Functional pulmonary atresia in neonatal Marfan's syndrome: Successful treatment with inhaled nitric oxide

Functional pulmonary atresia is characterized by a structurally normal pulm onary valve not opening during right ventricular ejection. We report this r are condition in a premature newborn of a twin pregnancy, in which fetal ec hocardiography findings were consistent with critical pulmonary stenosis. A fter birth, features of neonatal Marfan's syndrome were noted. Echocardiogr aphy showed a morphologically normal but immobile pulmonary valve with cont inuous regurgitation. Right ventricular pressure was subsystemic. In this c ase, initial treatment with nitric oxide, followed by pharmacological duct closure, was successful. Differentiating between anatomic and functional pu lmonary valve atresia may be difficult. The echocardiographic criteria are discussed.