Quadricuspid aortic valve: a rare cause of aortic insufficiency diagnosed by doppler echocardiography. Report of two cases and review of the literature.

Abstract

Quadricuspid aortic valve is an uncommon congenital valve disease mostly occurring as isolated lesion or sometimes in association with truncal anomalies. Approximately 50% of patients with quadricuspid aortic valve have aortic regurgitation. Before the advent of echocardiography most cases were diagnosed at the time of surgery or at post-mortem examination. We describe 2 cases of patients with quadricuspid aortic valve diagnosed by echocardiography. The first case, a quadricuspid aortic valve with four equal-sized cusps (type A, according to the classification of Hurwitz and Roberts), was identified in a 26-year-old man undergoing echocardiography because of a heart murmur. The second case, a quadricuspid aortic valve with three relatively equal cusps and one smaller cusp (type B, according to the classification of Hurwitz and Roberts), was identified in a 47-year-old man with a history of murmur. The identification and periodical non-invasive evaluation of a quadricuspid aortic valve is important, because such valves are more vulnerable to infection and need adequate prophylaxis against endocarditis.