congenital total pulmonary venous return anomaly
Findings
No curated finding names congenital total pulmonary venous return anomaly yet. Everything below is reference data from Mondo, HPO and GenCC, not evidence about what affects it.
Definition
Total pulmonary venous return (TAPVR) is a form of congenital pulmonary venous return where all of the pulmonary veins drain into the right atrium or one of its tributaries, instead of the left atrium, leading to various manifestations such as fatigue, exertional dyspnea, pulmonary arterial hypertension, cyanosis and progressive congestive heart failure.
Definition from the Mondo Disease Ontology (MONDO:0007130), read 2026-09-29. CC BY 4.0.
Features
40 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.
- Respiratory distressHPOHP:0002098
- Very frequent (80% to 99% of cases)
- Atrial septal defectHPOHP:0001631
- Frequent (30% to 79% of cases)
- Atrial situs ambiguousHPOHP:0011539
- Frequent (30% to 79% of cases)
- CyanosisHPOHP:0000961
- Frequent (30% to 79% of cases)
- Exertional dyspneaHPOHP:0002875
- Frequent (30% to 79% of cases)
- FatigueHPOHP:0012378
- Frequent (30% to 79% of cases)
- Paroxysmal dyspneaHPO
Show the remaining 28
- CardiomegalyHPOHP:0001640
- Occasional (5% to 29% of cases)
- DextrocardiaHPOHP:0001651
- Occasional (5% to 29% of cases)
- Double outlet right ventricleHPOHP:0001719
- Occasional (5% to 29% of cases)
- HepatomegalyHPOHP:0002240
- Occasional (5% to 29% of cases)
- HeterotaxyHPOHP:0030853
- Occasional (5% to 29% of cases)
- Hypoplastic left ventricleHPOHP:0004383
- Occasional (5% to 29% of cases)
Where it sits
Other names
2 names
Resolves to: congenital total pulmonary venous return anomaly
- Also called
- TAPVRtotal anomalous pulmonary venous return