Tricuspid Atresia

Treatment

Rashkind atrial septostomy

Occasionally, the hole between the atria (patent foramen ovale [PFO]) is too small or may become too small over time to allow sufficient blood to cross it, so it requires enlargement. It can be made larger through a procedure called Rashkind balloon atrial septostomy.

In this procedure, an interventional cardiologist inserts a small, thin tube (catheter) with a balloon on its tip into a vein in the leg and guides it into the heart’s right atrium and through the PFO into the left atrium. In the left atrium, the balloon is inflated. The catheter is then pulled back sharply into the right atrium, causing a tear in the atrial wall. This tear enlarges the opening between the two chambers to allow more blood to flow through.

To keep the ductus arteriosus open so that blood can continue to flow to the lungs from the aorta, a medication called prostaglandin E1 (PGE1) is given through an IV line. The medication can’t be used for prolonged periods, so a procedure is performed (e.g., a PDA stent) soon after birth to replace the ductus arteriosus with a manmade blood vessel.

Surgical repair

The goal of surgery in treating tricuspid atresia is to separate the circulation of oxygen-poor (blue) blood and oxygen-rich (red) blood. The surgery is not aimed at duplicating the anatomy of a normal heart. A new tricuspid valve can’t simply be placed between the right atrium and the right ventricle because the right ventricle is often extremely small. Surgery also aims to improve oxygen delivery to the body and relieve the extra burden placed on the heart as it works harder to supply blood and oxygen to the body.

A single ventricle pathway is typically chosen for children with tricuspid atresia. Separation of the blue and red circulation is achieved through three surgical procedures:

• Aortopulmonary shunt, modified Blalock-Taussig (B-T) shunt, or PDA stent – Soon after birth, a vessel is inserted between the aorta and the pulmonary artery to enable adequate blood flow to the lungs to mimic the function of the ductus arteriosus, which naturally will shrink with time.
• Bidirectional Glenn or hemi-Fontan procedure – In this procedure, which is usually performed at 3 to 6 months of age, the main vein that returns blood from the head, neck, and arms (superior vena cava) is connected directly into the blood vessels going to the lungs (pulmonary arteries). The previously placed B-T shunt or PDA stent is removed at this time, as it’s no longer needed since oxygen-depleted venous blood coming back from the upper body provides the blood supply to the lungs. Blood coming back from the veins of the lower body continues to pass directly into the heart and eventually mixes with the blood from the lungs before going out to the body. Thus, the bluish discoloration of the child’s skin (cyanosis) persists.
• Fontan procedure – When the child reaches about 18 months of age to 3 years, this third surgery is performed. In this procedure, the vein carrying oxygen-depleted venous blood from the lower body (inferior vena cava) is connected directly to the pulmonary arteries, either using a tube that bypasses the heart or by creating a baffle within the heart to direct the blood upward to the lungs. The pulmonary arteries then send the blood to the lungs to pick up oxygen. After completion of the Fontan procedure, the blue and red blood circulations are fully separated. However, there’s no pumping chamber to propel blood through the lungs: Blood passively moves through the lungs and returns to the heart via the pulmonary veins.