Clinical, haemodynamic, angiocardiographic, and necropsy findings are reported in a newborn infant who presented with typical findings of the severe form of the syndrome of absent pulmonary valve. In addition to the usual findings of aneurysmal dilatation of the pulmonary arteries, pulmonary insufficiency, and stenosis, this patient had an intact ventricular septum and large ductus arteriosus. The former is a rare finding and the latter is not known to have been reported in a patient with the severe form of this syndrome.
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