Congenitally unguarded morphologically tricuspid valvar orifice is a rare abnormality, usually associated with pulmonary atresia. We describe such a case found in association with discordant atrioventricular and ventriculoarterial connections in which the aortic valve was atretic. Presentation was with respiratory distress due to severe cardiac failure after birth. Echocardiography demonstrated a dilated systemic ventricle with poor function, left atrial enlargement, and aortic atresia. The clinical findings were confirmed at autopsy.
