A ten-year review of ruptured sinus of valsalva: clinico-pathological and echo-Doppler features

Rupture of the sinus of valsalva (RSOV) is an uncommon condition with a variety of manifestations ranging from an asymptomatic murmur to cardiogenic shock. This retrospective 10-year review (1985-1995) of 18 patients from a single institution revealed that 6 (33%) were female and 12 (67%) were male with a mean age of 37.6 +/- 13.4 years and that 72% were Chinese by ethnic descent with the remaining 28% being Malay. Eight patients (44.4%) presented with an asymptomatic murmur, 4 (22.2%) with acute chest pain, 4 (22.2%) with mild heart failure, 2 (11.1%) with severe heart failure, and 2 (11.1%) with cardiogenic shock. Rupture of the right aneurysmal coronary cusp (RCC) made up 15 (83.3%) while those of the non-coronary cusp (NCC) made up the remaining. Most of the RCC ruptures were directed into the right ventricle and all of the NCC ruptures were into the right atrium. Ventricular septal defects (VSDs) were found in 9 (50%) of the patients, (although detected by echocardiography in only one third of those patients), aortic regurgitation in 6 (33.3%) and aortic valve vegetations in 2 (11.1%). Echocardiography was found to be accurate in diagnosing RSOVs with 100% diagnostic accuracy after 1990 with four misdiagnoses before 1990. Of these four patients, two were misdiagnosed as having VSDs, one as having a coronary arteriovenous fistula and one as having a patent ductus arteriosus. The anatomical structure of the "windsock" was seen in 64% of the patients who were correctly diagnosed. The pattern of colour flow and spectral Doppler was seen in all patients and helped to localise the site of rupture and the direction of flow. In summary, echocardiography is a simple and accurate way of diagnosing and defining RSOVs and is the imaging modality of choice.