Absent Pulmonary Valve with Intact Interventricular Septum

Absence of the pulmonary valve is a rare and severe cardiac malformation, particularly in fetus and newborn, so prenatal diagnosis is essential in the survival and outcomes of these patients. It is characterized by the total or partial absence of the pulmonary ostium and in all cases is associated with pulmonary artery orifice stenosis and aneurysmal dilation of pulmonary arteries, leading to bronchial obstruction. Pulmonary agenesis may occur isolated, sporadically or associated with other anomalies in a genetic context. We present the case of a gravida I para I with 37 weeks of gestation, diagnosed with foetal pulmonary agenesis that presented to our clinic for a second opinion about management of delivery and further neonatal care. A complex team that included of obstetrician, cardiac pediatrician, neonatologist, and pediatric surgeon was formed. The delivery occured at 38 weeks through cesarean section. Few hour later the newborn had surgical repair of the defect with a favorable evolution.