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Congenital heart disease characterized by underdevelopment of the right-sided structures of the heart, which causes inadequate blood flow to the lungs, cyanosis, and abnormal coronary supply, causing damage to the heart muscle.

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Congenital Isolated Hypoplasia of the Right Ventricle; Hypoplastic Right Heart Syndrome; Hypoplastic Right Ventricle.

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Autosomal recessive.

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In its usual form, there is attenuation of the apical trabecular zone of the right ventricle with a normal and nonobstructed pulmonary outflow tract. The tricuspid valve may be hypoplastic or frankly obstructive.

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Electrocardiography demonstrates sinus rhythm with right atrial enlargement. Chest radiography shows mild cardiac enlargement with an enlarged right heart border, decreased pulmonary segment, and reduced vascularity.

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Significant cyanosis at birth. Signs of venous congestion with hepatomegaly may occur within the first week. Death from progressive hypoxemia is common if appropriate therapy is not commenced.

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Adequate treatment of hypoxemia and heart failure. Echocardiography and cardiac catheterization are recommended to assess cardiac function and degree of right ventricular inflow obstruction. Need to exclude tetralogy of Fallot, Ebstein anomaly of the tricuspid valve, pulmonary atresia with intact septum, and tricuspid atresia with pulmonary stenosis.

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Right ventricular filling is restricted because of the small tricuspid valve and hypoplastic right ventricle. This situation necessitates unimpeded blood flow from the right atrium to the left atrium, which is commonly achieved with balloon atrioseptostomy at birth. Anesthesia may be required later for aortopulmonary shunt, Glenn anastomosis (superior vena cava to right pulmonary artery), or Fontan operation (right atrium to right ventricle anastomosis).

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Determined by cardiac function.

Joy MV, Venugopalan P, Sapru A, et al: Isolated hypoplasia of right ventricle with atrial septal defect: A rare form of cyanotic heart disease. Indian Heart J 51:440, 1999.  [PubMed: 10547948]
Medd, WE, Neufeld HN: Isolated hypoplasia of the right ventricle and tricuspid valve in siblings. Br Heart J 23:25, 1961.
Metras D, Chetaille P, Kreitmann B, et al: Pulmonary atresia with ventricular septal defect, extremely hypoplastic pulmonary arteries, major aorto-pulmonary collaterals. Eur J Cardiothorac Surg 20:590, 2001.  [PubMed: 11509284]

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