Tricuspid atresia

Tricuspid atresia (also known as tricuspid valve atresia) is a congenital heart defect in which the tricuspid valve — the valve that normally connects the right atrium to the right ventricle — fails to develop. Instead of a functioning valve, there is a solid plate of tissue blocking blood flow from the right atrium into the right ventricle. This results in an underdeveloped (hypoplastic) right ventricle and forces blood to follow abnormal pathways through the heart. To sustain life, an atrial septal defect (ASD) or patent foramen ovale must be present to allow blood to pass from the right atrium to the left atrium, and a ventricular septal defect (VSD) or patent ductus arteriosus (PDA) is typically needed to allow some blood to reach the lungs for oxygenation. Key symptoms present at birth or shortly after include cyanosis (bluish discoloration of the skin due to low oxygen levels), tachypnea (rapid breathing), poor feeding, and failure to thrive. The severity of symptoms depends on the degree of pulmonary blood flow, which is determined by the size of associated defects such as the VSD and the relationship of the great arteries. Some patients may also have transposition of the great arteries, which further complicates the hemodynamic picture. Without intervention, the condition is fatal. Treatment for tricuspid atresia is surgical and typically involves a staged approach over the first few years of life. Initial palliation may include a Blalock-Taussig shunt or pulmonary artery banding in the neonatal period, followed by a bidirectional Glenn procedure (superior cavopulmonary connection) at approximately 4–6 months of age, and ultimately the Fontan procedure (total cavopulmonary connection) at 2–4 years of age. The Fontan circulation allows deoxygenated blood to flow passively to the lungs without a functioning right ventricle. Long-term follow-up is essential, as patients with Fontan physiology may develop complications including arrhythmias, protein-losing enteropathy, liver dysfunction, and heart failure. Advances in surgical techniques and medical management have significantly improved survival, though lifelong cardiac care is required.

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