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.
Authors
- Teiji Akagi (ORCID: https://orcid.org/0000-0001-8936-9565)
- Alexandra Arvanitaki (ORCID: https://orcid.org/0000-0003-3180-7280)
- Jamil Aboulhosn (ORCID: https://orcid.org/0000-0002-0209-9505)
- Margarita Brida (ORCID: https://orcid.org/0000-0001-8754-8156)
- Barbro Kjellström (ORCID: https://orcid.org/0000-0002-7936-1209)
- G Diller
- Ewa Mroczek
- P. Martín Escribano
- Konstantinos Dimopoulos
- Roberto Badagliacca
- Stephan Rosenkranz
- Michele D’Alto
- George Giannakoulas
- Marc Humbert
- Michael A Gatzoulis
- Jeroen Bax
Institutions
- Karolinska University Hospital (SE)
- University of Rijeka (HR)
- Université Paris-Saclay (FR)
- Leiden University Medical Center (NL)
- AHEPA University Hospital (GR)
- Ronald Reagan UCLA Medical Center (US)
- Harefield Hospital (GB)
- Wroclaw Medical University (PL)
- Royal Brompton Hospital (GB)
- Okayama University Hospital (JP)
- Hospital Universitario 12 De Octubre (ES)
- University Hospital Münster (DE)
- National Heart Hospital (BG)
- Ospedale Monaldi (IT)
- University Hospital Cologne (DE)
- Bicêtre Hospital (FR)
- Sapienza University of Rome (IT)
Publication Details
- Journal
- European Journal of Heart Failure
- Published
- 2026-03-04
- DOI
- https://doi.org/10.1093/ejhf/xuag059
- Primary Topic
- Pulmonary Hypertension Research and Treatments
- Type
- article
- Field-Weighted Citation Impact
- 0.00