Tricuspid atresia is a congenital heart condition in which the tricuspid valve (the valve between the right atrium and right ventricle) is absent or completely obstructed, so no blood can flow directly from the right atrium into the right ventricle to be pumped to the lungs.
Survival after birth depends on alternative routes for blood to reach the lungs. Blood from the right atrium passes through an atrial septal defect or patent foramen ovale into the left atrium and mixes with oxygenated blood; some reaches the lungs via a ventricular septal defect (present in most cases) or via a patent ductus arteriosus. Because the right ventricle is often small and underdeveloped, tricuspid atresia is classified as a single ventricle condition: the left ventricle must support both the systemic and pulmonary circulations.
Management in the neonatal period involves prostaglandin infusion to maintain ductal patency and urgent surgical palliation. The standard surgical pathway follows the staged Fontan procedure: a series of operations over the first 2 to 5 years of life that ultimately direct systemic venous blood directly to the pulmonary arteries without passing through a right ventricle.
Adults who have completed Fontan palliation for tricuspid atresia are followed up in adult congenital heart disease (ACHD) services. Fontan complications including arrhythmias, protein-losing enteropathy, hepatic fibrosis, and declining cardiac function are important long-term concerns that require specialist surveillance and management.
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