Download Pictorial Essay: Sinus of Valsalva Anatomy and Pathological

Pictorial Essay: Sinus of Valsalva Anatomy and Pathological
Conditions Diagnosis by Multi-Detector CT
Award:
AOSR Best Exhibit Prize - Silver
Poster No.:
R-0211
Congress:
RANZCR-AOCR 2012
Type:
Educational Exhibit
Authors:
W. K. Tsang, A. Li
Keywords:
Cardiac, Cardiovascular system, CT, CT-Angiography, Computer
Applications-Detection, diagnosis, Education and training
DOI:
10.1594/ranzcraocr2012/R-0211
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Learning Objectives
After reading this presentation, the reader will be able to:
•
•
Understand the basic anatomy of the sinus of Valsalva and aortic root
Recognize various pathological conditions of the Sinus of Valsalva and
aortic root
Background
INTRODUCTION
Multi-detector row CT (MDCT) cardiac imaging has emerged to be useful in the
assessment of the anatomy and function of the heart. Apart from its established role in
the evaluation of the coronary arteries, MDCT can also accurately assess the sinuses
of Valsalva and aortic root. This pictorial essay serves to present the normal anatomy of
Sinuses of Valsalva and aortic root, as well as their various pathological conditions.
NORMAL ANATOMY
The aortic root (Fig. 1 on page 3)is the outflow tract from the left ventricle. It
surrounds and supports the aortic valvular leaflets, and connects the basal attachments
of the leaflets within the left ventricle to the sinotubular junction. The anatomic ventriculoaortic junction is a circular locus within the aortic root, formed where the supporting
ventricular structures give way to the fibro-elastic walls of the aortic sinuses.
Aortic sinuses, or sinuses of Valsalva (SOV) (Fig. 2 on page ,3)are three subtle
dilations between the aortic valve annulus and the sinotubular ridge. Two of the SOV
give rise to the coronary arteries and thus nominated as the right and left coronary aortic
sinuses. The posterior sinus does not give rise to a coronary artery, therefore called the
non-coronary aortic sinus. SOVs are important in allowing enough space for the aortic
valve leaflets to open during systole without causing occlusion of the coronary artery
ostia. They also prevent damage of the valve leaflets caused by direct striking of the
aortic root wall.
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Images for this section:
Fig. 1: Normal aortic root. Oblique coronal image of MDCT coronary angiogram shows
the structures at aortic root and proper measurements to be made. 1 = annulus, 2= sinus
of Valsalva, 3 = sinotubular junction, 4 = ascending aorta, red arrow = valve commissures.
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Fig. 2: Normal SOVs. Oblique axial image of MDCT coronary angiogram obtained at the
level of the aortic valve shows the SOVs and the coronary arteries. R = right coronary
cusp, L = left coronary cusp, N= noncoronary cusp, RCC = right coronary artery, LCC
= left coronary artery
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Table 1: Normal dimensions of aortic root structures.
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Imaging Findings OR Procedure Details
SINUS OF VALSALVA AND AORTIC ROOT PATHOLOGY
1. SINUS OF VALSALVA ANEURYSM (SVA)
Sinus of Valsalva aneurysm (SVA) is a rare condition which is related to weakness of the
elastic connective tissue at the junction of the aortic media and the annulus. Congenital
causes including underlying deficiency of normal elastic tissue like in those with Marfan
and Ehlers-Danlos syndromes. Acquired SVAs are most commonly related to infection,
degeneration or trauma.
Nonruptured SVAs may be asymptomatic, or they may present acutely with mass effect
on adjacent cardiac structures. Ruptured SVAs result in aortocardiac shunt and may
manifest as progressive congestive heart failure, severe acute chest pain with dyspnoea,
or even cardiac arrest. In general, symptomatic or large SVAs should be repaired to avoid
complications, whereas small asymptomatic SVAs may be monitored.
Illustrative cases
•
SVA with Patulous Right and Left Coronary Cusps
A 48-year-old lady diagnosed with aortic regurgitation underwent echocardiogram as
preoperational assessment of operation of varicose veins, which showed dilated aortic
root with large sinus of Valsalva aneurysm and moderate to severe aortic regurgitation
due to dilated aortic root. CT coronary angiography and thoracic aorta demonstrated
patulous right and left coronary cusps and a wide-neck SVA of the non-coronary cusp
(Fig. 3 on page 10 Fig. 4 on page 11 Fig. 5 on page 13). She underwent
replacement of the aortic valve, aortic root and ascending aorta three months later. She
was all along well in the subsequent 3 years post operative follow up period.
•
Acute Ruptured SVA
A 49-year-old gentleman presented with haemoptysis during his treatment for
suspected tuberculous spondylodiscitis. Chest radiograph showed congested lung fields.
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Echocardiogram revealed flailed aortic valve with moderate aortic regurgitation, aortic
valvular leaflets thickening and vegetations. Features were compatible with infective
endocarditis. CT coronary angiogram showed prolapsed aortic valve and a wide-neck
aneurysm with lobulated contour arising from the undersurface of the right coronary
sinus. A fistula connecting the basal aspect of the aneurysm running to the right atrium
was noted (Fig. 6 on page 13). Overall features were consistent with ruptured SVA
with fistula to right atrium. He then underwent emergency open heart surgery with
drainage of perivalvular abscess, closure of the fistula and aortic valvular replacement.
Post-operative recovery was stormy. Patient eventually succumbed due to disseminated
intravascular coagulation.
2. SOV PSEUDOANEURYSM
Infective SOV pseudoaneurysm is a rare condition resulting from extension of infection
from a paravalvular myocardial abscess complicating endocarditis. Clinically patients
have insidious onset of vague symptoms. Complications include rupture or formation of
fistula to right heart with left to right shunt. Prognosis is poor if untreated.
Illustrative case
•
SOV Pseudoanerusym with Aortic Root Abscess
A 30-year-old gentleman with history of substance abuse and gonorrhoea presented with
subacute onset of dyspnoea. Physical examination found ejection diastolic murmur at the
left lower sternal border. Coronary CT angiogram demonstrated a huge narrow-necked
contrast-filled sac arising from the left SOV, suggestive of SOV pseudoaneurysm (Fig.
7 on page 14). Soft tissue infiltrates in the mediastinal spaces surrounding the aortic
arch and roots of the great vessels raised the suspicion of mediastinitis and aortic root
micro-abscesses (Fig. 8 on page 15). Non-specific multifocal ground-glass opacities
and interstitial thickening were present in the included lung fields (Fig. 9 on page 16),
which could represent pulmonary edema related to acute heart failure or concurrent chest
infection. Emergency aortic valvular replacement was performed. Unfortunately, valve
dehiscence occurred and the patient underwent another open heart surgery within two
months. He finally succumbed four months later due to irreversible left ventricular failure.
3. BICUSPID AORTIC VALVE
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Bicuspid aortic valve (BAV) is the most common congenital anomaly of the aortic valve,
resulting from complex abnormal cusp formation during valvulogenesis. Since BAV
causes premature fibrosis and calcification of the aortic valves, aortic stenosis is the most
common complication. The classical bicuspid valve shows two symmetric aortic cusps.
During diastole, the open bicuspid aortic valve assumes a typical ellipsoid shape.
Aortic stenosis from bicuspid valve presents at an age range of 30 to 50 years, earlier
than those caused by degeneration. In the evaluation of patients with bicuspid valves
that require valve repair, the degree of calcification of the valve is important as coronary
implantation and aortic root replacement may be indicated in those with significant
calcification.
Illustrative case
•
BAV with Ascending Aortic Aneurysm
A 53-year-old lady with known history of moderate aortic stenosis and severe aortic
regurgitation was noted to have dilated aortic root of in echocardiogram. Cardiac CT was
arranged for preoperational planning of aortic graft during aortic vulvular replacement,
which showed thickened calcified bicuspid aortic valve with incomplete closure (Fig.
10 on page 17). Fusiform ascending aortic aneurysm and concentric left ventricular
hypertrophy were also revealed (Fig. 11 on page 18). Aortic valvular replacement was
arranged. Four months later, before receiving the surgery, she presented with acute chest
pain. Urgent CT thorax demonstrated Standford type A aortic intramural heamatoma (Fig.
12 on page 19). Cardiothoracic team assessed her and decided not for urgent open
heart surgery in view of her unstable condition. The patient condition deteriorated rapidly.
She succumbed two days after admission.
4. CORONARY ARTERY ANOMALIES
Congenital anomalies of the coronary arteries are uncommon yet important causes of
chest pain, with prevalence of 0.3-1% in general population. They can be classified
anatomically according to their origin, course, and termination. They could also be divided
as hemodynamically significant or insignificant (Table 2 on page 20). The former ones
are characterized by abnormalities of myocardial perfusion, which lead to an increased
risk of myocardial ischemia or sudden death.
Illustrative cases
Page 8 of 27
•
Anomalous Right Coronary Artery with Malignant Course
CT coronary angiogram of a 57-year-old lady who presented with chest pain revealed
the common origin of both the right and left coronary arteries (RCA and LCA) from
the left coronary cusp (Fig. 13 on page 20). The proximal RCA ran between the
pulmonary trunk and aortic root, consistent with anomalous origin of RCA with "malignant"
interarterial course (Fig. 14 on page 21). The patient deferred the option of operation.
No record of adverse cardiac event was noted in the subsequent 2-year follow up period.
•
Separate Left Anterior Descending and Left Circumflex Arteries
A 79-year-old lady with history of aortic valvular replacement due to aortic stenosis
developed silent anterior territory myocardial ischemia soon after she underwent left
mastectomy for treatment of breast carcinoma. Coronary CT angiogram revealed
seperate ostia of left anterior descending (LAD) and left circumflex arteries (LCx)
arising from the left coronary sinus (Fig. 15 on page 22), a normal variant
without hemodynamic effect upon coronary perfusion. No adverse cardiac event was
documented in the subsequent 4 years.
5. CORONARY ARTERY ANEURYSM WITH ASSOCIATED FISTULA
(CAAAF)
Approximately 3% of coronary artery fistulas (CAFs) are associated with coronary artery
aneurysm (CAA), a rare condition with only about 50 cases have been reported in the
English literatures. It is congenital in most cases. Acquired causes include complications
of previous open heart surgery, endomyocardial biopsy, percutaneous transluminal
coronary angioplasty (PTCA), myocardial infarction and trauma. One-third of the patients
with CAAAF are asymptomatic, and two-third of patients may experience symptoms such
as angina, dyspnoea on exertion or palpitation. Aneurysm repair, closure of fistula and
coronary artery bypass graft are the definitive treatment opinions.
Illustrative case
•
Right Coronary Artery Aneurysm with associated Fistula
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No abnormality was detected in thallium scan and echocardiogram of a 73-year-old
gentleman who presented with chest pain. Further workup with CT coronary angiogram
showed a huge fusiform aneurysm arising from the proximal RCA (Fig. 16 on page
23) with direct drainage into the coronary sinus and the inferior aspect of the heart
(Fig. 17 on page 24). Features are compatible with right coronary artery aneurysm
with associated coronary artery fistula (CAAAF). The patient subsequently underwent
left and right cardiac catheterization, which confirmed the presence of a 3cm proximal
right coronary artery aneurysm which drained into the coronary sinus. Cardiothoracic
surgery referral was arranged. However, the option of definite surgery was declined by
patient despite the risk of rupture or thrombosis of aneurysm explained. Fortunately he
was stable in the subsequent 3 years without major adverse cardiac events.
Images for this section:
Page 10 of 27
Fig. 3: Oblique axial image obtained at the level of the aortic valve, demonstrates
patulous right (R) and left (L) coronary cusps and a wide-neck SVA of the non-coronary
cusp (N).
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Fig. 4: Oblique sagittal image of MDCT coronary angiogram, showing wide-neck SVA of
the non-coronary cusp (N).
Fig. 5: CT coronary angiogram with volume rendered image, demonstrating patulous
right (R) and left (L) coronary cusps and a wide-neck SVA of the non-coronary cusp (N).
Page 13 of 27
Fig. 6: CT coronary angiogram with multiplanar reconstruction in oblique coronal view
demonstrates sinus of valvalsa aneurysm (*) with contrast jet (arrow) directing towards
the right atrium (RA), consistent with ruptured SVA aneuerysm with fistula to right atrium.
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Fig. 7: Axial MDCT image showing a huge narrow-necked contrast-filled sac (*) from the
left SOV (L), suggestive of SOV pseudoaneurysm.
Page 15 of 27
Fig. 8: Soft tissue infiltrates (arrowheads) in the mediastinal spaces surrounding the
aortic arch and root of the great vessels raise the suspicion of mediastinitis and aortic
root micro-abscesses.
Page 16 of 27
Fig. 9: Non-specific multifocal ground-glass opacities in the included lung fields can
represent pulmonary edema related to acute heart failure or concurrent chest infection.
Page 17 of 27
Fig. 10: CT coronary angiogram with multiplanar reconstruction image in oblique sagittal
view, demonstrates bicuspid aortic valve with thickening and calcification (arrowheads).
Page 18 of 27
Fig. 11: CT coronary angiogram with multiplanar reconstruction image, demonstrates
bicuspid aortic valve with thickening and calcification (arrow), fusiform ascending aortic
aneurysm (AA) and concentric left ventricular hypertrophy (arrowheads).
Page 19 of 27
Fig. 12: Pre contrast CT thorax. Axial image at the level of aortic arch shows standford
type A aortic intramural heamatoma.
Table 2: Coronary Artery Anomalies. Those highlighted in red are hemodynamically
significant which could lead to abnormal myocardial perfusion and even sudden death.
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Fig. 13: Common origin of RCA & LCA from the left coronary cusp (L).
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Fig. 14: CT coronary angiogram with volume rendered image. Common origin (black
arrow) of RCA & LCA (*) from the left coronary cusp. The proximal RCA ran between the
pulmonary trunk (P) and aortic root (A), consistent with "malignant" interarterial course
(arrowheads).
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Fig. 15: Separate ostia of LAD and LCx from left coronary sinus. This is considered as
normal variant without hemodynamic effect upon coronary perfusion.
Page 23 of 27
Fig. 16: A huge fusiform aneurysm (* ) arising from the proximal RCA (arrowhead). R =
right coronary cusp, L = left coronary cusp, N = non-coronary cusp.
Page 24 of 27
Fig. 17: CT coronary angiogram with volume rendered image, showing a 5cm right
coronary artery (RCA) aneurysm (*) near the ostium of the RCA (arrows). Also of note
is presence of coronary artery fistula (arrowheads) that subsequently drains via the
coronary sinus into the right atrium.
Page 25 of 27
Conclusion
Pathological conditions of Sinus of Valsalva and aortic root are rare yet important.
The clinical manifestations vary from asymptomatic incidental finding to acute rapidly
deteriorating course and death. Surgical repair is the mainstay of treatment, which can
be lifesaving in some conditions. Therefore a prompt and accurate diagnosis of the
disease is paramount. Multi-detector row CT (MDCT) cardiac imaging is excellent in
demonstrating the anatomy and function of the heart, and also a powerful tool in diagnosis
of various pathology of the sinus of Valsalva and aortic root.
Personal Information
Dr Tsang Wai Kan graduated from the Chinese University of Hong Kong in 2006.
She became a Fellow of the Royal College of Radiologists (UK) in 2011. She is now
receiving her higher training in Tuen Mun Hospital, Hong Kong. Her major interests
include musculoskeletal radiology, interventional radiology and cardiovascular imaging.
Dr Allen Li graduated from the Chinese University of Hong Kong in 1998. He is now the
consultant radiologist in North District Hospital, Hong Kong. He is also the organizer of
the MRI training program in his center. His major interests include cardiovascular imaging
and chest radiology.
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in patients undergoing percutaneous aortic valve replacement: Evidence of aortic root
remodeling. Journal of Thorac. Cardiovasc. Surg. 2009;137:950-956.
4. P Nataf, E Lansa. Dilation of the thoracic aorta: medical and surgical management.
Heart 2006;92:1345-1352
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