Long-term survival in an un-operated single ventricle

Single ventricle is a rare and complex congenital heart disease. Neonates with single ventricle have a high mortality. Survival into adulthood is rare without surgical intervention. A case of single ventricle with double inlet and double outlet combined with severe valvular pulmonary stenosis and mitral regurgitation (Grade II) is being reported here. A 36 years old man was admitted for bluish discoloration of lips, tongue and fingers; shortness of breath and recurrent palpitation. His ECG showed atrial fibrillation with fast ventricular rate. Chest X-Ray depicted an enlarged cardiac shadow and right sided pleural effusion. Final diagnosis was made by echocardiogram which demonstrated single ventricle with double inlet and double outlet, severe valvular pulmonary stenosis and mitral regurgitation (Grade II) with good ventricular systolic function.