Tetralogy of Fallot with absent pulmonary valve syndrome is a rare form of congenital heart disease. Among the different variations with this rare anomaly is nonconfluent pulmonary artery branches with anomalous origin of the left pulmonary artery from the ductus arteriosus. The authors present one such case which was diagnosed prenatally to have tetralogy of Fallot with absent pulmonary valve and identified postnatally to have nonconfluent pulmonary artery branches in addition. We discuss the conundrum of respiratory management in this patient pre- and postoperatively due to a unique ventilation perfusion mismatch problem, which varies between the two lungs.
|Original language||English (US)|
|Journal||World Journal for Pediatric and Congenital Hearth Surgery|
|State||Published - Aug 27 2018|
- absent pulmonary valve syndrome; nonconfluent pulmonary arteries; patent ductus arteriosus; tetralogy of Fallot