Atrial Fibrillation and Flutter in a Contemporary Cohort of Patients With Myotonic Muscular Dystrophy.

IF 8 1区医学 Q1 CARDIAC & CARDIOVASCULAR SYSTEMS

JACC. Clinical electrophysiology Pub Date : 2025-01-15 DOI:10.1016/j.jacep.2024.12.007

Melissa A Austin, Gregory E Supple, Samuel Carrell, Colin Quinn, Lauren Elman, Saman Nazarian

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Abstract

Background: Literature on the prevalence and management of atrial arrhythmias in patients with myotonic muscular dystrophy type 1 (MMD1) or myotonic muscular dystrophy type 2 (MMD2) is limited.

Objectives: This study sought to describe incidence, prevalence, and predictors of atrial fibrillation (AF) and atrial flutter (AFL) in a contemporary cohort of patients with myotonic muscular dystrophy (MMD).

Methods: Associations between patient factors and incident AF/AFL were analyzed in patients with MMD referred for routine electrophysiology evaluation between January 2013 and September 2023.

Results: We identified 120 patients (96 MMD1, 24 MMD2) seen for new electrophysiology clinic visits for MMD cardiac evaluation. Median age at MMD diagnosis was 34.6 years (Q1-Q3: 21.8-51.0 years), with a younger age at diagnosis for MMD1 patients (P < 0.001). AF or AFL was diagnosed in 31% of patients (31 of 96 MMD1 patients vs 6 of 24 MMD2 patients; P = 0.656) during the study period. AF occurred in 34 (28%) patients, AFL in 9 (8%), and 6 of 37 had both AF and AFL. Anticoagulation (AC) was prescribed at initial AF/AFL diagnosis in 41% (n = 15 of 37) of patients. The CHA₂DS₂-VASc (congestive heart failure, hypertension, age ≥75 years, diabetes mellitus, prior stroke or transient ischemic attack or thromboembolism, vascular disease, age 65-74 years, sex category) score was higher in patients who received AC (P < 0.001). Twelve (32%) patients underwent catheter ablation. One stroke occurred in the AF/AFL group (no AC, CHA₂DS₂-VASc score 0). Female MMD1 patients were less likely to develop AF/AFL (HR: 0.08). Diagnosis of MMD1 at a younger age (HR: 0.95 per year) and greater CTG nucleotide repeats (HR: 1.09 per 50 repeats) were associated with incident AF/AFL.

Conclusions: There was a high prevalence of AF/AFL in this single-institution MMD cohort. The CHA₂DS₂-VASc score may not be an effective tool in this population.

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来源期刊

JACC. Clinical electrophysiology CARDIAC & CARDIOVASCULAR SYSTEMS-

CiteScore

10.30

自引率

5.70%

发文量

250

期刊介绍： JACC: Clinical Electrophysiology is one of a family of specialist journals launched by the renowned Journal of the American College of Cardiology (JACC). It encompasses all aspects of the epidemiology, pathogenesis, diagnosis and treatment of cardiac arrhythmias. Submissions of original research and state-of-the-art reviews from cardiology, cardiovascular surgery, neurology, outcomes research, and related fields are encouraged. Experimental and preclinical work that directly relates to diagnostic or therapeutic interventions are also encouraged. In general, case reports will not be considered for publication.