Internationale Zeitschrift für Herz-Kreislauf-Forschung

Congenital Absence of Pulmonary Valve: A Rare Congenital Heart Defect in Newborns

Luvdeep Dogra, Leslie E Lewis, Ramesh Bhat Y, Jayashree P, Najih M, Ranjan S and Krishnananda N

Absent pulmonary valve (APV), defined as total or subtotal absence of pulmonary valve leaflets, is a rare defect. The estimated frequency is 6 in 3000 live born infants with congenital heart diseases. Pulmonary stenosis and aneurysmal dilatation of pulmonary artery usually coexist with the defect. At birth, the affected new bornmay have severe manifestations leading to diagnosing difficulties. Presented is a newborn with persistent cyanosis at birth requiring resuscitation in which subsequent echocardiography established the diagnosis of Fallot-type APV. This case highlights the need for fetal echocardiography in high risk settings.