Tetralogy of Fallot

Tetralogy of Fallot is a rare, complex congenital heart defect, meaning that it is present at birth. It causes oxygen-poor blood and oxygen-rich blood to mix together, with both being pumped out of the heart and into the blood vessels. As a result, blood leaving the heart has less oxygen than is needed by the organs and tissues of the body, causing a condition called hypoxemia. Ongoing and long-term lack of oxygen causes cyanosis, a condition marked by a bluish color of the skin, lips, and membranes inside the nose and mouth.

Tetralogy of Fallot involves four heart defects: a ventricular septal defect, or a hole in the wall between the heart's two lower chambers; pulmonary stenosis, or the narrowing of the pulmonary valve (which carries blood from the heart to the lungs), causing it to not open fully; right ventricular hypertrophy, in which the muscle of the right ventricle is thicker than usual; and a defect in the aorta, the main artery that carries oxygen-rich blood from the heart to the body. Doctors repair this defect with open-heart surgery, either soon after birth or later in infancy.

The UAB Congenital Heart Disease Program offers the most advanced care for structural heart disease, which often requires lifetime monitoring and care. Our multidisciplinary team of pediatric and adult cardiologists, cardiovascular surgeons, cardiovascular anesthesiologists, and maternal/fetal medicine specialists are uniquely qualified to treat patients before birth and into adulthood. In addition to seeing patients at the UAB Women & Infants Center and The Kirklin Clinic of UAB Hospital, UAB Medicine’s congenital heart disease specialists also see patients at North Alabama Children's Specialists in Huntsville and Physicians to Children/Central Alabama Children's Specialists in Montgomery.

Our modern ultrasound equipment allows many heart defects to be diagnosed before a child is born. Screening exams performed at 18-20 weeks are recommended for expectant mothers (or fathers) known to have congenital heart disease. If a defect is discovered, prenatal treatment and planning for delivery and postnatal treatment can be undertaken.

Thanks to advances in pediatric congenital care, the life expectancy for most patients now reaches far into adulthood, but more than 60% stop seeing a cardiologist once they turn 18. UAB’s Alabama Adult Congenital Heart Disease Program is designed to prevent that gap in care. As the only adult congenital heart disease program in the state and one of only a handful in the country, our expertise greatly increases the chances that symptoms will be identified early. This helps ensure that smaller problems are addressed before they develop into larger, more life-threatening issues such as heart failure, arrhythmia, residual congenital heart defects, endocarditis, and stroke.