Eisenmenger syndrome: from congenital shunt to multiorgan impact

Eisenmenger syndrome (ES) is a complex condition resulting from congenital heart defects that lead to significant anatomical shunts, primarily characterized by pulmonary arterial hypertension (PAH) and reversed shunting. Initially described by Victor Eisenmenger and later defined by Paul Wood, ES develops when a left-to-right shunt, due to congenital defects such as ventricular septal defects (VSD) oratrial septal defects (ASD), progresses to a right-to-left shunt as pulmonary vascular resistance increases. This condition manifests with symptoms including cyanosis, dyspnea, and fatigue, and can lead to severe complications such as heart failure, thrombo embolic events, and premature mortality. The natural history of ES is influenced by the size of the shunt and the presence of comorbidities, with a Significant impact on life expectancy. Diagnosis involves a combination of clinical assessment, imaging, and hemodynamic evaluation, while management strategies focus on symptom relief and improving hemodynamics, with limited surgical options. Despite advancements in treatment, ES remains associated with high morbidity and mortality, necessitating a multidisciplinary approach for optimal patient care.