Isolated right ventricular hypoplasia
ORPHA:439· ICD-10 Q22.6
Definition
Isolated right ventricular hypoplasia (IRVH) is a rare congenital heart malformation characterized by underdevelopment of the right ventricle associated with patent foramen ovale or interauricular communication and normally developed tricuspid and pulmonary valves. IRVH manifests with severe cyanosis, congestive heart failure, and in severe cases, death in early infancy.
- Prevalence
- Unknown
- Inheritance
- Autosomal recessive, Not applicable
- Age of onset
- Infancy, Neonatal