Timely PAH Identification in Adults With Repaired Congenital Heart Disease? The ACHD-QuERI Registry Insights
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Le résumé fourni par la source
Background: The Quality Enhancement Research Initiative (QuERI) in adults with congenital heart disease (ACHD) was developed to improve detection of pulmonary arterial hypertension (PAH) after repair of systemic-to-pulmonary arterial shunt lesions. Objectives: This study sought to standardize use of accepted criteria for PAH diagnosis and evaluate utility in at-risk patients with ACHD. Methods: Patients ≥18 years of age with ACHD repaired ≥1 year before enrollment and with additional risk factors for developing PAH were eligible. History, physical examination, electrocardiogram, transthoracic echocardiogram, World Health Organization functional class, and 6-minute walk distance were evaluated at baseline and yearly for 3 years. Pop-up reminders of patient-specific evidence-based recommendations for PAH detection appeared during data entry. Results: Among 217 eligible patients, mean age (enrollment) was 44.0 ± 15.9 years, 72.3% were women, and 82.0% were World Health Organization functional class I. Electrocardiogram was performed in >80% and TTE in >70% of patients annually; capture of required transthoracic echocardiography (TTE) measures and alignment between study- and core-center interpretation improved over time, with more frequent assessment of pulmonary arterial flow acceleration time and documentation of right ventricular outflow tract Doppler notching. Approximately 40% of patients had ≥2 high-risk features for PAH on TTE, but only 7% (6/82) underwent right heart catheterization (RHC). Using current definitions, 2 patients were confirmed by RHC to have a diagnosis of PAH (maximum follow-up 3 years). Conclusions: A structured protocol may improve screening for patients with repaired ACHD at risk of developing PAH. RHC may be underutilized in patients with ACHD with TTE findings suggestive of PAH. (Adult Congenital Heart Disease Registry [QuERI]; NCT01659411).
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Le contrôle bibliographique ouvert
DOI retrouvé dans Crossref DOI retrouvé ; titre concordant.
- Titre Crossref
- Timely PAH Identification in Adults With Repaired Congenital Heart Disease? The ACHD-QuERI Registry Insights
- Date Crossref
- 01/11/2023
- 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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Brigham and Women's Hospital Department of Medicine pays non établi dans la noticeÉtablissement de santé
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Boston Children's Hospital Department of Cardiology pays non établi dans la noticeÉtablissement de santé
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Harvard University pays non établi dans la noticeUniversité ou école supérieure
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Nationwide Children's Hospital pays non établi dans la noticeÉtablissement de santé
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Temple University Hospital pays non établi dans la noticeÉtablissement de santé
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University of Michigan pays non établi dans la noticeUniversité ou école supérieure
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Sunesis (United States) pays non établi dans la noticeEntreprise
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Cincinnati Children's Hospital Medical Center Actelion Pharmaceuticals US pays non établi dans la noticeÉtablissement de santé
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University of Cincinnati Department of Pediatrics pays non établi dans la noticeUniversité ou école supérieure
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Harvard Medical School pays non établi dans la noticeInstitution
Department of Medicine — Brigham and Women's Hospital, Department of Cardiology — Boston Children's Hospital et Harvard University, avec 7 autres affiliations.
Une affiliation ne permet pas de déduire la nationalité d’un auteur.