The Four Seasons of Ruptured Sinus of Valsalva Aneurysms: Case Presentations and Review

Abstract

The sinuses of Valsalva are 3 distinct outpouchings of the aortic wall associated with the 3 cusps of the aortic valve that may develop aneurysmal dilation because of weakness of or injury to the sinus wall. Rupture of a sinus aneurysm can create an aortocardiac fistula. Ruptured sinuses of Valsalva aneurysms (RSVAs) may present a diagnostic dilemma because of their varied clinical presentations. However, if included on a differential, they are easily diagnosed and surgically treated. In our article we detail 4 RSVA cases, each demonstrating a manner in which an RSVA may present clinically. Our first case involves a 68-year-old patient with an RSVA diagnosis after presenting with cardiac arrest and congestive heart failure. Our second case involves a 42-year-old patient with an RSVA diagnosis in the context of acute chest pain, ischemic electrocardiographic changes, and hypotension. Our third RSVA case involves a 60-year-old patient who presented solely with a sudden onset of lower-extremity edema. Our fourth case involves a 46-year-old asymptomatic patient with RSVA diagnosed during a routine physical exam. Comparisons of reported case series from around the world illustrate RSVA epidemiology, concomitant lesions, clinical presentations, and repair techniques. Comparisons of Eastern and Western series reveal that the incidence of RSVAs is higher in Eastern than in Western countries, with a 4:1 male preponderance across ethnic lines. Among the Eastern series reporting RSVAs, ventricular septal defects and aortic valve incompetence were the only frequently associated concomitant lesions. In contrast, Western series of RSVAs showed a wide range of concomitant lesions. The difficulty in diagnosing RSVAs is mainly due to the variability of their clinical impact and presentation. These factors largely depend on the cardiac chamber into which the aortocardiac fistula forms. However, once RSVA is on a differential, the advent of transesophageal and transthoracic radiography has made RSVA diagnosis relatively easy. Surgical repairs of RSVAs are of low risk and generally have an excellent long-term prognosis. As a result, many authors believe that early surgical intervention in patients with an RSVA is justified. Among the series studied, there is evidence that the patch technique is the safest approach because of its lower association with fistula recurrence. This article highlights for the clinician the diversity of clinical presentations of this often overlooked disorder.