Double orifice tricuspid valve with left atrioventricular valve atresia and univentricular connexion to a dominant left ventricle

We present a case of univentricular connexion to a dominant left ventricle with double orifice tricuspid valve, left atrioventricular valvar atresia, discordant ventriculo-arterial connexion and aortic arch hypoplasia. Diagnosis was made by cross-sectional echocardiography and angiocardiography. This case is exceedingly rare.