Paediatrics: Tricuspid atresia

Tricuspid atresia

In tricuspid atresia (TA) there is no connection between the right atrium and the right ventricle. Venous blood is diverted to the left side via a patent foramen ovale. Pulmonary blood flow is dependent on associated VSD or PDA.

Clinical features

Most patients with TA present in the first few days to early months of life with increasing cyanosis. The clinical features will vary depending on other associated cardiac abnormalities. The ECG shows a superior axis.

Treatment

In an emergency, duct patency is achieved with prostaglandin E infusion. Surgical palliation and procedures include:

• Blalock–Taussig shunt (neonatal period).
• Pulmonary artery banding (neonatal period).
• Glenn shunt (6mths of age).
• Fontan procedure (pre-school).