Hypoplastic left heart syndrome

Hypoplastic left heart syndrome is a serious congenital heart defect in which the left ventricle is severely underdeveloped. Treatment starts immediately upon diagnosis with administration of the medicine prostaglandin to prevent the patent ductus arteriosus from closing. A ventilator may be necessary to assist with breathing, as well as intravenous fluids and additional medications to strengthen the heartbeat. These measures are undertaken to stabilize the baby's condition while decisions regarding treatment can be made. Management of hypoplastic left heart syndrome largely depends on the family's wishes and the severity of the condition.

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For some babies, a three-stage surgical procedure is an option. The operation reconstructs the child's heart so that the single pumping chamber can meet the patient's lifetime needs. Completion of these operations allows separation of the blood without oxygen from the oxygenated blood.

Stage 1: Norwood Procedure

Performed as soon as possible after the diagnosis, the Norwood procedure enables the heart to pump blood to both the lungs and the body. This procedure involves reconstructing the aorta, the main blood vessel supplying blood to the body, and inserting a tube that connects the aorta to the pulmonary artery, the blood vessel that supplies blood to the lungs. The baby will still look blue (cyanotic) after this first-stage operation. Two heart catheterizations are typically performed in the next two years, usually just before each of the next two surgical stages.

Stage 2 Bidirectional Glenn Procedure or hemi-Fontan

The bidirectional Glenn procedure reduces the work of the heart by routing blood from the upper body directly to the lungs. This operation is usually performed at 6 months of age and increases the oxygenation of the blood.

Stage 3 Fontan ProcedureThe third and final operation is usually performed between 18 to 48 months. This procedure allows the blood coming back from the lower body to go directly to the lungs, eliminating mixing of oxygenated and nonoxygenated blood in the child's heart. After this operation, the patient's general health and appearance will improve and the child will no longer look blue.

The function of the reconstructed heart and blood vessels needs long-term monitoring and followup care, which may include ongoing treatment with cardiac medications. Regular monitoring should include evaluating the functions of the child's heart with echocardiography as the child grows and develops.

Alternatively, an infant with this defect can be put on a list to receive a heart transplant. The infant is then kept on prostaglandin until a donor heart becomes available.