In this issue of Vascular Disease Management, Zavala-Alarcon et al¹ present an interesting look at an uncommon cardiovascular problem and the unique opportunity to “witness” a potentially highly morbid event if not diagnosed and treated properly. Aneurysms of the aortic root involving the sinuses of Valsalva (SVA) are a distinct entity from atherosclerotic ascending aortic aneurysms or aneurysmal dilatation associated with a bicuspid aortic valve. The authors have done a nice job describing the pathogenesis and clinical signs and symptoms of SVA. I will reserve my comments on what I believe to be the important “take home messages” from this interesting case report.

Rupture of a SVA in the United States is indeed rare, primarily due to earlier diagnosis by echocardiography in the pediatric patient population. The average age of rupture is 30 years, and in my 25 years of experience, I have treated 4 cases (2 ruptures). SVA comprise 0.1–3.5% of U.S. congenital cardiac anomalies and are most often managed by pediatric cardiologists. SVA prevalence is significantly higher in Asians, therefore SVA should be high on the differential diagnosis for any young Asian male (male/female ratio is 4:1) presenting with cardiovascular symptoms or with an asymptomatic murmur. As mentioned by the authors, SVA is almost always associated with other anomalies, including ventricular septal defect and aortic insufficiency as exhibited in this case.

Treatment has historically been surgical with < 2% mortality and remains the mainstay treatment, primarily due to the frequent need for ventricular septal defect and aortic insufficiency surgical correction as demonstrated in this case. The timing between rupture and surgical repair will depend on the symptoms, size and location of the rupture. Interestingly, SVA rarely rupture into the pericardium, resulting in tamponade, but invariably rupture into the right atrium or right ventricle with 80% becoming severely symptomatic.² In general, surgical correction should be recommended after a short period of medical stabilization, as most of these patients are young and healthy otherwise. Survival is rare < 1 year after SVA rupture. A full life expectancy is anticipated after successful surgical repair. Endocarditis needs to be ruled out as an etiology, especially in non-Asian patients with atypical presentations.

Percutaneous closure of a ruptured SVA was first attempted in 1994 and successfully accomplished in 1995.^3,4 In general, associated anomalies will preclude percutaneous closure but ideal selected anatomic cases have been successfully treated with the AMPLATZER^® Atrial Septal Occluder (AGA Medical Corporation, Golden Valley, Minnesota).⁵ Any consideration for percutaneous closure should be reserved for very selected high-risk surgical cases and be attempted only at very experienced centers.

In conclusion, SVA should be on the differential diagnosis with any young patient, especially Asian, who presents with new onset of any cardiovascular symptoms, including shortness of breath, chest pain, or congestive heart failure.