Eisenmenger Syndrome: The Pulmonology Perspective

Eisenmenger syndrome (ES) is an advanced form of pulmonary arterial hypertension associated with congenital heart disease, marked by pulmonary vascular disease and right-to-left or bidirectional shunting. Although vascular remodeling is central, lung involvement is still underrecognized.

This review describes pulmonary and related aspects that accompany ES and their contribution to symptoms, prognosis, and management. A restrictive pattern is common on function testing, whereas obstructive defects and bronchial hyperresponsiveness may occur even without typical risk factors. Diffusion impairment related to vascular disease is frequent and linked to reduced exercise capacity and worse survival. Exercise intolerance reflects the combined effects of pulmonary vascular disease, shunt-related hypoxemia, and ventilation-perfusion mismatch, which increase ventilatory demand, promote early anaerobic metabolism, and reduce peak oxygen uptake. Cardiopulmonary exercise testing and the 6-minute walk test provide prognostic information, with peak oxygen consumption emerging as the strongest predictor. An additional complication is marked pulmonary artery dilatation, predisposing to in situ thrombosis and compressive syndromes. Hemoptysis, often bronchial in origin, requires a structured emergency pathway. Lower respiratory tract infections are relatively common and may increase morbidity and mortality. Long-term oxygen therapy provides limited benefit because hypoxemia is mainly shunt-driven; supervised rehabilitation is safe and can improve functional capacity. In advanced disease refractory to treatment, bilateral lung or heart-lung transplantation remains the only definitive option, despite suboptimal outcomes.

ES is a multisystem disorder in which pulmonary abnormalities meaningfully influence symptoms and prognosis; systematic lung assessment can refine risk stratification and support individualized supportive care by integrated cardiology-pulmonology teams.