Topsy-turvy heart: A first recorded case report with intracardiac anomaly

Topsy-turvy heart is a rare congenital anomaly; it has a specific characteristic of cardiac malrotation and superior-inferior arrangement of right/left ventricles. A five-day-old patient was diagnosed antenatally with hypoplastic left heart and right hydronephrosis and had been admitted to the hospital with symptoms of respiratory distress. The postnatal imaging studies revealed an abnormal ventricular arrangement with a large aortopulmonary window, ventricular septal defect (VSD), and the upside-down orientation resulting in the posterior-inferior displacement of the common arterial confluence and, in turn, the left main bronchial stenosis. For the first time, the diagnosis of a topsy-turvy heart with the intracardiac anomaly (VSD) was confirmed.