Tetralogy of Fallot (TOF) with Pulmonary Atresia

Tetralogy of Fallot (TOF) with pulmonary atresia is a more severe form of TOF, a type of heart defect. It’s a congenital condition, which means it’s something a baby is born with.

Babies who have TOF with pulmonary atresia have five heart abnormalities:

• Ventricular septal defect (VSD)
• Overriding aorta: This means the aorta is moved toward the right side of heart, just over the VSD.
• Thickened right ventricle. The right ventricle becomes thicker than normal from pumping blood against resistance.
• Complete pulmonary obstruction. In TOF, the pulmonary valve is narrowed, but in children with TOF with pulmonary atresia, there is no pulmonary valve at all to connect the right ventricle to the lungs. This means blood must find its way to the lungs through other, smaller arteries.
• Abnormal pulmonary arteries. These arteries may develop abnormally to help move blood to the lungs, but this can vary widely from child to child. In some children, they’re essentially normal, except for the fact that they are connected to the aorta rather than to the right ventricle. These abnormal pulmonary arteries are referred to as major aortopulmonary collateral arteries (MAPCAs).

TOF with pulmonary atresia is usually diagnosed shortly after birth. The most common symptom is a bluish tint to the skin, lips, and nail beds, called cyanosis.

Babies born with TOF with pulmonary atresia need surgery to correct the problem, usually before 6 months of age. The surgery brings all the pulmonary arteries together (unifocalization), so a connection can be created between the arteries and the right ventricle. Your child may need more than one operation, sometimes over a period of months or years.

How we care for tetralogy of Fallot with pulmonary atresia

Our team at the Boston Children’s Hospital Benderson Family Heart Center treats some of the most complex pediatric heart conditions in the world, including TOF with pulmonary atresia. Our overall success rates are the highest in the world among large pediatric cardiac centers.

Our areas of innovation for tetralogy of Fallot with pulmonary atresia

• complete or staged unifocalization of pulmonary artery branches
• coordinated, multidisciplinary approach