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CASE REPORT
Year : 2009  |  Volume : 10  |  Issue : 4  |  Page : 174-176 Table of Contents     

Large unruptured sinus of valsalva aneurysm burrowing into the interventricular septum


1 MBBch, F.E.S.C, Cardiology and Cardiothoracic Surgery Department Hamad Medical Corporation, Doha, Qatar
2 MBBch, Cardiology and Cardiothoracic Surgery Department Hamad Medical Corporation, Doha, Qatar
3 M.D, Cardiology and Cardiothoracic Surgery Department Hamad Medical Corporation, Doha, Qatar

Date of Web Publication17-Jun-2010

Correspondence Address:
Sherif M Helmy
MBBch, F.E.S.C, Cardiology and Cardiothoracic Surgery Department Hamad Medical Corporation, Doha
Qatar
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Source of Support: None, Conflict of Interest: None


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How to cite this article:
Helmy SM, Shaaban A, Chaikouni A, Hajar R. Large unruptured sinus of valsalva aneurysm burrowing into the interventricular septum. Heart Views 2009;10:174-6

How to cite this URL:
Helmy SM, Shaaban A, Chaikouni A, Hajar R. Large unruptured sinus of valsalva aneurysm burrowing into the interventricular septum. Heart Views [serial online] 2009 [cited 2019 Sep 18];10:174-6. Available from: http://www.heartviews.org/text.asp?2009/10/4/174/63684


   Introduction Top


Aneurysms of the sinus of valsalva are thin-walled outpouchings most commonly involving the right or non-coronary sinuses. Because they are asymptomatic, they are rarely discovered before they rupture and form aorto-cardiac fistula. We present a rare case of unruptured aneurysm of the right coronary sinus of valsalva burrowing into the interventricular septum with severe aortic incompetence and left ventricular dysfunction.


   Case presentation Top


A 41-year-old male Nepali presented to the Emergency Department and was admitted because of sudden onset of shortness of breath associated with profuse sweating, lasting a few hours. There were no other associated symptoms such as palpitations or chest pain. There was no history of chest trauma or fever.

On physical examination, the patient was alert, afebrile, with elevated JVP; BP 110/70mmHg and HR 90/min. Chest examination revealed bilateral normal air entry with crepitations in the lower and mid-zones. Cardiac examination showed apical displacement to the 6th intercostal space, Grade II/VI pansystolic murmur over the apex, and Grade IV/VI early diastolic murmur over the second intercostals space. Abdominal and neurological examinations were normal. ECG showed sinus tachycardia with first degree AV block and RBBB [Figure 1]. Chest x-ray showed cardiomegaly with bilateral hilar congestion. BNP was markedly elevated (13,698; normal 0- 85).

Doppler echocardiography showed aneurysmal dilatation of the right sinus of valsalva. The aneurysm burrowed into the interventricular septum with bulging into the right ventricle [Figure 2],[Figure 3] and [Figure 4]. The aneurysm also bulged into the right and left atrial cavities [Figure 3]. A mural thrombus was visualized within the right atrial portion [Figure 3]. The aortic annulus was deformed and the right coronary cusp showed malcoaptation [Figure 4]. There was severe aortic incompetence. The left ventricle was markedly dilated with severe impairment in global contractility (EF 28%). There was no evidence of rupture of the aneurysm. The origin of the RCA was visualized posterior to the aneurysmal sinus of valsalva. Left coronary artery origin was also visualized. There was mild central mitral incompetence and mild left atrial dilatation. The right ventricle and right atrium were normal in dimensions. RVSP was 41mmHg.

Patient was referred for urgent surgery. Coronary angiography prior to surgery confirmed the diagnosis and showed normal left coronary artery. The right coronary artery was visualized with difficulty as a small vessel.

At surgery, the right aortic valve cusp was rudimentary, thin and fibrotic. About 2.0 mm under the right coronary ostium, there was 2.0 x 1.0 cm opening of the aneurysm which led to a tri-lobulated cavity burrowing into the interventricular septum and containing old fixed mural clots. The largest cavity measured 3.0 x 3.0 x 3.0 cm. The aortic side of the aneurysm opening was closed with a Dacron patch sutured with 6/0 prolene avoiding the conduction system area and preserving the right coronary ostium. The aortic cusps were excised and a Sorin mechanical bileaflet aortic valve (# 25) was sutured to the aortic annulus with interrupted pledgeted stitches.

Patient had a stormy post-operative course. He developed inferior myocardial infarction, hypotension, bradycardia and ventricular fibrillation which were managed appropriately. Follow up echocardiography showed well-seated aortic valve prosthesis and the residual sinus of valsalva aneurysm within the interventricular septum with an organized thrombus. The ejection fraction improved from 28% to 38%. The patient was eventually discharged in stable condition and followed up in the outpatient clinic.


   Discussion Top


Sinus of valsalva aneurysms was first reported by J Hope in 1835 [1] . Most aneurysms are congenital in origin but maybe seen after bacterial endocarditis, atherosclerosis or chest trauma [2] . Congenital aneurysms of the aortic sinuses of valsalva are thought to result from weakness in the aortic media at its junction with the annulus fibrosus [3] . Aneurysms appear as small diverticuli or finger-like protrusions that extend most commonly from the right or non-coronary sinus.

The right ventricle and right atrium are common termination sites for aneurysms of the right coronary sinus. Aneurysms of the non coronary sinus usually enter the right atrium [4] . Rarely, aneurysms present because of compression to other cardiac structures producing symptoms [5].

Sinus of valsalva aneurysms are usually asymptomatic and are rarely discovered before they rupture and form aorto-cardiac fistula. They come to clinical attention most typically in adolescence and young adulthood when the protruding structure ruptures into the receiving chamber. Acute rupture of a large aneurysm causes retrosternal or epigastric pain and severe dyspnea from congestive heart failure. By contrast, perforation of a small aneurysm may go unnoticed until congestive heart failure develops from the long standing volume overload [3] . Coronary artery compression by a sinus of valsalva aneurysm is an unusual mode of presentation [6] . Mild aortic regurgitation is expected from distortion of aortic cusp and root enlargement as a result of long-standing volume overload. Severe aortic regurgitation should cause the suspicion of aneurysm rupture into the left ventricular outflow tract or secondary to endocarditis affecting the aortic valve leaflets [3] . Rarely, sinus of valsalva aneruysms burrow into the interventricular septum, causing AV conduction defects [3].

Our case is a rare case of unruptured, congenital sinus of valsalva aneurysm presenting with severe congestive heart failure. To our knowledge, burrowing of the sinus of valsalva aneurysm into the interventricular septum and its large tri-lobed appearance has never been described before. This rare extension of the right coronary sinus aneurysm caused deformity of the aortic right coronary cusp with consequent severe incompetence.

 
   References Top

1.Hope J. A Treatise on the Disease of the Heart and Great Vessels, 3rd edition, London: W Kidd, 1835:432-434.  Back to cited text no. 1      
2.Tomar M, Radhakrishnan S, Kausha S, et al. Unusual case of ruptured sinus of valsalva: rupture into left ventricular cavity along with distortion of mitral valve requiring double valve replacement. Images of Paediatric Cardiology, 009, 38:1-6.  Back to cited text no. 2      
3.Otto C, The Practice of Clinical Echocardiograph, 3rd Edition, 2007, page 1305.  Back to cited text no. 3      
4.Sakakibara S and Konno S: Congenital aneurysm of the sinus of valsalva: Anatomy and classification. Am Heart Journal, 1962, 63:405.  Back to cited text no. 4      
5.Segab C, Davy JM, Schebule C at el: Atrio-ventricular block disclosing an isolated congenital aneurysm of the sinus of Valsalva, extending into the septum and not ruptured. Arch Mal Coeur Vaiss, 74:1233, 1981.  Back to cited text no. 5      
6.Hiyamuta K, Ohtsuki T, Shimamatsu M, et al, Aneurysm of the left aortic sinus causing acute myocardial infarction. Circulation 67:1151, 1983.  Back to cited text no. 6      


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]



 

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