Supravalvular mitral stenosis in a patient with tetralogy of Fallot

Abstract Supravalvular mitral stenosis was found in association with tetralogy of Fallot in an 8 year old boy who died of an intercurrent respiratory infection. This association was not diagnosed during life. However, the electrocardiographic features of right ventricular strain combined with left atrial enlargement should have suggested mitral obstruction. The relatively high pulmonary artery pressure in a patient with a tetralogy and the angiographic findings of large branch pulmonary arteries and a left atrium with prolonged emptying should also lead one to suspect this combination. Surgical correction in such cases must include an attack on the mitral valve.