Michael J. Landzberg, Curt J. Daniels, Paul Forfia, Vallerie V. McLaughlin, Karimah S. Bell Lynum, Mona Selej, Alexander R. Opotowsky
Harvard Medical School, Brigham and Women’s Hospital and Boston Children’s Hospital. Ohio State University College of Medicine and Nationwide Children’s Hospital. Temple University Hospital. University of Michigan Medical Center. Actelion Pharmaceuticals US, Inc. Cincinnati Children’s Hospital and University of Cincinnati College of Medicine.
United States
Journal of the American College of Cardiology Advances
JACC Adv 2023; 2:
DOI: 10.1016/j.jacadv.2023.100649
Abstract
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).
Category
Class I. Pulmonary Hypertension Associated with Congenital Cardiovascular Disease
Diagnostic Testing for Pulmonary Vascular Disease. Non-invasive Testing
Diagnostic Testing for Pulmonary Vascular Disease. Invasive Testing
Diagnostic Testing for Pulmonary Vascular Disease. Risk Stratification
Age Focus: Adult Pulmonary Vascular Disease
Fresh or Filed Publication: Filed (PHiled). Greater than 1-2 years since publication
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Free PDF File or Full Text Article Available Through PubMed or DOI: Yes