Institute of Cardiovascular Sciences, University of Birmingham, Birmingham, UK.
Department of Cardiology, Queen Elizabeth Hospital, Birmingham, UK.
Open Heart. 2023 Jul;10(2). doi: 10.1136/openhrt-2023-002316.
Fabry disease (FD) is an X-linked lysosomal storage disorder caused by enzyme deficiency, leading to glycosphingolipid accumulation. Cardiac accumulation triggers local tissue injury, electrical instability and arrhythmia. Bradyarrhythmia and atrial fibrillation (AF) incidence are reported in up to 16% and 13%, respectively.
We conducted a systematic review evaluating AF burden and bradycardia requiring permanent pacemaker (PPM) implantation and report any predictive risk factors identified.
We conducted a literature search on studies in adults with FD published from inception to July 2019. Study outcomes included AF or bradycardia requiring therapy. Databases included Embase, Medline, PubMed, Web of Science, CINAHL and Cochrane. The Risk of Bias Agreement tool for Non-Randomised Studies (RoBANS) was utilised to assess bias across key areas.
11 studies were included, eight providing data on AF incidence or PPM implantation. Weighted estimate of event rates for AF were 12.2% and 10% for PPM. Age was associated with AF (OR 1.05-1.20 per 1-year increase in age) and a risk factor for PPM implantation (composite OR 1.03). Left ventricular hypertrophy (LVH) was associated with AF and PPM implantation.
Evidence supporting AF and bradycardia requiring pacemaker implantation is limited to single-centre studies. Incidence is variable and choice of diagnostic modality plays a role in detection rate. Predictors for AF (age, LVH and atrial dilatation) and PPM (age, LVH and PR/QRS interval) were identified but strength of association was low. Incidence of AF and PPM implantation in FD are variably reported with arrhythmia burden likely much higher than previously thought.
CRD42019132045.
法布里病（FD）是一种 X 连锁溶酶体贮积病，由酶缺乏引起，导致糖鞘脂积累。心脏蓄积引发局部组织损伤、电不稳定性和心律失常。据报道，缓慢性心律失常和心房颤动（AF）的发生率分别高达 16%和 13%。
我们进行了一项系统评价，评估 FD 患者的 AF 负荷和需要永久性起搏器（PPM）植入的缓慢性心律失常，并报告确定的任何预测风险因素。
我们对截至 2019 年 7 月发表的成人 FD 研究进行了文献检索。研究结局包括需要治疗的 AF 或缓慢性心律失常。数据库包括 Embase、Medline、PubMed、Web of Science、CINAHL 和 Cochrane。非随机研究的风险偏倚评估工具（RoBANS）用于评估关键领域的偏倚。
纳入 11 项研究，其中 8 项提供了 AF 发生率或 PPM 植入的数据。AF 的加权估计发生率分别为 12.2%和 10%。年龄与 AF 相关（年龄每增加 1 岁，OR 1.05-1.20），也是 PPM 植入的危险因素（复合 OR 1.03）。左心室肥厚（LVH）与 AF 和 PPM 植入相关。
支持 AF 和需要起搏器植入的缓慢性心律失常的证据仅限于单中心研究。发病率各不相同，诊断方式的选择在检测率中起着作用。确定了 AF（年龄、LVH 和心房扩张）和 PPM（年龄、LVH 和 PR/QRS 间隔）的预测因素，但关联强度较低。FD 中 AF 和 PPM 植入的发生率差异报告，心律失常负担可能远高于先前认为的那样高。
PROSPERO 数据库：CRD42019132045。
