Sinus of Valsalva Aneurysms in Non-marfan Patients: An Underestimated Entity? A Case Series

Abstract

Aims: Sinus of Valsalva aneurysms are rare anomalies. Aneurysmal dilatation of the sinuses of Valsalva in Marfan syndrome has been extensively studied. The aim of this study was to investigate clinical manifestation, diagnosis, histopathological findings and management of sinus of Valsalva aneurysms in non-Marfan patients. Study Design: Retrospective analysis, case-series. Methodology: A search of digital echocardiographic recordings, histopathology and electronic patient databases in our institution for the period 2004 – 2012 was performed. All patients with asymmetrical dilatation of at least one coronary sinus of Valsalva at postmortem and ruptured (or dissected) aneurysms on echocardiogram were included. Patients with Marfan syndrome and those with non-ruptured sinus of Valsalva aneurysms on echocardiogram were excluded. Results: A total of 12 patients (7 males; mean age 36.1 years) were studied. The right coronary sinus was the most commonly affected (9/12). Involvement of more than 1 sinus was seen in 3/12 cases. Four patients died suddenly and another 6 had acute or rapidly worsening symptoms. Three sudden deaths were due to dissection or rupture with haemopericardium and tamponade. Five cases had concomitant congenital heart defects. Conclusion: Sinus of Valsalva aneurysms are an uncommon cause of morbidity and mortality in non-Marfan patients. They are associated with certain congenital heart defects. There is often associated aortopathy. Sudden death can be the first manifestation and is most commonly due to aneurysm rupture or aortic dissection into the pericardial space. Echocardiography is the investigation of choice for diagnosis and follow-up. Prompt surgical or percutaneous intervention has an excellent long-term outcome.