Updated review on pulmonary arterial hypertension: Differences between down syndrome and non-down syndrome populations

IF 3 3区 医学 Q2 CARDIAC & CARDIOVASCULAR SYSTEMS
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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.

肺动脉高压最新综述:唐氏综合征与非唐氏综合征人群的差异
背景肺动脉高压(PAH)是唐氏综合征(DS)和先天性心脏病(CHD)患者的一个主要问题。了解这些人群 PAH 的独特特征对于制定有针对性的管理策略至关重要。本综述研究了患有先天性心脏病(CHD)的唐氏综合征患者与非唐氏综合征(nDS)患者在 PAH 方面的差异,重点关注病理生理学、临床表现、血液动力学特征和治疗效果。方法对 93 名患有 PAH 的成人患者进行了回顾性分析,其中包括 18 名患有唐氏综合征的患者和 75 名患有先天性心脏病但不患有唐氏综合征(nDS)的患者。通过超声心动图和右心导管检查收集了有关人口统计学、临床表现、合并症和血液动力学参数的数据。统计分析包括 Mann-Whitney U 检验、学生 t 检验和 Kaplan-Meier 生存分析,以比较 DS 组和 nDS 组。与 nDS 患者(29.3%;P = 0.012)相比,DS 患者中甲状腺功能减退症的发病率更高(61.1%)。血液动力学评估显示,DS 患者的平均动脉压(MAP)较低(76.24 ± 11.6 mmHg),而 nDS 患者的平均动脉压(93.95 ± 15 mmHg; p < 0.001)较高(TAPSE/PASP 比值为 0.41 vs. 0.23; p = 0.009),表明右心室功能障碍的严重程度较轻。与 nDS 患者相比,DS 患者具有明显的生存优势(p = 0.043)。早期发现和针对性治疗对改善预后至关重要。进一步的研究应完善这些策略并探索新的疗法。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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来源期刊
Current Problems in Cardiology
Current Problems in Cardiology 医学-心血管系统
CiteScore
4.80
自引率
2.40%
发文量
392
审稿时长
6 days
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.
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