Hypoplastic right heart syndrome

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Hypoplastic right heart syndrome
Classification and external resources
ICD-10 Q22.6
ICD-9 746.8 (CDC/BPA 746.882)

Hypoplastic right heart syndrome is a congenital heart defect in which the right atrium and right ventricle are underdeveloped.


A healthy heart has four valves, separated by flaps that open and close to control blood flow between the chambers. When your heart beats, oxygen-poor blood enters the right atrium. The blood then flows into the right ventricle, where it enters the pulmonary artery to travel to the lungs for oxygen. Oxygen-rich blood returns to the left atrium, where it then travels into the left ventricle. The left ventricle pushes the oxygenated blood into the aorta to be circulated to the rest of the body.[1]


In people with hypoplastic right heart syndrome, the heart is not able to adequately pump blood to the lungs. The result of this is an inadequate supply of oxygenated blood to be circulated to the body. The severity of underdevelopment varies for each individual. A special team of pediatric cardiologists is required to develop a treatment plan.

Diagnosis and prevalence[edit]

Hypoplastic right heart syndrome is less common than hypoplastic left heart syndrome. In some cases it can be identified before birth.[2]

It can be associated with aortic stenosis.[3]


There is no cure for hypoplastic right heart syndrome. A three-stage surgical procedure is commonly used to treat the condition. The surgeries rearrange the blood flow within the heart and allow the left ventricles to do the work for the underdeveloped right side of the heart. The three surgeries are spread out over the patients first few years of life. The first procedure, called the Norwood procedure is typically done within the first few days or weeks of life. The second procedure, called the Glenn procedure, is usually performed between four and twelve months of age. The last surgery, known as the Fontan procedure, is typically performed between the ages of 18 months and three years.


  1. ^ Heart Contraction and Blood Flow. National Institutes of Health. Retrieved September 18, 2014.
  2. ^ Paulick J, Tennstedt C, Schwabe M, Körner H, Bommer C, Chaoui R (May 2004). "Prenatal diagnosis of an isochromosome 5p in a fetus with increased nuchal translucency thickness and pulmonary atresia with hypoplastic right heart at 14 weeks". Prenat. Diagn. 24 (5): 371–4. doi:10.1002/pd.877. PMID 15164412. 
  3. ^ Sharma J, Friedman D, Schiller M, Flynn P, Alonso ML (December 1997). "Aortic stenosis in hypoplastic right heart syndrome, associated with interstitial deletion of chromosome 2". Int. J. Cardiol. 62 (3): 199–202. doi:10.1016/S0167-5273(97)00255-6. PMID 9476678.