Quadricuspid Aortic Valve: A Comprehensive Review

Abstract

Quadricuspid aortic valve (QAV) is a rare congenital heart disease. The functional status of QAV is predominantly a pure aortic regurgitation. Clinical manifestations of patients with a QAV depend on the functional status of the QAV and the associated disorders. Significant valvular regurgitation and (or) stenosis is often present with subsequent operation performed at the fifth to sixth decade of life. The functional status of QAV is predominantly regurgitant; whereas pure stenotic QAV can be as few as in only 0.7% of the patients. QAV is usually an isolated anomaly, but other congenital heart defects can be present in 18-32% of the patients. About one-fifth of them require a surgical operation. Tricuspidalization is a preferred technique for QAV repair. As not all the patients with a QAV necessarily warrant a surgical operation, decision-making in patient selection and surgical procedure of choice are crucial. Antibiotic prophylaxis against infective endocarditis is necessary in the QAV patients with unequal-sized cusps.

A four‐leaf clover: A case report of quadricuspid aortic valve stenosis

“…Hurwitz and Roberts described seven anatomical variants of QAV based on the size of the valve leaflets. Type A, in which the four cusps are of equal size is one of the most common variants 1,2 . Our patient had type A quadricuspid valve, with the classical four‐leaf clover appearance.…”

“…Quadricuspid aortic valve (QAV) is a rare congenital malformation 1 . The diagnosis is often made incidentally, but some patients present with hemodynamic complications, most commonly aortic regurgitation 2 . During the diagnostic work‐up for degenerative severe aortic stenosis, QAV is rare.…”

Quadricuspid aortic valve: Case series and review of literature

“…The most common valve pathology in patients with QAV is aortic insufficiency noted in 75% of cases, with normal aortic valve function in 16%, and stenosis being less common . The progression of aortic insufficiency with QAV is thought to be due to the size and placement of unequal cusps.…”

“…The progression of aortic insufficiency with QAV is thought to be due to the size and placement of unequal cusps. This causes an uneven distribution of hemodynamic stress and incomplete or malcoaptation during diastole, which may lead to progressive aortic insufficiency . For example, those with QAV with four equal leaflets are less likely to develop fibrous thickening and significant aortic regurgitation compared to a patient with an unequal size leaflet who would be more likely to develop abnormal coaptation and furthermore regurgitation.…”

“…A quadricuspid aortic valve (QAV) is a rare valve lesion, with a reported incidence of 0.05%‐0.1%, in patients undergoing aortic valve replacement for aortic insufficiency . The mean age at presentation is usually between 46 and 50 years, with a slight predominance for males .…”