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Cardiovascular Imaging

Hypoplastic right heart

cyanotic congenital anomaly consisting of any of a combination of tricuspid atresia, hypoplastic right ventricle, and pulmonary atresia with intact ventricular septum. Blood flow to the lungs is maintained early postpartum by a patent ductus arteriosus. Surgical construction of a systemic to pulmonary shunt is performed in the neonate to maintain pulmonary blood flow. Subsequent surgical treatment involves Glenn procedure and/or Fontan procedure. Chest X-ray usually shows pulmonary oligaemia and little or no cardiomegaly. Cardiomegaly is found infrequently and usually indicates a restrictive atrial septal defect or severe tricuspid regurgitation. Diagnosis is usually established by echocardiography. It can also be done with cardiac angiography and MRI. MRI can precisely demonstrate the size of the right ventricle (Fig.1).

CBH

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Fig.1

ECG-gated spin-echo coronal image demonstrates a severely hypoplastic right ventricle (arrow). The left ventricle is enlarged.
Hypoplastic right heart, Fig.1