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