly calcified Valve
Patent ductus arteriosus
Thoracic Aortic Aneurysm/Dissection
Intracranial and other arterial aneurysms
Mitral valve prolapse
Arterial Tortuosity, Arterial Stenoses and Aneurysms
Aortic Root Aneurysm, Arterial Tortuosity
Non Syndromic TAAD
Thoracic Aortic Aneurysm/Dissection
Thoracic Aortic Aneurysm/Dissection, cerebrovascular disease,
coronary artery disease
Aortic Root Aneurysm, Aortic Dissection, Arterial Aneurysms
and Dissections, Arterial Tortuosity, Mitral Valve Prolapse,
Congenital Cardiac Malformations*
Aortic Root Aneurysm, Aortic Dissection, Arterial Aneurysms
and Dissections, Arterial Tortuosity, Mitral Valve Prolapse,
Congenital Cardiac Malformations*
Aortic Root Aneurysm, Aortic Dissection, Arterial Aneurysms
and Dissections, Arterial Tortuosity, Mitral Valve Prolapse,
Congenital Cardiac Malformations*
Mild aortic root dilatation, mitral valve prolapse
Syndromic TAAD
Aortic Root Aneurysm, Aortic Dissection, Mitral Valve Prolapse,
Main Pulmonary Artery Dilatation, Left Ventricular
Dysfunction
Arterial Rupture and dissection without preceding dilatation/
aneurysm, severe valvular insufficiency
Main Cardiovascular Features
Overview of syndromic and nonsyndromic forms of TAAD with their corresponding genes and clinical features. Discriminative features are in bold.
FTAA with patent ductus arteriosus (PDA) (6)
FTAA with bicuspid aortic valve (BAV) (33, 34)
MYLK
SMAD3 (2%)
TGF␤2
ACTA2
TGFBR1/2 (3–5%)
ACTA2 (10–14%)
TGF␤2
TGF␤2 (24, 25)
Familial thoracic aortic aneurysm syndrome (FTAA) (30–32)
SMAD3
Aneurysm-Osteoarthritis (21–23)
SLC2A10
FBLN4
TGFBR1/2
Loeys-Dietz (2, 12)
TGF␤-related vasculopathies
Arterial Tortuosity Syndrome (28)
Cutis Laxa Syndromes (29)
COL3A1, COL1A2
Ehlers-Danlos (18–20) (vascular, valvular)
SKI
FBN1
Marfan (1, 16, 17)
Shprintzen-Goldberg syndrome (26, 27)
Gene(s)
Disorder
Table 1. Schematic Overview of Syndromic and Nonsyndromic TAAD (Thoracic Aortic Aneurysm and Disections)
Lack of Marfanoid skeletal features, livedo
reticularis, iris flocculi
Lack of syndromal features
Lack of Marfanoid skeletal features, livedo
reticularis, iris flocculi, coronary
artery/cerebrovascular disease)
Gastro-intestinal abnormalities
Craniosynostosis, distinctive craniofacial
features, skeletal changes, neurologic
abnormalities, mild-to-moderate intellectual
disability
Hyperlax skin and -joints
Hyperlax skin and -joints, mild emphysema
Osteoarthritis, soft skin, flat feet, scoliosis,
recurrent hernia’s, hypertelorism, pectus
abnormalities
Club feet, soft translucent skin
Lens luxation, skeletal features (arachnodactylia,
pectus deformity, scoliosis, flat feet, increased
armspan, dolichocephalia)
Thin, translucent skin, dystrophic scars, facial
characteristics (Madonna face, thin lips, deep
set eyes)
Bifid uvula/cleft palate, hypertelorism, pectus
abnormalities, scoliosis, club feet
Additional Clinical Features
Basic Science for the Clinician
137
Volume 1, Issue 2: 135–145
138
can be used or z-scores can be calculated, the latter
method being more convenient for reporting. Aortic
dilatation is confirmed if the z-score exceeds 2, corresponding to an observed value ⬎1.96 standard deviations above the predicted value for age, gender, and
body size. In children, growth needs to be taken into
account and z-scores ⬎3 have been suggested [37].
Further investigations will depend on the age and
cardiovascular risk profile of the patient.
Consideration of a genetic entity is especially of
interest in young subjects with no additional risk factors. Detailed family history taking, including pedigree
drawing and clinical assessment of first degree relatives, is required to differentiate between familial and
isolated forms of TAAD. Next, careful multidisciplinary
clinical evaluation of the proband is undertaken,
which will help us in the identification of specific
syndromes as reported in Table 1.
Since TAAD is a genetically heterogeneous disease
with important clinical overlap between the known
genetic entities, there is a clear need for simultaneous
testing of multiple genes. Until recently, strategies for
genetic testing were limited as only one gene at the
time could be analyzed, and both the time required as
well as the costs for screening of multiple genes were
substantial. The need for high-throughput techniques
enabling simultaneous testing of several genes was
met by the recent development and progress made in
the field of Next Generation Sequencing. Previously,
our Center reported a mutation detection strategy
using massive parallel sequencing of the FBN1 and
TGFBR-1 and -2 genes for the molecular diagnosis of
MFS and LDS [38]. In a next stage, we implemented a
novel screening strategy that allows simultaneous sequencing of 16 TAAD-associated genes. To this purpose, two complementary panels of genes were designed, of which all coding regions and flanking
sequences can be amplified in a fully automated fashion followed by sequencing on an Illumina MiSeq
sequencer (Illumina, San Diego, California). The first
gene panel comprises FBN1, TGFBR1/2, SMAD3, TGFB2,
ACTA2, and COL3A1. The second gene panel comprises
MYH11, MYLK, SLC2A10, NOTCH1, FBN2, ADAMTS10,
FBLN4, FLNA, and ELN.
Correct interpretation of the results obtained by
molecular genetic testing requires basic knowledge of
these different genes and clinical entities - all the
more since medical and surgical management may
differ according to the underlying diagnosis.
De Backer, J. et al.
Basic Science for the Clinician
Importantly, the simultaneous sequencing of multiple TAAD-associated genes is not always justified. In
patients presenting with a thoracic aortic aneurysm in
combination with lens luxation for instance, Marfan
syndrome is very likely and molecular genetics can be
restricted to the FBN1 gene. Or, from a cardiovascular
perspective, extensive vascular disease such as aortic
aneurysms at different locations and/or involvement
of side branches makes a diagnosis of Marfan syndrome much less likely and in these cases, TGF␤associated disease should be excluded first. A flow
chart illustrating the diagnostic process (clinical and
genetic evaluation) is provided in Figure 1.
Genes and Pathogenesis
In addition to its usefulness in a diagnostic setting,
molecular genetics have been very useful in unraveling the complex pathogenesis of TAA formation.
One of the most inspiring findings over recent years
was the observation of the involvement of the
Transforming Growth Factor ␤ (TGF␤) pathway in
several connective tissue disorders. The TGF␤ superfamily consists of a number of cytokines that regulate diverse cellular functions, including proliferation, differentiation, and synthesis of a wide array of
gene products.
The first heritable connective tissue disorder linked
to the TGF␤ pathway was Marfan syndrome (MFS). The
underlying pathogenesis of aneurysm formation in
MFS was initially considered to be a consequence of
inherent structural weakness of the tissues due to
structurally abnormal fibrillin-1 fibers. Prospects for
causal treatment were pessimistic in this view since
this would require a means to alter the structural
composition of inherently weak tissues.
Fortunately, recent developments have changed
this insight and it is now recognized that fibrillin-1
containing microfibres also play an important functional role in the complex TGF␤ pathway. Although it
is clear that the TGF␤ pathway plays a role in the
pathogenesis of MFS, the mechanism of TGF␤ activation remains controversial. On the one hand, it has
been suggested that TGF␤ is activated as a consequence of improper sequestration of the latent TGF␤
complex, which is the result of a reduction of fibrillin-1
below a certain threshold [39]. On the other hand,
Charbonneau and colleagues demonstrated that an
Fbn1 mouse in which the latent TGF␤ binding protein
site (LTBPs) was deleted (Fbn1H1⌬) did not present
Genes in Thoracic Aortic Aneurysms and Dissections
Basic Science for the Clinician
Figure 1.
139
Flow chart illustrating the diagnostic process (clinical and genetic evaluation). NGS: Next Generation Sequencing.
features of MFS [40]. Hence, instead of reduced TGF␤
sequestration, mutant microfibrils probably influence
TGF␤ activation in a different way. Sakai and coworkers demonstrated that fibrillin-1 was homologous to
the family of LTBPs which serve to hold TGF␤ in an
inactive complex in various tissues, including the extracellular matrix [41]. Fibrillin-1 binds TGF␤ and LTBPs
[41–44].
Since the large latent complex binds TGF␤, abnormal fibrillin-1 fibers will lead to failed matrix sequestration of the latent TGF␤ complex and hence to
increased amounts of active TGF␤, which is in turn at
the basis of the pleiotropic manifestations in MFS [39].
Indeed, increased TGF␤ signaling has been demonstrated in aortic tissue samples and in mitral valve
tissue from patients with MFS. These findings have
opened new perspectives for treatment through inhibition of TGF␤-signaling (see below).
Additional evidence for the involvement of the
TGF␤ pathway in aneurismal disease came from the
findings that mutations in several genes that encode
different components of the pathway result in aneurysm conditions that have undeniable clinical overlap
Aorta, July 2013
with MFS. Initially, these disorders were given names,
the first one being the Loeys-Dietz syndrome (LDS),
caused by mutations in the TGFBR1 and TGFBR2
genes. In 2011, mutations in the SMAD3 gene were
identified in patients with a very similar phenotype
but also presenting with osteoarthritis, hence the
name “Aneurysm-Osteoarthritis syndrome (AOS).”
Soon thereafter a family with juvenile polyposis associated with aortopathy and mitral valve disease
caused by SMAD4 mutations was reported [45]; and
finally, mutations in the TGF␤-2 ligand were very recently identified in several families displaying a very
similar phenotype [25,46,47]. It is clear that these disorders are all part of a broad spectrum and it may be
more convenient to group them under the term
“TGF␤-related vasculopathies.” Figure 2 provides a
schematic and abbreviated overview of the TGF␤ signalling pathway with indication of aneurismal diseases
linked to it.
Increased TGF␤ signaling has also been reported in
human aortic specimens of patients with familial
TAAD and underlying ACTA2 or MYH11 mutations [48].
The exact link is not yet fully understood but links
Volume 1, Issue 2: 135–145
140
Basic Science for the Clinician
Provides a schematic and abbreviated overview of the TGF␤ signaling pathway with indication of aneurismal
diseases linked to it. The TGF␤ pathway and related vasculopathies. Following its release from the Extrcellular Matrix, TGF␤
binds to its type II cell surface receptor (T␤RII), which recruits and phosphorylates the type I receptor (T␤RI). T␤RI then
recruits and phosphorylates SMAD2 and/or SMAD3. These P-SMADs then bind to the common SMAD (co-SMAD) SMAD4 to
form a heterodimeric complex. This complex enters the cell nucleus where it acts as a transcription factor for various
TGF␤-dependent genes, such as connective tissue growth factor (CTGF), plasminogen activator inhibitor-1 (PAI-1) and
multiple collagens.
Figure 2.
between the contractile cytoskeleton and many aspects of the TGF␤ signaling pathway have been established, including trafficking and activity of TGF␤
receptors and signaling effectors [49,50].
Gene-Tailored Follow-up and Management in TAAD
A schematic overview of the medical management
is provided in Table 2.
Imaging Studies
Confirmation of the exact diagnosis in the proband
facilitates the set-up of a personalized strategy for
follow-up and treatment in the patient and his/her
family. Since the clinical manifestation of the disease is
age dependent and may progress subclinically until
later in life, lifelong follow-up is required in all mutation-carriers, even if aortic diameters are normal on
repeated measurements. The frequency and modality
for follow-up and treatment may differ according to
the underlying diagnosis as summarized in Table 2.
Importantly, clinical monitoring and follow-up with
De Backer, J. et al.
cardiovascular imaging is also warranted in family
members of TAAD patients in whom no causal mutation was identified since familial clustering is observed
in more than 20% of TAAD cases [51,52].
Echocardiography is the primary imaging tool for
evaluation and follow-up of the diameters of the aortic root and ascending aorta. CT or MRI can be used in
case of insufficient visualization of the ascending aorta
by echocardiography. The imaging study should be
repeated in all patients six months after the initial
diagnosis to assess evolutionary changes. Further follow-up is guided by the diameter, evolution, underlying diagnosis, and family history. Stable diameters
⬍45 mm in patients with Marfan syndrome or isolated
TAA and no family history for dissection require yearly
follow-up. Biannual controls are recommended in all
other cases.
On initial diagnosis, imaging of the entire aorta and
side branches (“head-to-pelvis” study) should be performed in order to detect aneurysms at other sites
Genes in Thoracic Aortic Aneurysms and Dissections
Aorta, July 2013
No trials yet — adopt from Marfan syndrome
No trials yet — adopt from Marfan syndrome
Same as in Marfan syndrome
Consider coronary/cerebrovascular
imaging in ACTA2 mutation carriers
Echocardiography q6mo-1y (also
related to valvular function)
Same as in Marfan syndrome
AoD: Aortic Root Diameter; AR: Aortic Regurgitation; MR: Mitral Regurgitation; MRA: Magnetic Resonance Angiography.
FTAA with bicuspid
aortic valve (33, 34)
FTAA with patent
ductus arteriosus (6)
Loeys-Dietz (2, 12)
Aneurysm-Osteoarthritis (21–23)
TGF␤2 (24, 25)
Arterial Tortuosity Syndrome (28)
Cutis Laxa Syndromes (29)
Familial thoracic aortic aneurysm syndrome (FTAA) (30–32)
No trials yet — adopt from Marfan syndrome
Ehlers-Danlos (18-20) (vascular, valvular)
CT/MRI head to pelvis q6mo-1y
surgery when AoD ⬎50 mm or ⬎46 mm in case
of familial history of dissection or rapid
growth (⬎2 mm/y) or severe AR or MR
Celiprolol
Surgery uncertain
No trials yet — adopt medical treatment from
Marfan syndrome
Surgery when AoD ⬎43-45 mm
echocardiography q1y when diameter
⬍45 mm q6m in all other cases
MRAq5y when aortic diameters outside
the sinuses of Valsalva are normal,
MRAq1y in all other cases
Unclear (dissection/rupture often at
normal diameters)
Echocardiography q6mo
␤-blocking agents, losartan?
Marfan (1, 16, 17)
TGF␤-related
vasculopathies
Follow-up
Treatment
Disorder
Table 2. Overview of Suggested Treatment and Follow-up in TAAD
Basic Science for the Clinician
141
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142
and/or arterial tortuosity. Regular extensive vascular
imaging from head to pelvis is recommended in patients with a TGFBR1/2, SMAD3, and TGF␤2 mutation
and for rare diseases such as cutis laxa and arterial
tortuosity syndrome (ATS). In vascular Ehlers-Danlos
syndrome (EDS) where dissections often occur at normal diameters, the modality and frequency of vascular
imaging is debatable. Evaluation for coronary artery
and cerebrovascular disease can be considered in patients with an ACTA2 mutation [53].
Medical Treatment
The initial medical approach of TAAD patients
should include reduction of cardiovascular risk factors,
such as blood pressure control, smoking cessation,
and optimization of the lipid profile. Central stimulating drugs, such as cocaine, amphetamine and derivatives are known triggers for aortic dissection and
should therefore be avoided [54,55].
Medical treatment with a ß-blocking agent in MFS
reduces the progression of aortic dilatation in most
patients through reduction of wall shear stress in the
aorta and is used as a standard therapy in MFS patients [56]. As already mentioned, it has been clearly
demonstrated that the TGF␤ pathway plays an important role in aneurysmal disease. This knowledge has
led the search for strategies to interfere with TGF␤
signalization. From studies in nephrology, it was documented that losartan, an angiotensin receptor blocking agent, inhibits TGF␤ signaling. An initial experiment with TGF␤ neutralizing antibodies in a mouse
model for MFS showed a dramatic decrease in aortic
root growth as well as restoration of aortic wall architecture [57]. A trial with losartan in MFS mice showed
significant rescue of aortic root aneurysm progression
as well as aortic wall architecture, compared to treatment with either placebo or propranolol [57]. A subsequent small study in children with severe MFS
showed similar very promising results [58]. Large-scale
trials in MFS patients are currently underway [59] and
need to be awaited prior to large-scale prescription.
In patients with vascular EDS, reduction of fatal
vascular events was observed with treatment with
celiprolol, a ␤-blocker with ␤2 mimetic action [60]. The
possible role of medical treatment in other TAAD
diseases is not well studied but pragmatically, treatment as for MFS is adopted.
De Backer, J. et al.
Basic Science for the Clinician
Surgery
It is beyond any doubt that elective surgical aortic
root replacement leads to better survival in patients
with genetic aortic disease. Modalities for surgical intervention are beyond the scope of this contribution.
We do want to spend some words on the timing of
surgery taking the underlying diagnosis into account.
It has been demonstrated that the risk for dissection or rupture for thoracic aortic aneurysms of nondegenerative origin rises at lower diameters when
compared to degenerative aortic disease. Accordingly,
the threshold for surgery of the aortic root is lower
than the conventional 55 mm. Indeed if conforming to
the European Society of Cardiology guidelines on
Grown-up Congenital Heart Disease, the conventional
surgical indication for MFS is an aortic diametermeasured at the sinuses of Valsalva at 50mm or more.
This threshold is reduced to 46 mm in the case of a
positive family history of aortic dissection or rapid
growth of the aorta (⬎2 mm/yr). When there is a
desire of pregnancy, aortic repair at 45 mm is recommended [61]. In certain other syndromic and nonsydromic TAAD entities such as LDS or AOS, aortic dissection can occur at smaller diameters, therefore
requiring an adjusted treatment policy. Results of surgical intervention in LDS and AOS are good [3,62].
Taking these data into account, the current guidelines
of the American College of Cardiology recommend
prophylactic surgery in the following cases [63,64]: (i)
patients with a mutation in TGFBR1 or TGFBR2 (as well
as patients with LDS as familial TAAD), when the
diameter of the ascending aorta reaches 42mm measured by echocardiography or 44 – 46 mm on CT or MR
imaging; (ii) patients with familial TAAD and/or mutation in MYH11 or ACTA2, when the diameter of the
ascending aorta measures between 45 and 50 mm; (iii)
patients with familial TAAD with relatives with an
aortic dissection at minimal dilatation of the thoracic
aorta (⬍50 mm); (iv) for all other TAAD patients when
the ascending aorta or aortic root reaches a diameter
of 50 mm, in case of rapid growth of the aorta (ⱖ5
mm/y) and/or in the presence of severe aortic stenosis or
regurgitation. Patients with a mutation in the MYLK
gene can have an aortic dissection at small diameters
of the aorta, as indicated by the study of Wang et al.
[15]. Guidelines regarding the role of prophylactic
surgery in this group of patients are lacking. In contrast to patients with a TGFBR2 mutation, aortic dissection in patients with a TGFBR1 mutation would
Genes in Thoracic Aortic Aneurysms and Dissections
Basic Science for the Clinician
143
rather occur at larger diameters (⬎50 mm) [65]. In
view of these data, early referral for surgery may be
questioned.
this knowledge. Close collaboration between cardiovascular surgeons, cardiologists, and clinical geneticists is strongly recommended in the care of
these patients and families.
Conclusion
Acknowledgments
In the current era of improved availability of highthroughput molecular genetic techniques, knowledge
of the indications and limitations for these tests in
daily clinical practice is increasingly important. In the
case of TAAD, additional genetic testing may be helpful for confirmation of the correct diagnosis. Since
follow-up and treatment of patients may be
adapted according to the underlying condition, clinicians dealing with these patients should acquire
Julie De Backer is supported by a grant as Senior
Clinical Investigator from the Fund for Scientific Research, Flanders (Belgium). Bert Callewaert is supported
as a postdoctoral fellow by the Fund for Scientific Research, Flanders (Belgium). Anne De Paepe is holder of a
Methusalem grant from the Flemish government.
Comment on this Article or Ask a Question
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RM, Carr VF, Casey DE, Jr., et al. 2010ACCF/
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guidelines for the diagnosis and managethey Matter? Translation and Integration
ment of patients with Thoracic Aortic Disof Research and Modern Genetic Techease: a report of the American College of
niques into Daily Clinical Practice. Aorta
Cardiology Foundation/American Heart As2013;1(2):135–145.DOI:http://dx.doi.org/
sociation Task Force on Practice Guidelines,
10.12945/j.aorta.2013.13-024
American Association for Thoracic Surgery,
EDITOR’S COMMENTS
Dr. De Backer and colleagues, from the distinguished Ghent group, provide a clinically oriented
primer on the current state of knowledge regarding
the genetics of thoracic aortic aneurysm. They tell
us just how to use genetic testing in the present era.
As well, they provide useful management and clinical guidelines that take into account the emerging
knowledge of aneurysm behavior in specific genetic
syndromes. They usher us into the era of personal-
Aorta, July 2013
ized aortic management based on molecular genetics.
The Editors only point of difference concerns frequency of imaging. Since the aorta grows very slowly
in the vast majority of thoracic aneurysm patients
(about 1 mm per year), after the first yearly ECHO, CT,
or MRI are done, we decrease frequency of imaging to
every two to three years. (ECHOs can be done frequently, if desired, because of low cost and zero toxicity. We use restraint in CT and MRI.)
Volume 1, Issue 2: 135–145
Images in Aortic Disease
Aorta, July 2013, Volume 1, Issue 2: 146 –148
DOI: http://dx.doi.org/10.12945/j.aorta.2013.13-023
Received: April 9, 2013
Accepted: May 25, 2103
Published online: July 2013
Imaging Assessment of Periaortic Inflammation
in Erdheim-Chester Disease
Thierry Couvreur, MD1, Györgyi Lipcsei, MD2, Alain Nchimi, MD1*
1
Department of Medical Imaging, University Hospital–Sart Tilman, Liège, Belgium; 2Department of Pathology, CHC–St Joseph, Liège,
Belgium
Abstract
Reaching etiologic diagnoses for retroperitoneal fibrosis may be challenging. We report the case of a 75-year
old male with history of ruptured abdominal aortic
aneurysm and subsequent retroperitoneal fibrosis who
developed four years later a soft tissue infiltration surrounding the ascending thoracic aorta. Thanks to his
medical records and multimodality imaging assessment, the patient escaped an open-chest biopsy
through histolgical reassessment of the abdominal
periaortic samples that allowed the definitive diagnosis
of Erdheim-Chester disease, a rare non-Langerhans
histiocytosis.
Copyright © 2013 Science International Corp.
Key Words
Aortitis · Histiocytosis · Erdheim-Chester · Magnetic Resonance Imaging · Positron Emission Tomography
Case Report
A 75-year-old male presented with three selfsubsiding episodes of malaise and nausea. Medical
history included surgery for ruptured inflammatory
abdominal aortic aneurysm, four years earlier; pathology of the surgical specimen showed idiopathic perianeurysmal fibrosis. Postoperative follow-up was
marked by periaortic graft infiltration involving
both ureters, necessitating bilateral ureteral stenting and steroid therapy for eight months. The patient had remained asymptomatic for three years
until the onset of malaise. Clinical examination and
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© 2013 Aorta.
Published by Science International Corp.
ISSN 2325-4637
Accessible online at:
http://aorta.scienceinternational.org
ECG were unremarkable. The serum C-reactive protein was at 7 mg/L (normal 0 – 6), and blood cell
count showed neutrophilia (85%) without hyperleucocytosis and a slight microcytic anemia (hemoglobin: 12.4 g/100 mL). On echocardiography, a 10 mm
pericardial effusion (PE) was noticed and subsequent computed tomography (CT) displayed a layer
of abnormal soft tissue surrounding the thoracic
aorta (Figure 1A, arrows), which was not present on
a prior examination (not shown). Whole-body transverse 18F-fluoro-deoxyglucose positron emission tomography (FDG-PET) (Figure 1B) and high b-value
(800 s/mm2) diffusion-weighted MRI (DW-MRI) (Figure 1C) were performed and, respectively, color
map-fused with CT (Figure 1D and 1E), showing in a
roughly similar distribution, an increased FDG uptake and decreased water diffusion (arrows). No
other pathological area was identified. Both molecular
imaging techniques helped with differentiating multiorgan malignancy from perivascular inflammatory
diseases, allowing hypothesis that the current disease
and the previous perianeurysmal fibrosis are actually
two expressions of the same disease. We, therefore,
discussed either Erdheim–Chester disease, or large
vessel vasculitis such as Takayasu and giant cell arteritis. Erdheim–Chester disease is an uncommon nonLangerhans cell histiocytosis characterized by a
perivascular, adipose, and connective tissue tropism
[1–3] that may be responsible for widespread vascular
involvement, including “chronic periaortitis,” a ge*Corresponding author:
Alain Nchimi, MD
Department of Medical Imaging
University Hospital
Domaine Universitaire du Sart-Tilman B 35
B - 4000 Liege, Belgium
Tel: ⫹32 4 3667259, Fax: ⫹32 4 3667224, E-Mail: [email protected]
Images in Aortic Disease
147
A 75-year-old male with malaise and history of inflammatory abdominal aortic aneurysm rupture four years earlier. (A)
Unenhanced transverse computed tomography (CT) of the chest demonstrates a perivascular soft tissue mass, encasing the aortic
arch (arrows). (B) 18F-Fluorodeoxyglucose positron emission tomography (FDG PET) and (C) color intensity map fusion with CT
showed a remarkable uptake of the tissue. (D) Transverse diffusion-weighted magnetic resonance with a diffusion-factor value
of 800 s/mm2 and intensity color maps fusion to CT (E) images showed restricted diffusion (arrows) in a roughly identical
distribution to FDG uptake. Histological reassessment of the samples obtained during abdominal aortic surgery (F) demonstrates
inflammatory foci with predominance of foamy cells (histiocytes) infiltrates with cytoplasmic brown deposition at immunoperoxydase stain with CD68 (arrows), consistent with the diagnosis of Erdheim–Chester disease for which other immunohistological
hallmarks were a negative staining for both CD1a and S-100 protein (not shown). (G) Unenhanced transverse CT showing mild
decrease of the perivascular soft tissue after treatment.
Figure 1.
neric term for perianeurysmal retroperitoneal fibrosis,
inflammatory abdominal aortic aneurysm, and idiopathic retroperitoneal fibrosis. This tropism noticed in
our patient history was a clue to the diagnosis, even
though the initial pathological evaluation may have
failed for several reasons, including undersampling.
We proceeded to a histopathologic reassessment of
the perianeurysmal samples obtained 4 years earlier. It
revealed inflammatory foci with a predominance of
foamy cells (histiocytes) infiltrates with cytoplasmic
Aorta, July 2013
brown deposition at immunoperoxydase stain with
CD68 (Figure 1F, arrows) that eventually allowed the
definitive diagnosis of Erdheim–Chester disease. Other
immunohistological hallmarks of the disease were a
negative staining for both CD1a and S-100 protein
(not shown). The patient was treated by further steroid administration of methylprednisolone. Follow-up
chest CT showed a marked decrease of the periaortic
infiltration (Figure 1G), while the patient remained
asymptomatic.
Volume 1, Issue 2: 146 –148
148
Acknowledgments
The authors express their gratitude to Professor RF
Dondelinger, Department of Medical Imaging, Univer-
Images in Aortic Disease
sity Hospital - Sart Tilman Liège, for the preparation of
the manuscript.
Comment on this Article or Ask a Question
References
1. Dion E, Graef C, Haroche J, Renard-Penna R, 3. Jennette JC, Falk RJ. The role of pathology in
Cluzel P, Wechsler B, et al. Imaging of thorathe diagnosis of systemic vasculitis. Clin Exp
coabdominal involvement in Erdheim-ChesRheumatol. 2007;25:S52–S56.
ter disease. AJR Am J Roentgenol. 2004;183:
1253–1260. 10.2214/ajr.183.5.1831253
2. Vaglio A, Salvarani C, Buzio C. Retroperitoneal
fibrosis. Lancet. 2006;367:241–251. 10.1016/
S0140-6736(06)68035-5
Couvreur, T. et al.
Cite this article as: Couvreur T, Lipcsei G,
Nchimi A. Imaging Assessment of Periaortic
Inflammation in Erdheim-Chester Disease.
Aorta 2013;1(2):146–148. DOI: http://dx.doi.
org/10.12945/j.aorta.2013.13-023
Imaging Assessment of Periaortic Inflammation
Poll the Editorial Board
Aorta, July 2013, Volume 1, Issue 2: 149 –151
DOI: http://dx.doi.org/10.12945/j.aorta.2013.13-026
Received: June 3, 2013
Accepted: June 3, 2013
Published online: July 2013
How Would You Correct an Aberrant Right
Subclavian Artery?
Bulat A. Ziganshin, MD
(on behalf of the Editorial Office)
Key Words
Aberrant subclavian artery · Treatment approach
A 63-year-old female presented having suffered an
embolic event to her right index finger. This resolved
successfully with conservative treatment via development of collateral channels. The finger is fully viable,
albeit mildly insensitive. She has had some dysphagia,
with one specific choking episode when a lozenge
became lodged in the esophagus, causing discomfort
and cough until it dissolved spontaneously.
Work-up revealed an aberrant right subclavian artery, with associated Kommerell’s dilatation and a 1
cm wide ulcerated area near its origin from the aorta,
as well as an arteriosclerotic irregularity of the proximal subclavian artery. Passage of the aberrant subclavian artery behind the trachea and esophagus produced esophageal compression. See computed
tomography (CT) scan images in Figure 1.
The question regarding this case was:
How would you correct this lesion?
● Open surgery
● Intraluminal endovascular treatment
● Combined surgical-endovascular approach (hybrid operation) with right subclavian artery
transposition and thoracic aortic stent graft
implantation
● Other approach
The respondents who selected the open surgery
option were asked:
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© 2013 Aorta.
Published by Science International Corp.
ISSN 2325-4637
Accessible online at:
http://aorta.scienceinternational.org
Please indicate— open surgery to include which of
the following (multiple choice question):
● Left thoracotomy for division of subclavian artery and, thus, interruption of vascular ring
● Neck approach for ligation of subclavian artery
(to allow thrombosis of aberrant artery), with
carotid to subclavian bypass for distal perfusion
● Ligation of thyrocervical trunk and internal
mammary artery (IMA)
● Other
Selection of the “Other approach” option to the
main question and the “Other” option to the secondary question prompted a text field where the respondents could describe their approach.
The poll was distributed among all current members
of the Editorial Board, who were asked to submit their
responses via an online survey tool. The list of Editorial
Board members can be found the AORTA journal website (http://aorta.scienceinternational.org). The members
of the Editorial Board whose practice does not lie within
the scope of this question were asked to disregard this
poll. Here we present the results of this poll.
Results of the “Poll the Editorial Board”
Thirty-three members of the Editorial Board submitted responses through our online survey tool. The
results are presented in the pie chart of Figure 2 and
Table 1.
Corresponding author:
Bulat A. Ziganshin, MD
AORTA Journal Editorial Office
Boardman 204
330 Cedar Street
New Haven, Connecticut 06510 (USA)
Tel: ⫹1 203 785 2551, Fax: ⫹1 203 785 3346, E-Mail: [email protected]
150
Poll the Editorial Board
Figure 2. Pie chart diagram illustrating the responses of the
Editorial Board members to the poll.
Table 1. Responses of the Editorial Board members (n ⫽ 10)
that specified preference for open surgery (multiple choice
question)
Preferred technique for open
surgical treatment of an
aberrant right subclavian
artery
Left thoracotomy for division
of subclavian artery and,
thus, interruption of
vascular ring
Neck approach for ligation of
subclavian artery (to allow
thrombosis of aberrant
artery), with carotid to
subclavian bypass for distal
perfusion
Ligation of thyrocervical trunk
and IMA
Other
No. of
votes
Percentage
7
70%
4
40%
0
2ⴱ
0
20%
ⴱThe two respondents that selected the response “Other” indicated the following as their preferred technique for open surgery:
1 –Left thoracotomy, division of right subclavian artery, and connection to ascending
aorta.
2 – (1) Medium sternotomy. (2) Suture of the aberrant artery at the level of its origin. (3)
Ascending aorta—right subclavian bypass graft OR reimplant of distal right subclavian
artery to the right carotid artery.
Axial CT scan images showing the aberrant right
subclavian artery (red arrow).
Figure 1.
Comment
The results of the poll show the increasing popularity of the hybrid procedures with the majority of
the respondents (55%) indicating their preference
Ziganshin, B.A.
for the combined surgical and endovascular approach, 30% of the respondents selecting open surgery as their preferred technique, while 12% favored an intraluminal endovascular treatment
approach. Interestingly, among the respondents
that showed preference for open surgery, seven
(70%) indicated the left thoracotomy approach to
Correction of an Aberrant Right Subclavian Artery
Poll the Editorial Board
be their preference. At the same time three (30%)
respondents stated that the combined left thoracotomy and neck approach is their preference. Only
one respondent (10%) selected the isolated neck
approach for ligation of the subclavian artery with a
carotid to subclavian bypass for distal perfusion,
while no respondents showed preference toward
ligation of the thyrocervical trunk and IMA. Two
Aorta, July 2013
151
respondents provided other alternative strategies
for open surgical treatment (see Table 1).
Comment on this Article or Ask a Question
Cite this article as: Ziganshin BA. How Would You Correct an
Aberrant Right Subclavian Artery? Aorta 2013;1(2):149 –151.
DOI: http://dx.doi.org/10.12945/j.aorta.2013.13-026
Volume 1, Issue 2: 149 –151
Upcoming Meetings
Aorta, July 2013, Volume 1, Issue 2: 152
DOI: http://dx.doi.org/10.12945/j.aorta.2013.13-025
Received: May 10, 2013
Accepted: May 10, 2013
Published online: July 2013
List of Upcoming Meetings
July 2013
1. Advanced Aortic and Mitral Valve Reconstructive Surgery
July 5– 6, 2013
Windsor, UK
Meeting information available at:
http://www.eacts.org/academy/2013program.aspx
2. International Academy of Cardiology—18th
World Congress on Heart Disease
July 26 –29, 2013
Vancouver, Canada
Meeting information available at:
http://www.cardiologyonline.com/wchd13/
index.html
November 2013
5. International College of Angiology—
55th Annual Congress
November 7–9, 2013
New Haven, CT, USA
Meeting information available at:
http://www.intlcollegeofangiology.org
6. Surgery of the Thoracic Aorta—7th Postgraduate Course
November 11–12, 2013
Bologna, Italy
Meeting information available at:
http://www.aosp.bo.it/content/presentationcourse
December 2013
October 2013
3. 27th EACTS Annual Meeting
October 5–9, 2013
Vienna, Austria
Meeting information available at:
http://www.eacts.org/annual-meeting.aspx
4. The Southern Thoracic Surgical Association
(STSA) 60th Annual Meeting
October, 30 –November 2, 2013
Scottsdale, AZ, USA
Meeting information available at:
http://stsa.org/60thannualmeeting/
7. Innovations in Cardiovascular Interventions
December 1–3, 2013
Tel-Aviv, Israel
Meeting information available at:
http://www.icimeeting.com
January 2014
8. STS 50th Annual Meeting & STS/AATS TechCon 2014
January 25–29, 2014
Orlando, FL, USA
Meeting information available at:
http://www.sts.org/abstracts