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Eisenmenger Syndrome: The Pulmonology Perspective
Bianca Baino1, Michele D'Alto2, Konstantinos Dimopoulos3
1Respiratory Medicine Section, Department of Clinical Medicine and Surgery, Federico II University, Naples, Italy.
Chest
|June 24, 2026
Summary
Eisenmenger syndrome (ES) involves significant lung issues impacting pulmonary arterial hypertension and congenital heart disease. Understanding these pulmonary aspects is crucial for managing ES symptoms and prognosis.
Area of Science:
- Cardiology
- Pulmonology
- Genetics
Background:
- Eisenmenger syndrome (ES) is an advanced stage of pulmonary arterial hypertension (PAH) linked to congenital heart disease (CHD).
- Pulmonary vascular disease (PVD) and shunting are key features, but lung involvement is often overlooked.
- ES presents complex challenges due to multisystemic involvement.
Purpose of the Study:
- To review the pulmonary and related aspects of Eisenmenger syndrome.
- To elucidate the contribution of pulmonary factors to ES symptoms, prognosis, and management.
- To highlight the importance of systematic lung assessment in ES.
Main Methods:
- This is a review article.
- It synthesizes information on pulmonary manifestations in ES.
- It discusses diagnostic tools and prognostic indicators.
Main Results:
- Restrictive lung function patterns are common; obstructive defects and bronchial hyperresponsiveness can also occur.
- Impaired diffusion capacity due to PVD is frequent, correlating with reduced exercise capacity and survival.
- Exercise intolerance stems from PVD, hypoxemia, and ventilation-perfusion mismatch.
- Pulmonary artery dilatation, thrombosis, hemoptysis, and infections are significant complications.
- Cardiopulmonary exercise testing and the six-minute walk test offer prognostic value, with peak VO2 being a strong predictor.
Conclusions:
- Pulmonary abnormalities significantly impact ES symptoms and prognosis.
- Systematic lung assessment aids in risk stratification and personalized care.
- Integrated cardiology-pulmonology teams are essential for managing ES.
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