Journal of Pediatric Cardiology and Cardiac Surgery

A female neonate had left isomerism with absent pulmonary valve syndrome and unbalanced atrioventricular septal defect. She was intubated immediately after birth because of respiratory distress. On day 27, she underwent division of the pulmonary trunk and plication of the dilated pulmonary artery concomitantly with construction of a modified Blalock–Taussig shunt as the first palliation. She passed away due to an unknown cause at 8 months of age while waiting for the second stage surgery. We report this extremely rare combination of congenital heart malformations.