{"title":"Updated review on pulmonary arterial hypertension: Differences between down syndrome and non-down syndrome populations","authors":"","doi":"10.1016/j.cpcardiol.2024.102840","DOIUrl":null,"url":null,"abstract":"<div><h3>Background</h3><p>Pulmonary arterial hypertension (PAH) is a major concern in patients with Down syndrome (DS) and congenital heart disease (CHD). Understanding the unique characteristics of PAH in these populations is essential for developing tailored management strategies. This review examines differences in PAH between DS and non-DS (nDS) patients with CHD, focusing on pathophysiology, clinical presentation, hemodynamic profiles, and treatment outcomes.</p></div><div><h3>Methods</h3><p>A retrospective analysis of 93 adults with PAH was conducted, including 18 with DS and 75 with CHD but without DS (nDS). Data on demographics, clinical presentations, comorbidities, and hemodynamic parameters were collected using echocardiography and right heart catheterization. Statistical analyses included Mann–Whitney U tests, Student's <em>t</em>-tests, and Kaplan–Meier survival analysis to compare the DS and nDS groups.</p></div><div><h3>Results</h3><p>DS patients presented with PAH at a younger age (mean age 25.06 years) compared to nDS patients (mean age 42.4 years; <em>p</em> < 0.001). Hypothyroidism was more prevalent in DS patients (61.1 %) than in nDS patients (29.3 %; <em>p</em> = 0.012). Hemodynamic assessments showed lower mean arterial pressure (MAP) in DS patients (76.24 ± 11.6 mmHg) versus nDS patients (93.95 ± 15 mmHg; <em>p</em> < 0.001), and a higher TAPSE/PASP ratio (0.41 vs. 0.23; <em>p</em> = 0.009), suggesting less severe right ventricular dysfunction. DS patients had a significant survival advantage over nDS patients (<em>p</em> = 0.043).</p></div><div><h3>Conclusions</h3><p>DS patients have distinct clinical and hemodynamic profiles in PAH, requiring personalized management. Early detection and tailored treatment are crucial for improving outcomes. Further research should refine these strategies and explore new therapies.</p></div>","PeriodicalId":51006,"journal":{"name":"Current Problems in Cardiology","volume":null,"pages":null},"PeriodicalIF":3.0000,"publicationDate":"2024-09-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"Current Problems in Cardiology","FirstCategoryId":"3","ListUrlMain":"https://www.sciencedirect.com/science/article/pii/S0146280624004754","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
Background
Pulmonary arterial hypertension (PAH) is a major concern in patients with Down syndrome (DS) and congenital heart disease (CHD). Understanding the unique characteristics of PAH in these populations is essential for developing tailored management strategies. This review examines differences in PAH between DS and non-DS (nDS) patients with CHD, focusing on pathophysiology, clinical presentation, hemodynamic profiles, and treatment outcomes.
Methods
A retrospective analysis of 93 adults with PAH was conducted, including 18 with DS and 75 with CHD but without DS (nDS). Data on demographics, clinical presentations, comorbidities, and hemodynamic parameters were collected using echocardiography and right heart catheterization. Statistical analyses included Mann–Whitney U tests, Student's t-tests, and Kaplan–Meier survival analysis to compare the DS and nDS groups.
Results
DS patients presented with PAH at a younger age (mean age 25.06 years) compared to nDS patients (mean age 42.4 years; p < 0.001). Hypothyroidism was more prevalent in DS patients (61.1 %) than in nDS patients (29.3 %; p = 0.012). Hemodynamic assessments showed lower mean arterial pressure (MAP) in DS patients (76.24 ± 11.6 mmHg) versus nDS patients (93.95 ± 15 mmHg; p < 0.001), and a higher TAPSE/PASP ratio (0.41 vs. 0.23; p = 0.009), suggesting less severe right ventricular dysfunction. DS patients had a significant survival advantage over nDS patients (p = 0.043).
Conclusions
DS patients have distinct clinical and hemodynamic profiles in PAH, requiring personalized management. Early detection and tailored treatment are crucial for improving outcomes. Further research should refine these strategies and explore new therapies.
期刊介绍:
Under the editorial leadership of noted cardiologist Dr. Hector O. Ventura, Current Problems in Cardiology provides focused, comprehensive coverage of important clinical topics in cardiology. Each monthly issues, addresses a selected clinical problem or condition, including pathophysiology, invasive and noninvasive diagnosis, drug therapy, surgical management, and rehabilitation; or explores the clinical applications of a diagnostic modality or a particular category of drugs. Critical commentary from the distinguished editorial board accompanies each monograph, providing readers with additional insights. An extensive bibliography in each issue saves hours of library research.