Chair of Cardiology, Department of Translational Medical Sciences, University of Campania "Luigi Vanvitelli" Monaldi Hospital, Naples, Italy.
Chair of Cardiology, Department of Translational Medical Sciences, University of Campania "Luigi Vanvitelli" Monaldi Hospital, Naples, Italy.
Neuromuscul Disord. 2021 Apr;31(4):281-290. doi: 10.1016/j.nmd.2021.01.002. Epub 2021 Jan 9.
Cardiac involvement is recorded in about 80% of patients affected by myotonic dystrophy type 1 (DM1). The prevalence of cardiac conduction abnormalities is well described. Data regarding the prevalence of atrial fibrillation (AF) are still conflicting. The primary objective of this review was to assess the prevalence of AF in DM1. The secondary aim was to examine the association of clinical features with AF, to detect predisposing and/or influencing prognosis factors. A systematic search was developed in MEDLINE, EMBASE, Cochrane Register of Controlled Trials and Web of Science databases, to identify original reports between January 1, 2002 and January 30, 2020, assessing the prevalence of AF in DM1 population. Retrospective/prospective cohort studies and case series describing the prevalence of atrial fibrillation evaluated by periodic electrocardiogram (ECG) and/or ECG Holter 24 h, external loop recording (ELR) and implantable devices interrogation in DM1 patients were included. Case reports, simple reviews, commentaries and editorials were excluded. Thirteen reports fulfilled eligibility criteria and were included in our systematic review. According to the results from all the evaluated studies, the mean prevalence of AF in DM1 patients was 10.9% (n = 404) in 3677 DM1 patients. Male sex, conduction defects, echocardiographic findings of prolonged atrial electromechanical delay seem to be strongly associated with atrial fibrillation, representing factors favoring its onset. DM1 patients who develop AF seem to have a higher risk of cardiovascular and non-cardiovascular death. Further studies are needed to assess the prevalence of AF in DM1 patients and to investigate ECG abnormalities and other clinical features associated with this condition.
心脏受累在约 80%的肌强直性营养不良 1 型（DM1）患者中被记录。心脏传导异常的患病率已有详细描述。关于心房颤动（AF）的患病率的数据仍存在争议。本综述的主要目的是评估 DM1 患者中 AF 的患病率。次要目的是检查与 AF 相关的临床特征，以发现易患因素和/或影响预后的因素。在 MEDLINE、EMBASE、Cochrane 对照试验注册库和 Web of Science 数据库中进行了系统检索，以确定 2002 年 1 月 1 日至 2020 年 1 月 30 日期间评估 DM1 人群中 AF 患病率的原始报告。纳入了描述通过定期心电图（ECG）和/或 24 小时 ECG 动态监测、外部环路记录（ELR）和植入式设备检查评估 DM1 患者心房颤动患病率的回顾性/前瞻性队列研究和病例系列研究。排除了病例报告、简单综述、评论和社论。符合入选标准的有 13 项研究报告，并纳入了我们的系统综述。根据所有评估研究的结果，在 3677 例 DM1 患者中，10.9%（n=404）的 DM1 患者患有 AF。男性、传导障碍、超声心动图显示心房机电延迟延长，似乎与心房颤动密切相关，是其发病的有利因素。发生 AF 的 DM1 患者似乎有更高的心血管和非心血管死亡风险。需要进一步研究来评估 DM1 患者中 AF 的患病率，并研究与该疾病相关的 ECG 异常和其他临床特征。
