Sex reversal and hypoplastic left heart syndrome

The prognosis of congenital heart disease, especially in its most complex forms, is complicated when associated with extracardiac anomalies. Meacham syndrome, or PAGOD syndrome (pulmonary tract and pulmonary artery hypoplasia, agonadism, omphalocele, diaphragmatic defect, and dextrocardia) is a constellation of genital, cardiac, and pulmonary malformations that carries a poor prognosis. We report the case of a patient with sex reversal and hypoplastic left heart syndrome without congenital diaphragmatic hernia who has survived to Fontan completion and is doing well.