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 V
3 days ago
- #risk stratification
- #pulmonary arterial hypertension
- #congenital heart disease
- Current guidelines recommend 3- and 4-strata risk stratification models for pulmonary arterial hypertension (PAH) at baseline and follow-up.
- Risk stratification models for PAH are mainly derived from idiopathic PAH cohorts and may not fully apply to PAH associated with congenital heart disease (CHD), especially Eisenmenger syndrome.
- Differences in pathophysiology and clinical phenotype in PAH-CHD patients necessitate additional considerations for risk stratification.
- Factors like shunt location, CHD complexity, cyanosis degree, iron deficiency, syndromic co-morbidity, and specific biomarkers are important for prognostication in PAH-CHD.
- This scientific statement proposes a comprehensive risk stratification model for PAH-CHD patients, focusing on Eisenmenger syndrome.