isolated right ventricular hypoplasia
Findings
No curated finding names isolated right ventricular hypoplasia yet. Everything below is reference data from Mondo, HPO and GenCC, not evidence about what affects it.
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.
Definition from the Mondo Disease Ontology (MONDO:0010179), read 2026-09-29. CC BY 4.0.
Features
19 features
What the disease is reported to present with, and how often among people who have it — never how often a feature means the disease. HPO, annotations 2026-09-02.
- CyanosisHPOHP:0000961
- Very frequent (80% to 99% of cases)
- DyspneaHPOHP:0002094
- Very frequent (80% to 99% of cases)
- HypoxemiaHPOHP:0012418
- Very frequent (80% to 99% of cases)
- Right-to-left shuntHPOHP:0001694
- Very frequent (80% to 99% of cases)
- Atrial septal defectHPOHP:0001631
- Frequent (30% to 79% of cases)
- ClubbingHPOHP:0001217
- Frequent (30% to 79% of cases)
- FatigueHPO
Show the remaining 7
- Abnormal atrioventricular conductionHPOHP:0005150
- Occasional (5% to 29% of cases)
- Bidirectional shuntHPOHP:0012383
- Occasional (5% to 29% of cases)
- CardiomegalyHPOHP:0001640
- Occasional (5% to 29% of cases)
- Complete right bundle branch blockHPOHP:0011712
- Occasional (5% to 29% of cases)
- Congestive heart failureHPOHP:0001635
- Occasional (5% to 29% of cases)
- Muscular ventricular septal defectHPOHP:0011623
- Occasional (5% to 29% of cases)