Severe Aortic Regurgitation Due to Quadricuspid Aortic Valve

Quadricuspid aortic valve is a rare congenital malformation with prevalence of <0.05%, often manifesting in mid- to late adulthood with aortic regurgitation (AR). We present a case of a 60-year-old man with chronic severe AR due to phenotype F quadricuspid aortic valve and mild to moderate functional mitral regurgitation. Cardiac catheterization revealed multivessel coronary artery disease. The patient underwent successful surgical aortic valve replacement with a bioprosthesis and 2-vessel coronary artery bypass graft surgery. Echocardiography at 1-month follow-up showed improved left ventricular volumes, normal aortic bioprosthesis, and trivial mitral regurgitation. This case highlights the role of echocardiography in diagnosing quadricuspid aortic valve and guiding management.

Quadricuspid aortic valve is a rare cause of AR that can be readily diagnosed on echocardiography. Surgical aortic valve replacement is an effective treatment for severe symptomatic AR due to quadricuspid aortic valve.