In single ventricle there is one ventricle receiving blood from both atria. A small outlet chamber is present in single ventricle, however it does not communicate directly with the atria, instead it receives blood from the single ventricle through a bulboventricular foramen. The aorta and pulmonary arteries in single ventricle are either normally arranged or transposed. Clinical presentation and management in single ventricle is controlled by extent of pulmonary blood flow: Severe pulmonary stenosis (PS): the pulmonary blood flow (PBF) will be restricted resulting in cyanosis. These patients will require systemic to pulmonary arterial shunt to improve PBF. No PS: PBF will be unrestricted resulting in congestive heart failure (CHF) and only mild cyanosis. These patients will require banding of pulmonary artery to restrict PBF. Moderate PS: PBF will be somewhat restricted and the child will present later with mild cyanosis and CHF. These patients can be observed till Glenn shunt is placed at 4–5 months of age.