Hypoplastic right heart syndrome is a congenital cardiac anomaly. It is characterized by an underdeveloped right side of the heart, including the right ventricle, tricuspid valve, pulmonary valve, and pulmonary arteries.
It may be present in around 1.1% of stillbirths and is rarer than hypoplastic left heart syndrome.
Neonates are cyanotic at birth.
It is caused by decreased blood flow in and out of the right ventricle during development. The most common right-sided defect to cause this syndrome is pulmonary atresia with an intact ventricular septum. Coronary arterial supply may be also abnormal.
Prenatal ultrasound may show a small right ventricle with hypertrophy and a small or absent pulmonary artery, with decreased or absent flow through the tricuspid valve and pulmonary valve 1.
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