Tricuspid atresia with absent pulmonary valve and intact ventricular septum: a rare association.

The absence of the pulmonary valve in connection with tricuspid atresia and intact ventricular septum is a rare malformation, associated with a poor prognosis. The right ventricle is severely hypertrophied, resembling a cardiac mass protruding into the left ventricular outflow tract. We report such a case that underwent successful palliation with a Blalock-Taussig shunt followed by a superior cavopulmonary anastomosis.