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March 4, 2026European Journal of Heart Failure

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

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Why the study?

Current PAH risk stratification models are mainly derived from idiopathic PAH cohorts and are not automatically applicable to PAH associated with congenital heart disease, particularly Eisenmenger syndrome.

Population

Adult patients with PAH-CHD, mainly those with Eisenmenger syndrome

Design

Scientific statement

Authors

MDMichele D’AltoGGGeorge GiannakoulasJAJamil Aboulhosn

Discussion

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Overview

Supports tailored prognostication in PAH-CHD; extends generic PAH models with CHD-specific factors.

Structured PICO

P
Population
Adult patients with pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD), mainly those with Eisenmenger syndrome

This scientific statement proposes a tailored, comprehensive risk stratification model for patients with PAH-CHD, particularly Eisenmenger syndrome, addressing the limitations of applying idiopathic PAH models to this population.

Cite This Study

D’Alto et al. (2026) studied this question.

synapsesocial.com/papers/69fa8a199fa336b96d47d904https://doi.org/10.1093/ejhf/xuag059
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Also Consider

Synapse has enriched 5 closely related papers on similar clinical questions. Consider them for comparative context:

  1. 1Pulmonary arterial hypertension related to congenital heart disease with a left-to-right shunt: phenotypic spectrum and approach to management2024 · 5 citations
  2. 2Potts Shunt Improves Right Ventricular Function and Coupling With Pulmonary Circulation in Children With Suprasystemic Pulmonary Arterial Hypertension2018 · 58 citations
  3. 3Eisenmenger syndrome. Factors relating to deterioration and death1998 · 527 citations
  4. 42020 ESC Guidelines for the management of adult congenital heart disease2020 · 2,295 citations
  5. 52025 ESC Guidelines for the management of cardiovascular disease and pregnancy2025 · 268 citations