A scientific statement of the European Society of Cardiology Working Group on Pulmonary Circulation & Right Ventricular Function, the ESC Working Group on Adult Congenital Heart Disease (PAH-CHD), and the Association of Cardiovascular Nursing & Allied Professions of the ESC was recently published in the European Journal of Heart Failure.
The authors say that current guidelines recommend risk stratification models for pulmonary arterial hypertension that were largely derived from idiopathic pulmonary arterial hypertension cohorts and may not be well suited to patients with pulmonary arterial hypertension associated with congenital heart disease. The review examines the limitations of these models, with a particular focus on Eisenmenger syndrome.
The authors find that standard risk tools may miss important prognostic differences in pulmonary arterial hypertension associated with congenital heart disease patients due to distinct pathophysiology and clinical profiles. Factors such as shunt location, complexity of the underlying heart defect, degree of cyanosis, iron deficiency, syndromic comorbidities and biomarkers beyond BNP can all influence prognosis and are not captured by current models.
The authors conclude that disease-specific clinical and biological features should be incorporated into risk assessment to better serve this unique patient population.
Read more at this link on the European Journal of Heart Failure
Citation
D’Alto M, Giannakoulas G, Aboulhosn J, Akagi T, Arvanitaki A, Badagliacca R, Brida M, Bax J, Dimopoulos K, Escribano P, Kjellström B, Mroczek E, Rosenkranz S, Humbert M, Diller GP, Gatzoulis MA. Risk stratification for adult patients with pulmonary arterial hypertension associated with congenital heart disease. A scientific statement of the ESC Working Group on Pulmonary Circulation & Right Ventricular Function, the ESC Working Group on Adult Congenital Heart Disease, and the Association of Cardiovascular Nursing & Allied Professions of the ESC. Eur J Heart Fail. 2026 Mar 9:xuag059. doi: 10.1093/ejhf/xuag059. Epub ahead of print. PMID: 41800797.