Prenatal physiologic findings and postnatal advanced imaging in the management of absent pulmonary valve syndrome with intact ventricular septum.

Absent pulmonary valve syndrome is a congenital heart defect usually associated with tetralogy of Fallot. Rarely, absent pulmonary valve syndrome can occur independently of tetralogy of Fallot and presents with an intact ventricular septum and a patent ductus arteriosus. This case report describes the prenatal diagnosis of absent pulmonary valve syndrome with intact ventricular septum via echocardiogram and the postnatal management of the resulting physiologic effects secondary to this rare congenital heart disease.