Download Sinus of Valsalva Aneurysm with Rupturing into the Right Atrium ¾ A

Ruptured Sinus of Valsalva Aneurysm
Case Report
Acta Cardiol Sin 2006;22:96-101
Sinus of Valsalva Aneurysm with Rupturing into
the Right Atrium ¾ A Case Report and Review
of the Literature
Chao-Chien Chang,1 Chih-Hui Chin,1 Meng-Ling Chen,1 Thay-Hsiung Chen2 and Hung-Shun Lo1
Sinus of Valsalva aneurysm accounts for only 1% of congenital cardiac anomalies. Sinus of Valsalva aneurysm can
cause aortic insufficiency, coronary artery flow compromise, cardiac arrhythmia, or aneurysm rupture. A 44year-old male presented with a 3-month history of palpitation and exertional dyspnea. Cardiac auscultation revealed
a grade III/VI ‘‘to and fro’’ murmur along the left sternal border. Electrocardiography showed ventricular bigeminy.
Transthoracic and transesophageal echocardiography revealed an aneurysm of the right sinus of Valsalva rupturing
into the right atrium. Aortography also confirmed the presence of an aneurysm of the right coronary sinus, extending
into the right atrium. The patient underwent open heart surgery to close the ostium of the aneurysm and to suture the
right atrial fistula directly. The patient was well upon follow-up after the operation.
Key Words:
Sinus of Valsalva aneurysm · Right atrium · Transthoracic echocardiography ·
Transesophageal echocardiography
INTRODUCTION
most of the patients.
We present a rare case of right sinus of Valsava
aneurysm that ruptured into the right atrium. The usefulness of various diagnostic methods, as well as the surgical options, are also discussed.
Sinus of Valsalva aneurysm, also known as coronary
sinus fistula, is a rare cardiac abnormality. Aneurysms,
either congenital or acquired, arise from a discontinuity
between aortic tunica media and aortic valve annulus.
Sinus of Valsalva aneurysms can coexist with other congenital disease and can rupture into the cardiac chambers,
causing acute clinical symptoms. The right coronary sinus is the commonest site of aneurysm formation.1 The
majority of right coronary cusp aneurysms rupture into
the right ventricle.2 Rupture of sinus of Valsalva aneurysm can be easily diagnosed by echocardiography.
Early diagnosis and surgical treatment can save lives for
CASE PRESENTATION
A 44-year-old man with hypertension was admitted
with chief complaints of sudden-onset palpitations, light
headedness, and breathlessness. On examination, he was
apyrexial, with blood pressure of 166/70 mm Hg and
pulse rate of 110 beats per minute. He had grade III/VI
‘‘to and fro’’ murmur along the left sternal border. The
electrocardiogram showed sinus rhythm with ventricular
bigeminy, and chest X-ray showed cardiomegaly. Full
blood count and cardiac enzymes were normal. The patient had a transthoracic echocardiography (TTE) a day
later, which showed dilatation of four chambers with
good systolic function, and no vegetations or valvular
abnormalities. An aneurysmal dilation of the right sinus
Received: October 21, 2005 Accepted: December 23, 2005
1
Divisions of Cardiology and 2Cardiac Surgery, Cathay General
Hospital, Taipei, Taiwan.
Address correspondence and reprint requests to: Dr. Hung-Shun
Lo; Division of Cardiology, Department of Medicine, Cathay
General Hospital, 280, Section 4, Jen-Ai Road 106, Taipei, Taiwan.
Tel: 886-2-2708-2121 ext. 3118; Fax: 886-2-2932-4969; E-mail:
[email protected]
Acta Cardiol Sin 2006;22:96-101
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Ruptured Sinus of Valsalva Aneurysm
aneurysm had ruptured into the right atrium (Figure 3).
The patient was referred for surgical repair of the ruptured sinus. The patient recovered completely and has
been well for three years till the present time.
of Valsalva protruded into the right atrium with a
turbulent-color Doppler flow. Additionally, a systolicdiastolic flow was presented in the continuous-wave
Doppler (Figure 1); ruptured sinus of Valsalva aneurysm
was highly suspected. Transesophageal echocardiography (TEE) confirmed the presence of a ruptured right
sinus Valsalva aneurysm (Figure 2). Urgent cardiac
catheterization revealed elevated right heart pressures
and a step-up in oxygen saturations at the right atrial
level. Ascending aortic pressure was 170/60 mm Hg.
The aortogram confirmed that the right sinus of Valsalva
DISCUSSION
The three sinuses of Valsalva are located in the most
proximal portion of the aorta, just above the cusps of the
aortic valve; the sinuses end in the areas of the sino-
Figure 1. Transthoracic echocardiography revealed a “windsock” dilatation of the right sinus of Valsalva rupturing into the right atrium from
parasternal short-axis view (A) and a systolic-diastolic continuous flow (B).
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Acta Cardiol Sin 2006;22:96-101
Chao-Chien Chang et al.
Figure 2. Transesophageal echocardiography confirmed the presence of a ruptured right sinus Valsalva aneurysm.
Figure 3. The aortogram confirmed patency of the right coronary artery, and that the right sinus of Valsalva aneurysm had ruptured into the right atrium.
aortic media and the annulus fibrosis of the aortic valve.3
Acquired sinus of Valsalva aneurysms can exist in patients with endocarditis, 4 syphilis, 1 injury, 5 Bechet’s
tubular junction. Causes of sinus of Valsalva aneurysm
are classified into congenital or acquired. Congenital
type occurs where a lack of fusion exists between the
Acta Cardiol Sin 2006;22:96-101
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Ruptured Sinus of Valsalva Aneurysm
disease 6 and Marfan’s syndrome. 7 Deficiency of the
aortic media at the attachments on the aortic annulus
portion may progressively lead to dilation of aortic
sinus, usually over many years. Therefore, sinus of Valsalva aneurysm can remain subclinical for several years
until it eventually ruptures between the 3rd and 4th
decades of life. The commonest sites of aneurysm ruptures are cardiac chambers, interventricular septum,8 or
pericardial space.9 Dilated sinus of Valsalva aneurysm
also can lead to distortion of aortic valve and aortic regurgitation.
Un-ruptured sinus of Valsalva aneurysms usually do
not cause any symptom and are often found during echocardiogram examination and cardiac catheterization.
However, un-ruptured sinus of Valsalva aneurysm may
cause right ventricular outflow tract obstruction10 and
myocardial ischemia due to the mass compression effect
of aneurysms.11 Symptomatic aortic regurgitation (AR)
can occur in cases with progressive dilation of sinus of
Valsalva aneurysm.
The ruptured sinus of Valsalva aneurysms most often
involve with the right coronary sinus (76.8 to 83.3%) or
with other non-coronary sinus, while an aneurysm of the
left sinus is rare.2,12 Most right sinus of Valsalva aneurysms rupture into the right ventricle (86.7%),2 or into
the right atrium. Patients may remain asymptomatic for
several years even if the sinus of Valsalva aneurysms has
ruptured. However, dyspnea and exercise intolerance
may develop because of the increased shunting and volume overloading. Sudden massive rupture may result in
acute chest pain and dyspnea. Patients with a smaller insidious rupture may be asymptomatic, but also may be
associated with progressive symptoms of exertional
dyspnea and heart failure. In general, the in-and-out
blood flow from a ruptured sinus of Valsalva aneurysm
can produces a continuous murmur. A diastolic murmur
of aortic regurgitation may be heard if aortic cusp dilation occurs. If physical findings are suggestive of a sinus
of Valsalva aneurysm, patients may be evaluated using a
combination of TTE, TEE, magnetic resonance imaging
and cardiac catheterization. Traditionally, the gold standard for the diagnosis of ruptured sinus of Valsalva
traditionally is cardiac catheterization. The cardiac catheterization is used to detect the ruptured site and to
establish the patency of coronary artery. With the advancement of newer-generation echocardiographic ma-
chines, TTE and TEE have played an important role in
the diagnostic confirmation of Valsalva aneurysm sinus.13
TTE and TEE can investigate the proximal aorta,
sinuses, aortic valve, and surrounding structures. Doppler findings may identify the exact shunt location. The
“windsock” dilatation of sinus of Valsalva aneurysm is
the feature of sinus of Valsalva aneurysm. Doppler features include a continuous high velocity flow from the
aorta to the heart chamber. The diagnosis usually is
confirmed by color Doppler flow mapping. The color
Doppler flow mapping may reveal a continuous mosaic
flow jet from the aneurysm to the heart chamber.
Continuous rotation of a multiplane TEE may be particularly helpful to identify the exact point of rupture.
Echocardiography is also useful for the differential
diagnosis of other disorders, such as patent ductus arteriosus, coronary fistula, or aortopulmonary window
that cause similar continuous murmur. Additionally,
echocardiography can provide information for coexisting AR.
No specific medical therapy exists for sinus of Valsalva aneurysm. Treatment of congestive heart failure
may be required if rupture of the aneurysm does occur.
Additionally, concurrent cardiac arrhythmias and infectious endocarditis should be treated. Surgical repair of
the ruptured sinus of Valsalva aneurysm is the definitive
treatment. In 2001, Vural et al. reported the therapeutic
categories for sinus of Valsalva aneurysm.14 Surgical repair was required for both ruptured sinus of Valsalva
aneurysm and for symptomatic unruptured aneurysm. If
the size of the unruptured aneurysm is greater than 50%
of the other two normal coronary sinuses or is increased
in the consecutive echocardiographic examinations, surgical intervention also should be considered. Surgery is
also required for the unruptured aneurysm compressing
to the adjacent tissues.
Surgical treatments for sinus of Valsalva aneurysm
include direct suturing of the aneurysm or suturing with
a patch.15 Attempts should be made to preserve the aortic
valve, if possible. However, aortic valve replacement
may be necessary when AR is significant. Patients receive surgical repair for sinus of Valsalva aneurysm
usually have low operative risk and high long-term survival rates.16 Therefore, an early surgical intervention
should be encouraged.
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REFERENCES
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sinus of Valsalva into the left ventricle: report and review the
literature. J Am Soc Echocardiog 1997;10:862-865.
2. Shah RP, Ding ZP, Ng ASH, Quek SSS. A ten-year review of
ruptured sinus of valsalva: clinico-pathological and echo-Doppler
features. Singapore Med J 2001;42:473-476.
3. Henze A, Huttunen H, Bjork VO. Ruptured sinus Valsalva
aneurysms. Scand J Thorac Cardiovasc Surg 1983;17:249-253.
4. Shumacher JHB. Aneurysms of the aortic sinuses of Valsalva
due to bacterial endocarditis, with special reference to their
operative management. J Thorac Cardiovasc Surg 1972;
63:896-902.
5. Murray EG, Minami K, Kortke H, et al. Traumatic sinus of
Valsalva fistula and aortic valve rupture. Ann Thorac Surg
1993;55:760-761.
6. Koh KK, Lee KH, Kim SS, et al. Ruptured aneurysm of the sinus
of Valsalva in a patient with Bachet’s disease. Int J Cardiol
1994;47:177-179.
7. Knight JL, Jacka M, Charrette JP. Repair of isolated, symptomatic, sinus of Valsalva aneurysm in a patient with Marfan’s
syndrome. Can J Cardiol 1988;4:214-216.
8. Choudhary SK, Bhan A, Reddy SC, Sharma R, et al. Aneurysms
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of sinus of Valsalva dissecting into interventricular septum. Ann
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Biabnam KR, Roberts WC. Fatal intrapericardial rupture of sinus
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Tami LF, Turi ZG, Arbulu A. Sinus of Valsalva aneurysms
involving both coronary ostia. Cathet and Cardiovasc Diagn
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Chue SH, Hung CR, How SS. Ruptured aneurysm of the sinus
Valsalva in oriental patients. J Thorac Cardiovasc Surg 1990;
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Wang KY, St John SM, Ho HY, Ting CT. Congenital sinus of
Valsalva aneurysm: a multiplane transesophageal experience. J
Am Soc Echocardiog 1997;10:956-963.
Vural KM, Sener E, Tasdemir O, Bayazit K. Approach to sinus of
Valsalva aneurysms: a review of 53 cases. Eur J Cardio Thorac
Surg 2001;20:71-76.
Pannu HS, Shivaprakash K, Bazez S. Geographical variations in
the presentation of ruptured aneurysms of sinuses of Valsalva:
evaluation of surgical repair. J Card Surg 1995;10:316-324
Takach TJ, Reul GJ, Duncan JM, et al. Sinus of Valsalva
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Case Report
Acta Cardiol Sin 2005;21:96−101
右主動脈竇動脈瘤破裂至右心房
 病例報告及文獻回顧
張釗監 1 秦志輝 1 陳孟麟 1 陳瑞雄 2 羅鴻舜 1
台北市 國泰綜合醫院 心臟內科1 心臟外科2
主動脈竇動脈瘤是少見的，大都破裂後才被發現。一位 44 歲男性，有 3 個月心悸及運動時
呼吸困難病史，心臟聽診在胸骨左緣聽到“去返性”心雜音，心電圖顯示心室性期外收縮，
胸前及經食道超音波發現右主動脈竇動脈瘤破裂至右心房，主動脈攝影確定右主動脈竇動脈
瘤破裂至右心房，冠狀動脈攝影是正常的。患者於開心手術中接受動脈瘤開口縫合，患者術
後狀況良好。
關鍵詞：主動脈竇動脈瘤、右心房、胸前心臟超音波、經食道心臟超音波。
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