Cor triatriatum associated with degenerative aortic insufficiency in an adult patient.

Cor triatriatum is a rare cardiac anomaly which can be congenital or acquired in origin. Congenital cor triatriatum is due to an alteration of the common pulmonary vein resorption and therefore the left atrium is divided into two chambers, a proximal one, in communication with the pulmonary veins, and a distal one, in communication with the mitral valve orifice. The diagnosis is usually made at birth, but in rare cases, when the communication between the two chambers is wide and the patient is asymptomatic, the lesions may be diagnosed incidentally during a routine echocardiographic examination. We report a 32-year-old man, admitted to our hospital with a diagnosis of aortic insufficiency, in whom echocardiography revealed the presence of cor triatriatum. The patient underwent aortic valve replacement and resection of the atrial membrane. Histology of the aortic valve revealed myxoid degeneration of the spongiosa.