Isolated right ventricular hypoplasia

Orpha code: 439OMIM code: 277200

Definition

Isolated right ventricular hypoplasia (IRVH) is a rare congenital heart malformation (see this term) characterized by underdevelopment of the right ventricle associated with patent foramen ovale or interauricular communication (see these terms) and normally developed tricuspid and pulmonary valves. IRVH manifests with severe cyanosis, congestive heart failure, and in severe cases, death in early infancy.

Disease data
Classification

Morphological anomaly

ORPHA code
439
OMIM code
277200
ICD10 code
Q22.6
ICD11 code
LA88.Y

No additional description.

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