Risk stratification for adult patients with pulmonary arterial hypertension associated with congenital heart disease

For patients with pulmonary arterial hypertension (PAH), current guidelines recommend a 3- and 4-strata risk stratification model at baseline and follow-up, respectively. Risk stratification models in PAH are mainly derived from idiopathic PAH cohorts and are not automatically applicable to all patients with PAH associated with congenital heart disease (CHD), especially in those with Eisenmenger syndrome, given the differences in pathophysiology and clinical phenotype. Additional features such as shunt location, complexity of CHD, degree of cyanosis, iron deficiency, syndromic co-morbidity and biomarkers, other than brain natriuretic peptide may play an important role in the prognostication of these patients. This scientific statement aims to discuss in detail individual prognosticators and propose a comprehensive model of risk stratification for patients with PAH-CHD, mainly those with Eisenmenger syndrome.