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Pulmonary atresia-intact ventricular septum syndrome

Synonym(s) -
Prevalence -
Inheritance -
Age of onset -
  • Q22.6
  • C0344975
MeSH -
MedDRA -


Disease definition

Pulmonary atresia with intact ventricular septum (PA-IVS) is a rare form of cyanotic congenital heart malformation characterized by severe cyanosis and tachypnea. PA-IVS presents significant morphologic diversity: at the end of the spectrum are patients with a mildly hypoplastic and tripartite right ventricle (RV) and mild tricuspid valve (TV) hypoplasia, and at the other end are patients with severe RV and TV hypoplasia, often with RV-dependent coronary circulation.

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Detailed information

Clinical practice guidelines
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