double outlet right ventricle
MONDO:0018089Mondo
Findings
No curated finding names double outlet right ventricle yet. Everything below is reference data from Mondo, HPO and GenCC, not evidence about what affects it.
Definition
Double outlet right ventricle (DORV) is a rare cono-truncal anomaly in which both the aorta and pulmonary artery originate, either entirely or predominantly, from the morphologic right ventricle.
Definition from the Mondo Disease Ontology (MONDO:0018089), read 2026-09-29. CC BY 4.0.
Features
28 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.
- Double outlet right ventricleHPOHP:0001719
- Obligate (100% of cases)
- CyanosisHPOHP:0000961
- Very frequent (80% to 99% of cases)
- Abnormal external ear cartilage morphologyHPOHP:3000022
- Frequent (30% to 79% of cases)
- Cleft palateHPOHP:0000175
- Frequent (30% to 79% of cases)
- Depressed nasal bridgeHPOHP:0005280
- Frequent (30% to 79% of cases)
- Failure to thriveHPOHP:0001508
- Frequent (30% to 79% of cases)
- Feeding difficultiesHPOHP:0011968
- Frequent (30% to 79% of cases)
- Heart murmurHPOHP:0030148
- Frequent (30% to 79% of cases)
- HypertelorismHPOHP:0000316
- Frequent (30% to 79% of cases)
- Hypoplastic left ventricleHPOHP:0004383
- Frequent (30% to 79% of cases)
- Mild intellectual disabilityHPOHP:0001256
- Frequent (30% to 79% of cases)
- Narrow mouthHPOHP:0000160
- Frequent (30% to 79% of cases)
Show the remaining 16
- Narrow palpebral fissureHPOHP:0045025
- Frequent (30% to 79% of cases)
- Pulmonic stenosisHPOHP:0001642
- Frequent (30% to 79% of cases)
- Short statureHPOHP:0004322
- Frequent (30% to 79% of cases)
- Submucous cleft hard palateHPOHP:0000176
- Frequent (30% to 79% of cases)
- TachycardiaHPOHP:0001649
- Frequent (30% to 79% of cases)
- TachypneaHPOHP:0002789
- Frequent (30% to 79% of cases)
Where it sits
- Narrower terms (5)
- double outlet right ventricle with atrioventricular septal defect, pulmonary stenosis, heterotaxy
- double outlet right ventricle with non-committed subpulmonary ventricular septal defect
- double outlet right ventricle with subaortic or doubly committed ventricular septal defect
- double outlet right ventricle with subaortic or doubly committed ventricular septal defect with pulmonary stenosis
- double outlet right ventricle with subpulmonary ventricular septal defect
Other names
1 name
Resolves to: double outlet right ventricle
- Also called
- DORV