This is a congenital heart defect in which there is under development of left side of the heart (left ventricle, aortic valve, and aorta). This is one of the most serious congenital heart defect which needs attention almost immediately after birth. In this defect no blood goes to the left side of the heart and therefore whole systemic circulation suffers. Life persists only on some connection between right side and left side either through a patent ductus or atrial septal defect. Spontaneous closure of this connection specially the ductus leads to severe circulatory embarrassment and death of the child.

Poor feeding, shortness of breath, cold extremities, poor peripheral pulses, enlarged liver and low urine output. These symptoms manifests very quickly after birth.

Echocardiogram is the definitive diagnosis. Antenatal diagnosis by fetal echocardiography helps in quick management of such cases.

Treatment begins immediately after birth,. First the baby is put into ventilator to assist breathing and ductus is kept open by prostaglandin. This is followed by a complicated surgical repair in stages (called Norwood procedure).

In stage one, the functioning right ventricle is connected to the aorta after its enlargement to deliver blood to the whole body (systemic circulation). To maintain blood supply to the lungs a systemic to pulmonary artery shunt is created (called BT shunt).

In stage two, the superior vena cava is attached to the pulmonary artery (called Glenn Shunt) and BT shunt is removed.

In stage three,  the blood from inferior vena cava is diverted to the pulmonary artery (called completion Fontam) by a tunnel through the right atrium or by using a special synthetic conduit. After the third procedure the child is no longer blue.

If left untreated this condition is always fatal. Survival depends on early diagnosis and undergoing correct surgical procedure.