Eisenmenger syndrome
Findings
No curated finding names Eisenmenger syndrome yet. Everything below is reference data from Mondo, HPO and GenCC, not evidence about what affects it.
Definition
Eisenmenger syndrome (ES) is an form of pulmonary arterial hypertension (PAH) associated with unoperated congenital heart disease and is characterized by congenital heart malformations with reversed or bi-directional shunting through an intra-cardiac or intervascular (usually aorto-pulmonary) communication with the development of PAH.
Definition from the Mondo Disease Ontology (MONDO:0019944), read 2026-09-29. CC BY 4.0.
Features
58 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 heart morphologyHPOHP:0001627
- Very frequent (80% to 99% of cases)
- Exercise intoleranceHPOHP:0003546
- Very frequent (80% to 99% of cases)
- Pulmonary arterial hypertensionHPOHP:0002092
- Very frequent (80% to 99% of cases)
- Atrial fibrillationHPOHP:0005110
- Frequent (30% to 79% of cases)
- Exertional dyspneaHPOHP:0002875
- Frequent (30% to 79% of cases)
- FatigueHPOHP:0012378
- Frequent (30% to 79% of cases)
- Heart murmurHPOHP:0030148
- Frequent (30% to 79% of cases)
- HypoxemiaHPOHP:0012418
- Frequent (30% to 79% of cases)
- Increased pulmonary vascular resistanceHPOHP:0005317
- Frequent (30% to 79% of cases)
- Muscle weaknessHPOHP:0001324
- Frequent (30% to 79% of cases)
- PalpitationsHPOHP:0001962
- Frequent (30% to 79% of cases)
- Supraventricular arrhythmiaHPOHP:0005115
- Frequent (30% to 79% of cases)
Show the remaining 46
- Supraventricular tachycardiaHPOHP:0004755
- Frequent (30% to 79% of cases)
- Abdominal distentionHPOHP:0003270
- Occasional (5% to 29% of cases)
- Abnormal circulating B-type natriuretic peptide concentrationHPOHP:0031138
- Occasional (5% to 29% of cases)
- Abnormality of the liverHPOHP:0001392
- Occasional (5% to 29% of cases)
- Angina pectorisHPOHP:0001681
- Occasional (5% to 29% of cases)
- Aortopulmonary windowHPOHP:0011604
- Occasional (5% to 29% of cases)
Where it sits
Other names
1 name
Resolves to: Eisenmenger syndrome
- Also called
- Eisenmenger's syndrome