Tetralogy of Fallot (fuh-LOE) is an uncommon condition consisting of four (“tetra”) defects within the heart structures of infants and young children. Together these defects cause reduced blood flow to the lungs and mixing of blood from both sides of the heart. This results in oxygen-poor blood flowing out of the heart and into the body. Consequently, infants and children with tetralogy of Fallot usually have blue-tinged skin (cyanosis).Tetralogy of Fallot, which is present at birth, is usually diagnosed during infancy. However, it may not be detected until later in life, depending on the severity of the heart defects and symptoms. Tetralogy of Fallot occurs in about five out of every 10,000 babies and affects males and females equally. With early diagnosis followed by corrective surgery and ongoing care, the majority of children with tetralogy of Fallot survive to adulthood.
Most babies with tetralogy of Fallot need a type of surgery called intracardiac repair, which is typically performed during the first year of life. During this procedure, the surgeon places a patch over the ventricular septal defect to close the hole between the ventricles. He or she also repairs the narrowed pulmonary valve and widens the pulmonary arteries to increase blood flow to the lungs. After intracardiac repair, the oxygen level in the blood increases and your baby’s symptoms will lessen.
Occasionally babies need to undergo a temporary surgery before having intracardiac repair. If your baby was born prematurely or has pulmonary arteries that are underdeveloped (hypoplastic), doctors will create a bypass (shunt) between the aorta and pulmonary artery. This bypass increases blood flow to the lungs. When your baby is ready for a full repair, the shunt will be removed.