Absent pulmonary valve syndrome with tetralogy of Fallot and associated dextrocardia detected at an early gestational age of 26 weeks

Abstract

Absent pulmonary valve syndrome is a rare congenital anomaly, usually seen in association with a ventricular septal defect. It has been reported to occur in 3-6% of cases of tetralogy of Fallot. Absence of the pulmonary valve results in a dilated main pulmonary artery, which can be seen as a cystic, pulsatile, paracardiac lesion on antenatal USG. Such a lesion, though rare, can easily be detected. We report a case of this rare anomaly which was present in association with a ventricular septal defect, tetralogy of Fallot, and dextrocardia. The case was detected at 26 weeks of gestation.