Registry-based estimation of cardiac event–free survival in congenital heart disease complicated by pulmonary hypertension: A nationwide registry study from Japan
Rattachement africain : jp. Niveau de preuve : code pays fourni par la source.
Le résumé fourni par la source
Background: Pulmonary hypertension (PH) is a major determinant of outcomes in congenital heart disease (CHD), yet tools for individualized prognostic estimation are limited. This study aimed to develop an exploratory, clinically oriented prediction model for estimating cardiac event-free survival in patients with CHD-PH. Methods: Data from the nationwide Japanese Association of CHD-PH Registry were analyzed in a retrospective cohort derived from a prospectively maintained registry. Cardiac event-free survival was evaluated using Cox proportional hazards models incorporating prespecified, routinely available clinical and hemodynamic variables. Cardiac events were defined as death, transplantation, clinical worsening requiring treatment escalation, or PH-related hospitalization. Results: A total of 224 patients were included, and 23 experienced 29 cardiac events during a median follow-up of 1.5 years. These included 7 cardiovascular deaths, 1 lung transplantation, and 1 atrial septostomy, 13 cases of clinical worsening. The cardiac event-free survival rates were 94.0% and 86.2% at 1 and 2 years, respectively. In multivariable analysis, trisomy 21 (hazard ratio [HR] 3.63), elevated pulmonary vascular resistance index (PVRI; HR 13.2), and elevated central venous pressure (CVP; HR 3.27) were associated with worse outcomes. These associations were consistent in sensitivity and subgroup analyses. Model-based estimates demonstrated risk gradients across clinically relevant profiles and were incorporated into a prototype prediction tool. Conclusions: An exploratory pragmatic prediction model for cardiac event-free survival in CHD-PH was developed using nationwide registry data. These findings support the feasibility of a registry-based prognostic framework; however, further validation is required before clinical application.
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Le contrôle bibliographique ouvert
DOI retrouvé dans Crossref DOI retrouvé ; titre concordant.
- Titre Crossref
- Registry-based estimation of cardiac event–free survival in congenital heart disease complicated by pulmonary hypertension: A nationwide registry study from Japan
- Date Crossref
- 01/09/2026
- Éditeur
- Elsevier BV
- Type
- journal-article
Ce recoupement confirme des métadonnées liées au DOI. Il ne confirme ni la méthode ni les conclusions de l’étude, et il ne compte pas comme une seconde source scientifique indépendante.
Où se fait cette recherche
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Tokyo Medical and Dental University pays non établi dans la noticeUniversité ou école supérieure
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The University of Tokyo Department of Pediatrics pays non établi dans la noticeUniversité ou école supérieure
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Tokyo Medical University Department of Physiology pays non établi dans la noticeUniversité ou école supérieure
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Musashino Red Cross Hospital pays non établi dans la noticeÉtablissement de santé
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Keio University Hospital Department of Pediatrics pays non établi dans la noticeÉtablissement de santé
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The University of Osaka Department of Pediatrics pays non établi dans la noticeUniversité ou école supérieure
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Toyama University Hospital Department of Pediatrics pays non établi dans la noticeÉtablissement de santé
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Kyushu Hospital Department of Pediatrics pays non établi dans la noticeÉtablissement de santé
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Hokkaido University Department of Pediatrics pays non établi dans la noticeUniversité ou école supérieure
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Mie University Department of Pediatrics pays non établi dans la noticeUniversité ou école supérieure
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Fukuoka Children's Hospital and Medical Center for Infectious Diseases Department of Cardiology pays non établi dans la noticeÉtablissement de santé
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Tokyo Metropolitan Children's Medical Center Department of Cardiology pays non établi dans la noticeÉtablissement de santé
Tokyo Medical and Dental University, Department of Pediatrics — The University of Tokyo et Department of Physiology — Tokyo Medical University, avec 9 autres affiliations.
Une affiliation ne permet pas de déduire la nationalité d’un auteur.