{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,28]],"date-time":"2026-08-28T23:31:50Z","timestamp":1787959910285,"version":"build-2784847793"},"reference-count":30,"publisher":"Oxford University Press (OUP)","license":[{"start":{"date-parts":[[2026,3,9]],"date-time":"2026-03-09T00:00:00Z","timestamp":1773014400000},"content-version":"am","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/pages\/standard-publication-reuse-rights"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Aims<\/jats:title>\n                    <jats:p>Current guidelines recommend a 3-strata risk model at baseline and a 4-strata model at follow-up for patients with pulmonary arterial hypertension (PAH). However, most risk stratification tools have been derived from cohorts with idiopathic PAH and may not be directly applicable to patients with PAH associated with congenital heart disease (PAH-CHD). This study aims to highlight the limitations of current risk models in this population and to discuss additional clinical features that may contribute to improved prognostic assessment.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>We reviewed the applicability of current guideline-recommended PAH risk stratification models in patients with PAH-CHD, with particular focus on Eisenmenger syndrome. Clinical characteristics and disease-specific factors that may influence prognosis in this population were examined.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>Standard PAH risk stratification models may not fully capture the prognostic heterogeneity of PAH-CHD patients due to important differences in pathophysiology and clinical phenotype compared with idiopathic PAH. Additional factors such as shunt location, complexity of CHD, degree of cyanosis, iron deficiency, syndromic comorbidities, and biomarkers beyond brain natriuretic peptide, may significantly influence prognosis and should be considered when assessing risk in this population.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>Current guideline-based risk stratification models have limitations when applied to patients with PAH-CHD, particularly those with Eisenmenger syndrome. Incorporating disease-specific clinical and biological features may improve prognostic assessment and risk stratification in this unique patient population.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1093\/ejhf\/xuag059","type":"journal-article","created":{"date-parts":[[2026,3,4]],"date-time":"2026-03-04T12:48:06Z","timestamp":1772628486000},"source":"Crossref","is-referenced-by-count":1,"title":["Risk stratification for adult patients with pulmonary arterial hypertension associated with congenital heart disease"],"prefix":"10.1093","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-5729-1038","authenticated-orcid":false,"given":"Michele","family":"D\u2019Alto","sequence":"first","affiliation":[{"name":"Adult Congenital Heart Disease Unit, Department of Cardiology, Monaldi Hospital , Via L. Bianchi, 80131 - Naples ,","place":["Italy"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7491-6319","authenticated-orcid":false,"given":"George","family":"Giannakoulas","sequence":"additional","affiliation":[{"name":"Cardiology Department, Aristotle University of Thessaloniki, AHEPA University Hospital , Thessaloniki ,","place":["Greece"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0209-9505","authenticated-orcid":false,"given":"Jamil","family":"Aboulhosn","sequence":"additional","affiliation":[{"name":"Ahmanson\/UCLA Adult Congenital Heart Disease Center, Ronald Reagan Medical Center at UCLA , Los Angeles ,","place":["USA"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Teiji","family":"Akagi","sequence":"additional","affiliation":[{"name":"Adult Congenital Heart Disease Center, Department of Cardiovascular Medicine, Okayama University Hospital , Okayama ,","place":["Japan"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3180-7280","authenticated-orcid":false,"given":"Alexandra","family":"Arvanitaki","sequence":"additional","affiliation":[{"name":"Adult Congenital Heart Centre and National Centre for Pulmonary Hypertension, Royal Brompton and Harefield Hospitals, Guy\u2019s and St Thomas\u2019s NHS Foundation Trust, Imperial College , London ,","place":["UK"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Roberto","family":"Badagliacca","sequence":"additional","affiliation":[{"name":"Sapienza University of Rome, Cardiovascular and Respiratory Science, Pulmonary Hypertension Centre , Rome ,","place":["Italy"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8754-8156","authenticated-orcid":false,"given":"Margarita","family":"Brida","sequence":"additional","affiliation":[{"name":"Department of Sports and Rehabilitation Medicine, Medical Faculty University of Rijeka , Rijeka ,","place":["Croatia"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7368-0500","authenticated-orcid":false,"given":"Jeroen","family":"Bax","sequence":"additional","affiliation":[{"name":"Cardiology, Leiden University Medical Center , Leiden ,","place":["Netherlands"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Konstantinos","family":"Dimopoulos","sequence":"additional","affiliation":[{"name":"Royal Brompton Hospital and National Heart and Lung Institute, Imperial College London , London ,","place":["UK"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Pilar","family":"Escribano","sequence":"additional","affiliation":[{"name":"Pulmonary Hypertension Unit, Cardiology Department, Hospital Universitario 12 de Octubre , Madrid ,","place":["Spain"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Barbro","family":"Kjellstr\u00f6m","sequence":"additional","affiliation":[{"name":"Department of Cardiology, Karolinska Institutet and Karolinska University Hospital , Stockholm ,","place":["Sweden"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ewa","family":"Mroczek","sequence":"additional","affiliation":[{"name":"Department of Cardiology, Wroclaw Medical University , Wroklaw ,","place":["Poland"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6237-1470","authenticated-orcid":false,"given":"Stephan","family":"Rosenkranz","sequence":"additional","affiliation":[{"name":"Clinic III for Internal Medicine (Department of Cardiology, Pulmonology and Intensive Care Medicine), and Cologne Cardiovascular Research Center (CCRC), Heart Center at the University Hospital Cologne , K\u00f6ln ,","place":["Germany"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marc","family":"Humbert","sequence":"additional","affiliation":[{"name":"Faculty of Medicine, Universit\u00e9 Paris-Saclay , Le Kremlin-Bic\u00eatre ,","place":["France"]},{"name":"Service de Pneumologie et Soins Intensifs Respiratoires, Centre de R\u00e9f\u00e9rence de L\u2019Hypertension Pulmonaire, H\u00f4pital Bic\u00eatre, Assistance Publique H\u00f4pitaux de Paris , Le Kremlin-Bic\u00eatre ,","place":["France"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gerhard P","family":"Diller","sequence":"additional","affiliation":[{"name":"Department of Cardiology III-Adult Congenital and Valvular Heart Disease, University Hospital Muenster , Muenster 48149 ,","place":["Germany"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2700-7232","authenticated-orcid":false,"given":"Michael 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