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 congenital (present at birth) heart disease, which often requires lifetime monitoring and care. Our multi-specialty team of pediatric and adult cardiologists, cardiovascular surgeons, cardiovascular anesthesiologists, and maternal-fetal medicine specialists have unique expertise in treating patients before birth and into adulthood.

UAB Medicine’s 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 expecting mothers or fathers known to have congenital heart disease. If a defect is discovered, our experts provide prenatal treatment and develop a plan for delivery and treatment after birth.

Thanks to advances in pediatric congenital care, the life expectancy for most patients now reaches far into adulthood. However, more than half of the people with congenital heart problems 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 few in the country, our expertise greatly increases the chances that symptoms will be identified early. This helps ensure that less serious problems are addressed before they develop into larger, more life-threatening issues such as heart failure, arrhythmia, residual congenital heart defects, endocarditis, and stroke.

Videos

Resources

• Pregnancy and Adult Congenital Heart Disease
• UAB Cardiovascular Surgery Patient Guide 2022
• UAB Division of Cardiovascular Disease
• UAB Division of Cardiothoracic Surgery
• UAB Division of Pediatric Cardiology
• Pediatric Cardiovascular Services
• American Heart Association

Related Specialties

• Atrial Septal Defect
  Bicuspid Aortic Valve
  Congenital Heart Disease
  Ebstein’s Anomaly
  Fetal Cardiac Services
  Hypoplastic Left Heart Syndrome (HLHS)
  Marfan Syndrome
  Transposition of the Great Arteries (TGA)
  Turner Syndrome
  Ventricular Septal Defect