Eur J Cardiothorac Surg 1999;15:100-102
© 1999 Elsevier Science NL
Cardiac tamponade and death from intrapericardial of sinus of Valsalva aneurysm
Marc-David Munka,
Michael A. Gatzoulisa,
David E.L. Kingb,
Gary D. Webba
a The Toronto Congenital Cardiac Centre for Adults, The Toronto Hospital, University of Toronto, Toronto, Canada
b Regional Forensic Pathology Unit, Hamilton Health Sciences Corporation, Hamilton, Ontario, Canada
Received 22 June 1998;
received in revised form 26 October 1998;
accepted 2 November 1998.
Corresponding author. The Toronto Hospital, 12EN, 200 Elizabeth St., Toronto, Ontario M5G 2C4, Canada. Tel.: +1-416-340-3709; 3872fax: +1-416-340-5014; e-mail: gatzoul@ibm.net
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Abstract
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A 35-year-old woman presented with dyspnea and chest pain. She had a large aneurysm of the non-coronary sinus of Valsalva. Before her scheduled urgent surgery, the patient collapsed and died of cardiac tamponade secondary to intrapericardial rupture of the aneurysm. We would advocate urgent repair of this type of lesion to prevent such an outcome. We are aware of no other specificpublished reports addressing extracardiac rupture of non-coronary cuspthis type of aneurysms.
Key Words: Non-coronary sinus • Sinus of Valsalva aneurysm • Extracardiac rupture
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Introduction
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Congenital sinus of Valsalva aneurysms (SVAs) are rare and their management is controversial. Rupture, if it occurs, is usually intracardiac, and generally leads to subsequent surgical repair. Occasionally, however, rupture may be extracardiac, leading to an acutely compromised patient.
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Case report
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We report a 35-year-old housewife presenting with exertional dyspnea and atypical chest pain, developing over a period of 10 months. Prior to this, she had been in good health with no cardiac complaints. Physical exam revealed a palpable A2, a 2/6 systolic heart murmur and a 2/6 decrescendo diastolic heart murmur. Transthoracic echo showed mild mitral valve prolapse, moderately severe aortic regurgitation and a large SVA. This was confirmed by trans-esophageal echo (SVA diameter 3.9 cm) which also demonstrated a distorted aortic valve with moderately severe aortic regurgitation (
Fig. 1
). The left ventricle was normal in size and function. Angiography showed a SVA extended inferiorly and laterally toward the right atrium, and moderately severe (3+) aortic insufficiency (
Fig. 2
). Semi-urgent surgical repair was scheduled to be within 2 weeks. Eight days later, prior to her surgical date, the patient collapsed in her doctor's office and could not be resuscitated.
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Fig. 1. Short axis trans-esophageal echocardiographic view of the aortic valve. Note the dilation of the non-coronary cusp.
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Fig. 2. LAO aortic angiogram showing markedly dilated non-coronary cusp aneurysm with moderately severe aortic regurgitation.
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Postmortem examination revealed 350 ml of fluid and fresh clotted blood in the pericardium, with no obvious trauma to the heart. A thin-walled 2.5 3 cm diameter SVA of the right non-coronary sinusadjacent to the right coronary ostium was found with a small rupture in its center adjacent to the pericardium. The heart was not enlarged or hypertrophied. The cause of death was cardiac tamponade secondary to intrapericardial rupture of the SVA.
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Discussion
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SVAs are rare (noted in 0.96–0.14% of surgical series at three centers)
[1]. Their true incidence is unknown as unruptured SVAs are often silent and may remain undiagnosed. SVAs most frequently develop in the right coronary cusp, less often in the non-coronary cusp and rarely in the left coronary cusp
[2]. A recent report incorporating three independent studies and 196 patients with diagnosed SVAs showed that only 2% of non-coronary SVAs had ruptured into the pericardium
[1].
Our case clearly represents a rare subset of a rare condition. Progressive exertional dyspnea, fatigue and chest pain are common in patients with ruptured SVAs, whereas most unruptured SVAs are asymptomatic
[3]. However, among patients with unruptured SVAs (non-coronary sinus, as in our report) a recent study reported two with exertional dyspnea, the third with atypical chest pain
[4]. The cause of the symptoms is not clear; they maycould be related to the displacement or compression of cardiac structures by the aneurysmal mass, to aneurysmal stretching, and/or to external compression of coronary arteries or indeed to aortic regurgitation, when severe
[5]
[6].
The management of unruptured SVAs has been somewhat contentious. Some authors have argued that many of these lesions should be monitored with a view toward correction if enlargement or hemodynamic compromise occur
[7]
[8]. Others argue that the risks of rupture makes correction of the unruptured SVA advisable; that asymptomatic aneurysms should generally be scheduled for elective surgery and that large or symptomatic unruptured SVAs be managed without delay
[4]
[9]. On the basis of our experience with this case, Wwe would support the latter viewconsensus.
Intrapericardial rupture of a sinus of Valsalva aneurysm is a rare complication which leads to cardiac tamponade and a highsignificant probability of death
[3]
[10]. To prevent such a catastrophic events, we advocate an urgent and aggressive approach toward treating patients with large unruptured aneurysms of the non-coronary sinus of Valsalva.
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References
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- Holman WL. Aneurysms of the sinuses of Valsalva. In: Sabiston DC Jr, Spencer FC, editors. Surgery of the Chest, 6th edn. Philadelphia: Saunders, 1995:1316–1326.
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- Brabham K.R., Roberts W.C. Fatal intrapericardial rupture of sinus of valsalva aneurysm. Am Heart J 1990;120(6):1455-1456.[Medline]
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- Howard R.J., Moller J., Castaneda A.R., Varco R.L., Nicoloff D.M. Surgical correction of sinus of valsalva aneurysm. J Thorac Cardiovasc Surg 1973;66:420-424.[Medline]
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- Mayer J.H., Holder T.M., Canent R.V. Isolated unruptured sinus of valsalva aneurysm: serendipitous detection and correction. J Thorac Cardiovasc Surg 1975;69:429-432.[Abstract]
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Abstract
Introduction
