Double Superior Vena Cava and absent right pleura in Tetralogy of Fallot: An unusual association

Tetrology of fallot is the most common cause of cyanotic congenital heart disease with an incidence of 6-10 % among all congenital heart diseases. The anatomic defect comprising this entity leads to poor oxygenation resulting in symptoms of cyanosis, polycythemia and hypoxia. Most untreated patients die during childhood. Surgical treatment is the treatment of choice even during infancy. Many cardiac and extracardiac anomalies are associated with this entity. Here we report a 10 year old female patient of Tetrology of Fallot associated with double superior vena cava and absent right pleura. Total correction was made and the postoperative course was uneventful.