Download Hypertrophic Cardiomyopathy - Carolinas HealthCare System

The
www.sangerheart.org
Hypertrophic Cardiomyopathy
John D.
Symanski, MD,
FACC, FASE
Adult
Cardiologist
Hypertrophic cardiomyopathy
fibrosis [see Figure 2].6 The
(HCM) is defined as excessive ven-
characteristic symptoms of
tricular hypertrophy with a nondilated
HCM (shortness of breath, chest
ventricle in the absence of the typical
discomfort and syncope) are
provocative factors [see Figure 1].
related to several pathophysiologic
HCM is the most common genetic
processes, including: 1) obstruction
cause of heart disease, with an auto-
of blood flow through the narrowed
somal dominant mode of inheritance
left ventricular outflow tract
in most cases. This disorder affects
(LVOTO), 2) disturbance of the
approximately one out of 500 individ-
mitral valve apparatus with
uals in the general population. HCM
resultant mitral regurgitation,
is highly variable in terms of disease
3) subendocardial ischemia and
penetrance and clinical expression.
4) abnormal LV relaxation (diastolic
Age at onset, distribution of ventricu-
dysfunction) [see Figure 3].7
lar hypertrophy, symptom status and
Larry T. Watts,
MD, FACS
Cardiothoracic
Surgeon
Cardiac rhythm disturbances
sudden death risk are unique to each
including atrial fibrillation and
patient and may be related to an
ventricular tachyarrhythmias may also
individual’s specific gene mutation as
contribute to symptom production,
well as modifying factors.1-5
cardiac morbidity and mortality. It
is important to understand that the
Disease characteristics
source of symptoms in any given
Histopathologically, HCM is
patient may result from any one of
characterized by malalignment of
these factors or the combination of
cardiac muscle fibers (myocyte
several pathophysiologic processes.
disarray), dysplastic intramyocardial
arterioles and excessive myocardial
Continued on page 3
SPRING 2011 | Vol. 2 | issue 1
NEW! FREE ONLINE CME
Free CME credit is now available
for noted presentations. Visit
www.sangerheart.org and click
on the “Charlotte AHEC Connect”
logo to find archived and live CME
presentations.
sign up to Receive
The Sanger Report
via e-mail
If you’re interested in receiving
a copy of The Sanger Report via
e-mail, visit www.sangerheart.org
and fill out the short sign-up form
located on our home page. Privacy
policy: Your information will not be
used for third-party distribution.
Inside ...
Hypertrophic
Cardiomyopathy
continued
PAGE 3
Cardiovascular
Disease: Women’s
Initiative
PAGE 5
How Common is
Venous Disease?
PAGE 6
The latest news and best practices from Sanger Heart & Vascular Institute
Fighting
Cardiovascular
Disease
Sanger Heart & Vascular Institute Awards
Jan. 6, 2011 marked the 25th anni-
featured in the ACC’s magazine
versary of the first heart transplant
and on its website for his leader-
performed in Charlotte, NC, by
ship role.
Francis Robicsek, MD. Since then,
Welcome to the second volume of
Sanger Heart & Vascular Institute
 Samuel H. Zimmern, MD, was
The Sanger Report. This is our first issue for 2011,
(SHVI) physicians have performed
published in the HeartRhythm
featuring three clinical articles from our adult cardiology
more than 470 heart transplants,
Society Journal in December 2010,
and cardiothoracic surgery teams.
with our most recent mortality
for his article titled “Mutations
statistics second to none. “We
in the Cardiac L-Type Calcium
Adult Cardiology, and Larry T. Watts, MD, from the
John D. Symanski, MD, from the Department of
couldn’t have made it this far
Channel Associated with Inherited
Department of Cardiothoracic Surgery, explain hypertro-
without the dedication and sup-
J-Wave Syndromes and Sudden
phic cardiomyopathy (HCM), the most common genetic
port of both Carolinas HealthCare
Cardiac Death.” Dr. Zimmern
cause of heart disease and sudden cardiac death in
System and Sanger Heart & Vascular
recently chaired the 8th annual
children and athletes. They review the pathophysiology,
Institute,” says SHVI President
Cardiology Update for Primary Care
diagnosis and management of patients with HCM.
Paul G. Colavita, MD. “Since the
Physicians, held in Charlotte, NC.
Cheryl A. Russo, MD, from the Department of Adult
first transplant, we have developed a
Cardiology, outlines our current women’s initiative.
dedicated Heart Failure/Transplant
 Sherry J. Saxonhouse, MD, from
Dr. Russo brings new perspective to the topic of heart
Clinic, a Pediatric Cardiac
the Division of Electrophysiology,
disease as the leading cause of death among American
Transplant Program and expanded
was selected to serve on the ACC’s
women. She highlights the evidence-based guidelines
our LVAD Program to include
Accreditation Council for Continuing
for cardiovascular disease prevention and the expanding
destination therapy.”
Medical Education and as associ-
literature in the area of cardiovascular disease diagnosis
ate editor of Cardiac Arrhythmia
 Thomas V. Johnson, MD, is
for CardioSource. Dr. Saxonhouse
leading the effort with SHVI to
also has been selected to serve on
Division of Vascular Medicine and Surgery, discusses
participate in the American College
the ACC’s ABIM Board Question
venous disease, specifically varicose veins. The causes
of Cardiology’s (ACC’s) FOCUS
Writing Committee.
of varicose veins and the treatment options available for
Program. FOCUS is a national
this very common disease are also detailed.
in women.
In our closing article, Felipe Navarro, MD, from the
quality improvement initiative that
 Kevin W. Lobdell, MD, from
As you can see from this issue and those before
helps cardiovascular professionals
the Department of Cardiothoracic
it, Sanger Heart & Vascular Institute is committed to
best use Appropriate Use Criteria
Surgery, was published in The
providing high quality, evidence-based care for a broad
and ultimately reduce inappropriate
Annals of Thoracic Surgery in
spectrum of cardiovascular concerns—from congenital
imaging. Dr. Johnson was recently
January 2011, for his article titled,
heart disease to transplants. We will continue to be on
“Effect of Body Mass Index on
the forefront in the fight against cardiovascular disease,
Outcomes after Cardiac Surgery:
and will continue to highlight our progress in the coming
Is There an Obesity Paradox?”
issues of The Sanger Report.
 Francis Robicsek, MD, and
Sincerely,
the International Medical Outreach
Program recently received the
highest recognition for humanitarian
Paul G. Colavita, MD, FACC
and medical achievements in Central
President
America.
Sanger Heart & Vascular Institute
2
The Sanger Report | www.sangerheart.org
Trophy image © 2011 Thinkstock
Rodolfo Robles Award, the
Hypertrophic Cardiomyopathy
Continued from page 1
Disease diagnosis
The diagnosis of HCM may initially
be suspected based on characteristic
symptoms such as:
 an abnormal ECG
 a systolic murmur on cardiac
auscultation
 a history of a family member with
premature sudden death
Confirmation of the diagnosis
usually involves cardiac imaging with
either ultrasound (echocardiography)
or MRI. Genetic testing is now
commercially available and assuming
an increasingly important role in
diagnosis confirmation and family
screening.8,9 HCM remains the most
common cause of sudden death in
young athletes. Although the frequency
of this dreaded complication is low,
F
igure 1
Normal heart (left) and heart in HCM (right). HCM is characterized by a
nondilated ventricular cavity with excessive ventricular wall thickening.
LVH may be diffuse (concentric) or preferentially involve the ventricular
septum, LV free wall, or apex.3
identification and management of
high-risk patients remains an important
clinical challenge.6,10
Preventing sudden
cardiac death in
high-risk HCM patients
associated with an increased sudden
on Holter monitoring or exercise stress
death risk include:
testing
 a family history of HCM-related
 an abnormal or attenuated BP
sudden cardiac death
response
 a history of recent syncope or
Specific HCM gene mutations
sudden death
and presence of myocardial fibrosis
feared complication of the disease and
 extreme hypertrophy (greater than
(as identified by delayed gadolinium
may strike at any time in previously
3-cm wall thickness on echo or MRI)
enhancement on cardiac MRI) are
asymptomatic patients. Factors
 nonsustained ventricular tachycardia
gaining broader recognition in
Sudden death in HCM is the most
Continued on page 4
F
igure 2
Microscopic and histologic features of HCM
include myocyte disarray (left), abnormally
thickened walls of intramyocardial arterioles
with narrowed vessel lumen (center) and
patchy replacement of myocardial fibers
with fibrosis (right).7
www.sangerheart.org | The Sanger Report
3
Hypertrophic Cardiomyopathy
Continued from page 3
F
igure 3
In approximately two-thirds of patients with HCM, prominent septal
hypertrophy results in systolic left ventricular outflow tract obstruction
(LVOTO; white arrow) and distortion of the mitral valve apparatus
with resultant regurgitation (yellow arrow).3
has become less popular due to the
with severe LVOTO since the 1960s.
existence of more effective treatment
[See Figure 5.] Surgical decision-making
options. Alcohol septal ablation is
is tailored by thorough preoperative
favored at some institutions due to
echocardiographic analysis including 3D
its less invasive nature. Complete
reconstruction where specific attention
heart block necessitating pacemaker
is paid to the septal thickness/extent,
implantation, ventricular septal defects
aortic valvular abnormalities and other
and intractable ventricular arrhythmias
associated defects. Careful analysis
may complicate this procedure. The
must be made of the mitral valve and
time-honored surgical treatment option
its subvalvular apparatus looking for
for patients who have obstructive HCM
fusion or thickening of the papillary
is septal myectomy.
muscles with potential attachments to
the free wall, excessive papillary muscle
determination of high-risk individuals
[see Figure 4].6 Automatic implantable
cardioverter-defibrillator (AICD) therapy
Surgical therapy of HCM
Transaortic septal myectomy
has clearly been demonstrated to save
has been the standard surgical
lives in high-risk HCM patients.4-6,10
procedure for symptomatic patients
hypertrophy with anterior displacement,
abnormal chordae tendinae or accessory
Continued on page 8
Managing HCM
Traditionally, medical therapies
including beta-blockers, calcium
channel blockers, disopyramide and
amiodarone have been used alone
or in combination in HCM patients
to ameliorate symptoms and lessen
the frequency of ventricular and
supraventricular arrhythmias.2-5 For those
in whom symptoms are due principally
to LVOTO and who don’t respond or
are intolerant to conventional medical
therapies, mechanical interventions
to expand the LV outflow tract may
be pursued. Dual-chamber pacing
he time-honored surgical
“ Ttreatment
option for patients
who have obstructive HCM
is septal myectomy.
”
4
The Sanger Report | www.sangerheart.org
F
igure 4
Gadolinium-enhanced MRI indicates regions of myocardial
fibrosis (arrows) involving the midventricular septum.11
Cardiovascular Disease:
Women’s Initiative
Images on pages 1 & 6 © iStockphoto/DSGpro • Image on page 5 © iStockphoto/ranplett • Image on page 6 © iStockphoto/Dorling_Kindersley
Cheryl A.
Russo, MD,
FACC
Adult
Cardiologist
Heart disease is the leading
during pregnancy, increasing
cause of death among American
a patient’s risk of CVD in the
women, accounting for greater than
future. Finally, a woman’s
one in four deaths in this population.
childbearing status must
In the United States, more women
be considered not only in
die of cardiovascular disease (CVD)
prevention, but also in
than do their male counterparts
diagnosis and treatment.
each year. Among minority popula-
In addition to preven-
tions, disease disparities are even
tion, the expanded literature
greater. These facts don’t represent
has increased our knowledge in
a recent trend, rather one that has
diagnosing CVD. We’ve learned
been apparent for decades. Only in
that a woman’s presentation with
the last 10 to 15 years has significant
CVD often differs significantly from
to taking the lead in understanding
awareness been brought to the sub-
that of a man. Women more often
CVD in women. We’re striving to pro-
ject of CVD in women. As recently as
present with chest pain, as compared
vide the women of our community
1997, a mere 30 percent of women
to a specific acute coronary event.
and beyond with the highest level of
polled were aware that heart disease
There may be a variance in the
cardiovascular care. Recognizing that
was the No. 1 threat to their lives.
quality of pain as well as atypical
the risk of CVD is a spectrum along
Over the next decade, awareness
symptoms such as fatigue or dysp­
which all women have a place, we‘re
nearly doubled. During this period,
nea. There’s an awareness that
prepared to guide women at all stages
the scientific literature grew exponen-
the sensitivity and specificity of
of their lives and personalize their
tially and confirmed the differences
diagnostic and prognostic studies
care. We have assembled a team of
between genders in all aspects of
are not equal between the genders.
specialists who focus on the cardio-
Finally, much data has been
vascular needs of women, including
gathered regarding the treatment
primary and secondary prevention,
of CVD in women. Studies reveal
disease diagnosis and delivery of
evidence-based guidelines for CVD
that women are less likely than men
care to assure optimal outcomes.
prevention in women were updated
to be referred for appropriate
We also offer Heart of a Woman
in 2007 to incorporate this new data.
therapeutic procedures. We’ve
Support Group, a joint effort between
Some noteworthy advancements
learned that as compared to men,
SHVI and Carolinas HealthCare
within these guidelines include:
women’s in-hospital mortality after
System. Our goal is to unite women
 generalizing the approach to risk
myocardial infarction is higher and
who have survived a cardiac event
assessment, which now reflects a
overall survival is lower. Some of
with women who are striving to
woman’s high lifetime risk of CVD
these differences in outcomes may
prevent similar experiences. Through
 augmenting risk assessment with
be explained by confounding
education, sharing of information
nontraditional risk factors
factors, such as older age at
and encouragement, participants
 updating recommendations for
presentation and higher number
are empowered with the knowledge
hormonal and aspirin therapy
of comorbidities in women.
required to begin or maintain a
However, the difference is clear.
heart-healthy lifestyle. For more infor-
CVD, including prevention, diagnosis,
treatment and outcomes.
The American Heart Association’s
Also unique to preventing CVD in
the female patient is consideration
of comorbidities that may occur
At Sanger Heart & Vascular
Institute (SHVI), we’re committed
mation, visit www.sangerheart.org
or call 704-373-0212.
www.sangerheart.org | The Sanger Report
5
How Common is Venous Disease?
Experts estimate that
between 5 and 30 percent of
the American population suffers
Felipe
Navarro, MD,
FACC, FSVM
Vascular
Interventionalist
What causes
varicose veins?
Contracting calf muscles propel
are usually aggravated by prolonged
standing or sitting (due to the immobility of muscle pump in the calves).
from chronic varicose veins.
blood against gravity. The venous
Female patients may experience more
Venous disease often results
valves are one-way valves that pro-
pronounced symptoms during their
in symptoms such as achiness,
mote blood flow toward the heart.
menses.
fatigue, swelling and pain in
As the weakened venous walls cause
the legs. These symptoms can
venous dilatation (most commonly
However, left untreated, varicose
interfere with a patient’s activities
in the greater saphenous vein), the
veins can lead to complications
of daily life. At least 20 percent of
valvular leaflets no longer meet
such as:
patients with venous disease will
properly, thereby becoming incom-
 Stasis pigmentation. This condi-
develop leg ulcers due to greater
petent. This allows reflux in the
tion presents as a discoloration of
saphenous vein reflux disease.
venous segment, which subsequently
the skin, often with swelling.
most common
“ Triskhe factor
for developing
varicose veins in females
is the presence of
progesterone, which
is thought to weaken
the venous walls.
”
Most varicose veins are benign.
further dilates the vein. Reflux is
 Stasis dermatitis. Commonly
then further propagated distally
mistaken for infectious cellulitis and
along the vein and its tributaries,
treated unsuccessfully with antibiotics,
becoming visible varicose veins.
stasis dermatitis often presents as
an erythematous patch of skin that’s
What are the risk factors?
intensely pruritic.
The most common risk factor
 Stasis ulcerations. These are
for developing varicose veins in
extremely painful and difficult to
females is the presence of proges-
treat. Successful treatment includes
terone, which is thought to weaken
compressive therapy coupled with
the venous walls. Pregnancy intensi-
elimination of refluxing superficial
fies the progesterone surge, causes
veins, if present. About 0.4 percent
venous hypertension and increases
of venous ulcers transform into
venous capacity. Other risk factors
malignant lesions.
include prolonged standing, hered-
Varicose veins can also bleed,
ity, obesity and aging. In some
either spontaneously or as a result
patients, several risk factors are
of direct trauma.
present; the female hairdresser, for
example (gender and prolonged
standing), with a family history of
varicose veins.
What are the treatment
options?
Medical management reduces
symptoms and prevents the develop-
What are the symptoms?
Patients most often complain of
6
The Sanger Report | www.sangerheart.org
ment of secondary complications.
Initial treatment options usually
achiness and a feeling of heaviness in
include compression therapy with
their legs. Other symptoms include
graded elastic compression stock-
restless legs, focal burning or pruritis
ings. Leg elevation can help alleviate
and leg swelling. A patient’s symptoms
edema and lower hydrostatic
surgical ligation and stripping,
B
EFORE
both treatments are equally
efficient in eliminating greater
saphenous reflux, alleviating
symptoms and signs of saphenous
varicosities and improving quality
of life. Other studies suggest that
ablation results in an earlier return
to normal activity.
References
1. Beebe-Dimmer JL, Pfeifer JR, Engle JS, et al.
The epidemiology of chronic venous insufficiency and varicose veins. Ann Epidemiol.
2005;15(3):175–184.
a
fter
2. Min RJ, Khillnani N, Zimmet SE. Endovenous
laser treatment of saphenous vein reflux:
Long-term results. J Vasc Intervent Radiol.
2003;14(8):991–996.
pressure. A graded exercise program
introduced into the distal aspect of
3. Kabnick LS. Outcome of different endovenous
helps rehabilitate muscle pump and
the refluxing vein and fed proximally
laser wavelengths for great saphenous vein
improves symptoms of venous insuf-
to within 2 cm of the saphenofemoral
ficiency in varicose veins.
ablation. J Vasc Surg. 2006;43(1):88–88.e7.
or saphenopopliteal junction. The
4. Merchant RF, Pichot O. Closure Study Group.
Many interventional treat-
procedure is performed with tumes-
Long-term outcomes of endovenous radiofrequency
ment options are also available.
cent anesthesia, which is a mixture
obliteration of saphenous reflux as a treatment of
Sclerotherapy is one option that
of 0.1 percent lidocaine in saline.
helps obliterate telangiectases,
This provides a heat sink to reduce
reticular veins and varicose veins.
skin burns, helps to collapse the vein
5. Rasmussen LH, Bjoern L, Lawaetz M, et al.
In the United States, sodium
around the catheter and eliminates
Randomized trial comparing endovenous laser
tetradecyl sulfate and sodium
procedural pain.
morrhuate are approved for treat-
superficial venous insufficiency. J Vasc Surg.
2005;42:502–509.
ablation of the great saphenous vein with high
Laser treatment with an 810-nm,
ligation and stripping in patients with varicose
veins. J Vasc Surg. 2007;46(2):308–315.
ment of varicose veins. The use of
a 940-nm or a 980-nm diode has
these agents in a foam preparation
shown excellent results with saphe-
6. Lurie F, Creton D, Eklof B, et al. Prospective
has not been approved by the FDA.
nous vein obliteration in 93 percent
randomized study of endovenous radiofrequency
Endovenous radiofrequency
and laser ablation therapies are
of cases at one and two years.
Radiofrequency treatment yields
recent advances in the treatment
complete occlusion in 87.2 percent
of varicose veins. They’re useful in
of cases and a reflux-free rate of
treating varicose veins that arise
83.8 percent of cases after a five-
from refluxing greater or lesser
year follow-up period. When com-
saphenous veins. Catheters are
paring endovenous ablation with
obliteration (closure) versus ligation and vein
stripping (EVOLVeS): Two-year follow-up.
Eur J Vasc Endovasc Surg. 2005;29(1):67–73.
7. Darwood RJ, Theivacumar N, Dellagrammaticas D,
et al. Randomized clinical trial comparing endovenous laser ablation with surgery for the treatment of
primary great saphenous varicose veins. Br J Surg.
2008;95(3):294–301.
www.sangerheart.org | The Sanger Report
7
e-logo_2options_v2.ai
The
The Sanger Report
1001 Blythe Blvd.
Suite 300
Charlotte, NC 28203
Printed With Soy Ink
PRSRT STD
U.S. POSTAGE
PAID
Sanger Heart &
Vascular Institute
Printed With Soy Ink
Please Recycle This Publication
Please Recycle This Publication
Printed With Soy Ink
Please Recycle This Publication
Copyright © 2011 Sanger Heart & Vascular Institute
Printed With Soy Ink
Please Recycle This Publication
www.sangerheart.org
Hypertrophic Cardiomyopathy
3. Nishimura RA, Ommen SR, Tajik AJ.
Continued from page 4
Hypertrophic cardiomyopathy: A patient
perspective. Circulation. 2003;108:e133–e135.
papillary muscles. These anomalies
contribute to LVOTO and residual
Reduced
outflow
Mitral
regurgitation
Septal
myectomy
obstruction, if not addressed.
4. Spirito P, Seidman CE, McKenna WJ,
et al. The management of hypertrophic
cardiomyopathy. N Eng J Med.
Increased
outflow
Surgical techniques include
1997;336(11):775–785.
extensive resection from trigone
5. Nishimura RA, Holmes DR. [Clinical Practice]
to trigone, extending deep in the
Hypertrophic obstructive cardiomyopathy.
ventricular cavity to the base of both
papillary muscles. Care is taken to
avoid injury to the aortic valve or
N Eng J Med. 2004;350(13):1320–1327.
Incision line
through hypertrophied
basal septum
6. Maron BJ. Contemporary insights
conduction tissue or creation of a
F
igure 5
ventricular septal communication.
Surgical septal
and prevention of sudden death
Residual systolic anterior motion over
reoperation for residual
myectomy
in hypertrophic cardiomyopathy.
the medial papillary muscle of the
or recurrent obstruction
mitral valve and midcavitary residual
is less than 10 percent at eight to 10
7. Fifer MA, Vlahakes GJ. Management of symp-
obstruction can be avoided by
years. Importantly, symptomatic status
toms in hypertrophic cardiomyopathy. Circulation.
adequate resection. Barring intrinsic
is dramatically improved, with more
2008;117(3):429–439.
pathology, mitral valve intervention
than 50 percent of patients reporting
can be avoided with the expectation
functional Class I status after surgery
of improvement of mitral insufficiency
and the remainder improving one to
on postoperative studies.
two functional classes.2,3,5
In experienced hands, with proper
and strategies for risk stratification
5
Circulation. 2010;121(3):445–456.
8. Force T, Bonow RO, Houser SR, et al. Research
priorities in hypertrophic cardiomyopathy. Report
of a working group of the National Heart, Lung,
and Blood Institute. Circulation. 2010;122:1130-1133.
For more information about the
myocardial protection, surgical risks
services available at Sanger Heart &
9. Bos JM, Towbin JA, Ackerman MJ. Diagnostic,
are minimal with an initial mortality
Vascular Institute or to schedule an
prognostic and therapeutic implications of genetic
of less than 1 percent and successful
appointment, call 877-999-SHVI (7484)
testing for hypertrophic cardiomyopathy. J Am Coll
relief of LVOTO with absent gradients.
or visit www.sangerheart.org.
Surgical pacemaker placement is
necessary in only about 3 percent
of patients with normal conduction
References
1. Maron BJ. Hypertrophic cardiomyopathy: A
systematic review. JAMA. 2002;287(10):1308–1320.
preoperatively but increases to
30 percent if there is intraventricular
conduction delay or 70 percent
if right bundle branch block is
present prior to myectomy. Risk of
Cardiol. 2009;54(3):201–211.
10. Maron BJ, Estes NAM III, Maron MS, et al.
Primary prevention of sudden death as a novel
treatment strategy in hypertrophic cardiomyopathy.
Circulation. 2003;107(23):2872–2875.
2. Maron BJ, McKenna WJ, Danielson GK, et al.
American College of Cardiology/European Society
11. Maron MS, Maron BJ, Harrigan C, et al.
of Cardiology, Clinical expert consensus document
Hypertrophic cardiomyopathy phenotype revisited
on hypertrophic cardiomyopathy. J Am Coll Cardiol.
after 50 years with cardiovascular magnetic
2003;42(9):1687–1713.
resonance. J Am Coll Cardiol. 2009;54(3):220–228.