Alexandra C van Dissel, Michele D'Alto, Andrea Farro, Harold Mathijssen, Marco C Post, Pier P Bassareo, Arie P J van Dijk, Barbara J M Mulder, Berto J Bouma
{"title":"Improved Risk Prediction Using a Refined European Guidelines Instrument in Pulmonary Arterial Hypertension Related to Congenital Heart Disease.","authors":"Alexandra C van Dissel, Michele D'Alto, Andrea Farro, Harold Mathijssen, Marco C Post, Pier P Bassareo, Arie P J van Dijk, Barbara J M Mulder, Berto J Bouma","doi":"10.1016/j.amjcard.2024.08.027","DOIUrl":null,"url":null,"abstract":"<p><p>European guidelines advocate a goal-oriented treatment approach in pulmonary arterial hypertension (PAH), based on a comprehensive risk assessment instrument, which has been validated in several PAH subgroups. We investigated its discriminatory ability, and explored tricuspid annular plane systolic excursion (TAPSE) and revised thresholds to improve its predictability within the adult congenital heart disease (CHD) population. In total, 223 adults (42±16 years, 66% female, 68% Eisenmenger) were enrolled from five European PAH-CHD expert centers. Patients were classified as 'Low', 'Intermediate', or 'High' risk at baseline visit and at follow-up within 4-18 months. By the general PAH guidelines instrument, survival did not differ between the risk groups (P=n.s.), mostly due to skewed group distribution. Reclassifying patients using revised thresholds for N-terminal pro-brain natriuretic peptide (NT-proBNP) and 6-minute walk distance (6MWD) (i.e., 'Low', 'Intermediate', 'High' as <500, 500-1400, >1400 ng/l and >400, 165-400 and <165 m, respectively) and use of TAPSE ('Low', 'Intermediate', 'High' as >20, 16-20 and <16mm) significantly improved discrimination between the risk groups both at baseline and follow-up (P=0.001, ROC increase from 0.648 to 0.701), reclassifying 64 (29%) patients. Irrespective of follow-up risk group, survival was better for patients with higher proportions of low-risk variables. Improvement to a 'Low-risk' profile at a median of 9 months follow-up provided improved survival comparable to survival of patients who remained in the 'Low risk' group. In conclusion, the external validity of general risk instrument for PAH appeared to be of limited discriminatory value in PAH-CHD patients. We propose a refined risk instrument with improved discrimination for PAH-CHD.</p>","PeriodicalId":7705,"journal":{"name":"American Journal of Cardiology","volume":null,"pages":null},"PeriodicalIF":2.3000,"publicationDate":"2024-09-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"American Journal of Cardiology","FirstCategoryId":"3","ListUrlMain":"https://doi.org/10.1016/j.amjcard.2024.08.027","RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q2","JCRName":"CARDIAC & CARDIOVASCULAR SYSTEMS","Score":null,"Total":0}
引用次数: 0
Abstract
European guidelines advocate a goal-oriented treatment approach in pulmonary arterial hypertension (PAH), based on a comprehensive risk assessment instrument, which has been validated in several PAH subgroups. We investigated its discriminatory ability, and explored tricuspid annular plane systolic excursion (TAPSE) and revised thresholds to improve its predictability within the adult congenital heart disease (CHD) population. In total, 223 adults (42±16 years, 66% female, 68% Eisenmenger) were enrolled from five European PAH-CHD expert centers. Patients were classified as 'Low', 'Intermediate', or 'High' risk at baseline visit and at follow-up within 4-18 months. By the general PAH guidelines instrument, survival did not differ between the risk groups (P=n.s.), mostly due to skewed group distribution. Reclassifying patients using revised thresholds for N-terminal pro-brain natriuretic peptide (NT-proBNP) and 6-minute walk distance (6MWD) (i.e., 'Low', 'Intermediate', 'High' as <500, 500-1400, >1400 ng/l and >400, 165-400 and <165 m, respectively) and use of TAPSE ('Low', 'Intermediate', 'High' as >20, 16-20 and <16mm) significantly improved discrimination between the risk groups both at baseline and follow-up (P=0.001, ROC increase from 0.648 to 0.701), reclassifying 64 (29%) patients. Irrespective of follow-up risk group, survival was better for patients with higher proportions of low-risk variables. Improvement to a 'Low-risk' profile at a median of 9 months follow-up provided improved survival comparable to survival of patients who remained in the 'Low risk' group. In conclusion, the external validity of general risk instrument for PAH appeared to be of limited discriminatory value in PAH-CHD patients. We propose a refined risk instrument with improved discrimination for PAH-CHD.
期刊介绍:
Published 24 times a year, The American Journal of Cardiology® is an independent journal designed for cardiovascular disease specialists and internists with a subspecialty in cardiology throughout the world. AJC is an independent, scientific, peer-reviewed journal of original articles that focus on the practical, clinical approach to the diagnosis and treatment of cardiovascular disease. AJC has one of the fastest acceptance to publication times in Cardiology. Features report on systemic hypertension, methodology, drugs, pacing, arrhythmia, preventive cardiology, congestive heart failure, valvular heart disease, congenital heart disease, and cardiomyopathy. Also included are editorials, readers'' comments, and symposia.