We report a case of hypoplastic left heart syndrome with an anomalous left coronary artery from the pulmonary artery (ALCAPA) identified intraoperatively during the Stage-II palliation. Due to recurring ventricular fibrillation on sternotomy, a hybrid Stage-I palliation was performed. During comprehensive Stage-II, the ALCAPA was reimplanted in the neoaorta and measures, including a nontraditional Damus connection/arch reconstruction and classic bilateral Glenn procedures, were taken to avoid compression of the coronary artery. After a successful Fontan procedure, he continues to do well at 5 years old, becoming the first patient reported in the literature to survive all the three stages of single-ventricle palliation.
Keywords: Anomalous left coronary artery from the pulmonary artery; Fontan; bidirectional Glenn; hypoplastic left heart syndrome.