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Genetic and Rare Diseases Information Center (GARD)

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Hypoplastic right heart syndrome


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Overview


Hypoplastic right heart syndrome is a rare heart defect, present at birth (congenital), that results in low blood oxygen levels. It is caused by underdevelopment of the structures on the right side of the heart (tricuspid valve, right ventricle, pulmonary valve, and pulmonary artery) and commonly associated with atrial septal defect. The underdeveloped right side of the heart is unable to provide enough blood flow to the body, leading to low blood oxygen and cyanosis.[1] It differs from hypoplastic left heart syndrome which involves the underdevelopment of the structures on the left side of the heart.
Last updated: 6/18/2013

References

  1. Dib, C. et al. Hypoplastic Right Heart Syndrome Presenting as Multiple Miscarriages. Texas Heart Institute Journal. 2012; 39(2):249-254. http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3384066/. Accessed 6/12/2013.
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In Depth Information

  • Orphanet is a European reference portal for information on rare diseases and orphan drugs.  Access to this database is free of charge.
  • PubMed is a searchable database of medical literature and lists journal articles that discuss Hypoplastic right heart syndrome. Click on the link to view a sample search on this topic.