Hypoplastic right heart syndrome is a spectrum of congenital heart conditions in which the right side of the heart (the right ventricle and its associated valves) is abnormally small and underdeveloped, severely limiting its ability to pump blood into the pulmonary circulation to be oxygenated. The spectrum includes hypoplastic right ventricle associated with pulmonary atresia or pulmonary stenosis, Ebstein’s anomaly (where the tricuspid valve is displaced downward and part of the right ventricle is ‘atrialized’), and tricuspid atresia (where the tricuspid valve is absent).
Survival after birth depends on alternative pathways for blood to reach the lungs: a patent ductus arteriosus (normally closing shortly after birth) must be kept open with prostaglandin infusion to maintain some pulmonary blood flow until surgical intervention can be performed. Without this bridging treatment, neonates deteriorate rapidly as the ductus closes.
Management depends on the specific anatomy and may involve staged palliative surgery aiming to use the left ventricle as the sole pump for the entire circulation, similar to the Fontan pathway used for other single-ventricle conditions. In some cases with milder right ventricular hypoplasia, biventricular repair may be feasible. These are complex decisions made by specialist paediatric cardiac surgical teams.
Adults with repaired hypoplastic right heart conditions are followed up in adult congenital heart disease (ACHD) specialist services. They may be at elevated risk of arrhythmia and require lifelong cardiac monitoring with regular imaging and electrophysiological review.
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