囊性纤维化患者的心脏结构和功能
Lisa Steen Duus1, Maria Dons2, Rebekka Faber Thudium3
1Cardiovascular Non-Invasive Imaging Research Laboratory, Department of Cardiology, Copenhagen University Hospital - Herlev & Gentofte. Hospital, Gentofte Hospitalsvej 8, 2900 Hellerup, University of Copenhagen, Denmark.
概括
与健康个体相比,囊性纤维化 (CF) 患者的心脏功能异常率明显更高,包括左心室和右心室功能受损. 男性的性别和肺功能下降与CF中的这些心脏问题有关.
科学领域:
- 心脏病学 心脏病学
- 肺部病理学 肺部病理学
- 遗传学 是一个遗传学.
背景情况:
- 囊性纤维化 (CF) 是一种遗传性疾病,由于先进的治疗方法增加了预期寿命.
- 心脏参与CF并未完全理解,需要进一步调查.
- 新的囊性纤维化跨膜导电调节器 (CFTR) 调节器疗法需要对CF相关心脏病的最新研究.
研究的目的:
- 为了确定与匹配的对照人群相比,在患有CF (pwCF) 的人群中心脏变化的患病率.
- 在pwCF中识别与心脏功能障碍相关的潜在心血管风险因素.
- 评估CFTR调节器治疗对心脏功能的影响.
主要方法:
- 一项涉及104个pwCF和104个匹配对照的横截面研究.
- 对所有参与者进行了临床和心声回声评估.
- 使用多变量分析来调整潜在的混因素.
主要成果:
- 44%的pwCF表现出心脏功能异常,是对照组的两倍多.
- pwCF显示左心室尺寸较小,左心室扩张功能受损,右心室和左心室缩功能减少.
- 在pwCF中,男性性别和FEV1/FVC比率下降独立地与心脏功能异常有关.
结论:
- 在pwCF中存在显著的左侧和右侧心脏变化.
- 在对风险因素进行调整后,在pwCF中持续存在受损的RV和LV缩功能.
- 男性性别和肺功能减弱 (FEV1/FVC比率) 与CF患者的心脏功能异常有关.
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