Double-Orifice Mitral Valve Associated with Bicuspid Aortic Valve and Primary Pulmonary Vein Stenosis.

• DOMV is a rare condition. • DOMV is often associated with other cardiac congenital anomalies. • DOMV is not always hemodynamically significant and is often discovered incidentally. • Primary PV stenosis is very rare and is dynamic in nature. • The authors report the first case of DOMV associated with BAV and PV stenosis.