ABSENT PULMONARY VALVE SYNDROME WITH VENTRICULAR SEPTAL-DEFECT - ROLE OF THE ARTERIAL DUCT

Absent pulmonary valve syndrome is usually associated with tetralogy of Fallot, but it also has been described in the presence of a ventricular septal defect or an intact ventricular septum.1-8 The pulmonary valve is represented by rudimentary nodules without identifiable leaflets and it is associated with aneurysmal dilation of the pulmonary trunk and one or both of its branches. It has been postulated that closure of the arterial duct in early intrauterine life plays an important etiologic role in the genesis of this syndrome.3,6 The anatomic and hemodynamic features of absent pulmonary valve syndrome are readily recognized prenatally using cross-sectional and Doppler echocardiography.9 We present the prenatal echocardiographic findings in 5 fetuses with absent pulmonary valve syndrome and a ventricular septal defect, 2 with a patent arterial duct, and speculate on the possible role of the arterial duct in this condition. © 1990.