A quadricuspid aortic valve is an exceedingly rare congenital heart anomaly with around 200 cases reported in the literature since the first case was reported in 1862.

Today’s patient had a quadricusp aortic valve associated with moderate aortic stenosis and severe aortic insufficiency, combined with severe mitral stenosis —likely the first documented combination, requiring urgent surgery for acute on chronic combined systolic and diastolic heart failure due to complex valvular heart disease.
The patient underwent a double valve replacement using the Edwards Inspiris Bovine Aortic and Edwards Mitris Bovine Mitral Bioprosthetic Valves. The patient was extubated post-operatively, hemodynamically stable, with normal sinus rhythm.