Fallot type of absent pulmonary valve syndrome - A case report

Abstract

Absent pulmonary valve syndrome (APVS) is a rare congenital cardiac malformation characterized by absent, dysplastic, or rudimentary pulmonary valve leaflets in association with other cardiac anomalies. It has an incidence of 3-6% in cases of tetralogy of Fallot (TOF) and 0.2-0.4% of live-born infants with congenital heart disease (CHD). Absent pulmonary valve leads to dilated main pulmonary artery; presenting as a pulsatile, paracardiac cystic lesion on antenatal ultrasound (USG). We report a case of this rare anomaly in association with ventricular septal defect (VSD), TOF, and left axis deviation of heart detected at 23 weeks of gestation.

Surgical Treatment of Tetralogy of Fallot with Pulmonary Valve Agenesis in a 22 Years Old Patient

“…Pulmonary valve agenesis with ventricular septal defect is a rare variant of Tetralogy of Fallot. It occurs in 3% to 6% of Tetralogies of Fallot (ToF) [1]. Absent pulmonary valve syndrome is an uncommon variant of Tetralogy of Fallot, which manifests itself morphologically as vestigial pulmonary valve cusps at the right ventricle-pulmonary trunk junction, significant pulmonary regurgitation and aneurysmal dilatation of the proximal and mediastinal arteries, the aneurysmal dilated pulmonary arteries may compress the tracheobronchial tree and cause severe respiratory distress in the neonatal or infant period.…”