scimitar syndrome
MONDO:0015987Mondo
Findings
No curated finding names scimitar syndrome yet. Everything below is reference data from Mondo, HPO and GenCC, not evidence about what affects it.
Definition
Scimitar syndrome is characterized by a combination of cardiopulmonary anomalies including partial anomalous pulmonary venous return connection of the right lung to the inferior caval vein leading to the creation of a left-to-right shunt.
Definition from the Mondo Disease Ontology (MONDO:0015987), read 2026-09-29. CC BY 4.0.
Features
39 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.
- Abnormal cardiovascular system morphologyHPOHP:0030680
- Frequent (30% to 79% of cases)
- Abnormal heart morphologyHPOHP:0001627
- Frequent (30% to 79% of cases)
- Abnormal lung morphologyHPOHP:0002088
- Frequent (30% to 79% of cases)
- Abnormal vena cava morphologyHPOHP:0005345
- Frequent (30% to 79% of cases)
- Atrial septal defectHPOHP:0001631
- Frequent (30% to 79% of cases)
- Coarctation of aortaHPOHP:0001680
- Frequent (30% to 79% of cases)
- Congestive heart failureHPOHP:0001635
- Frequent (30% to 79% of cases)
- DextrocardiaHPOHP:0001651
- Frequent (30% to 79% of cases)
- Pulmonary artery hypoplasiaHPOHP:0004971
- Frequent (30% to 79% of cases)
- Pulmonary hypoplasiaHPOHP:0002089
- Frequent (30% to 79% of cases)
- Ventricular septal defectHPOHP:0001629
- Frequent (30% to 79% of cases)
- Abnormal hemidiaphragm morphologyHPOHP:0040045
- Occasional (5% to 29% of cases)
Show the remaining 27
- Anomalous pulmonary venous returnHPOHP:0010772
- Occasional (5% to 29% of cases)
- Bronchogenic cystHPOHP:0100730
- Occasional (5% to 29% of cases)
- CoughHPOHP:0012735
- Occasional (5% to 29% of cases)
- Descending aorta hypoplasiaHPOHP:0025495
- Occasional (5% to 29% of cases)
- Double outlet right ventricleHPOHP:0001719
- Occasional (5% to 29% of cases)
- HerniaHPOHP:0100790
- Occasional (5% to 29% of cases)
Where it sits
Other names
4 names
Resolves to: scimitar syndrome
- Also called
- congenital pulmonary venolobar syndromeEpibronchial right pulmonary vein syndromeHalasz syndromehypogenetic lung syndrome