Life span of patients with Eisenmenger syndrome is not superior to that of patients with other causes of pulmonary hypertension

Pulmonary hypertension (PH) carries heterogeneous prognosis due to heterogeneous mechanisms. Congenital heart diseases can be associated with pulmonary arterial hypertension (PAH) through different conditions. Eisenmenger syndrome (ES) is one of the conditions in which a congenital heart disease that initially causes left-to-right shunt, induces an increase in pulmonary vascular resistance which eventually results in reversal of the direction of the shunt and development of cyanosis (1). There is an emerging percentage of adult patients with repaired congenital heart disease (2) developing PAH despite the closure of the shunt: the closed-shunt associated with PAH group. In the recently-published adult congenital heart disease Dutch registry, the overall prevalence of PAH in septal defects was 6% (3). In this group, PAH was related to ES in only 58% of the cases. Among the 42% remaining patients, some of them had PAH occurring despite closure of the heart defect