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J Cardiovasc Electrophysiol. 2015 Mar;26(3):305-10. doi: 10.1111/jce.12604. Epub 2015 Feb 11.

Long-term arrhythmia follow-up of patients with myotonic dystrophy.

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1
Electrophysiology Section, Cardiovascular Division, Hospital of the University of Pennsylvania, Philadelphia, Pennsylvania, USA.

Abstract

BACKGROUND:

Myotonic dystrophy (MD) is the most common muscular dystrophy in adults and is associated with sudden death. Reported predictors of sudden death in this population include atrial tachyarrhythmias, a PR interval greater than 240 milliseconds, aberrant QRS conduction, and any degree of AV block.

OBJECTIVE:

We sought to report on the arrhythmic outcome of a cohort of patients with a new diagnosis of genetically proven MD.

METHODS:

We performed a retrospective review of 37 patients with genetically confirmed MD referred to our electrophysiology clinic for primary cardiac screening.

RESULTS:

There were 25 patients with MD type 1 (MD1) and 12 patients with MD type 2 (MD2). Eight patients with MD1 (32%) had atrial fibrillation, compared to only one patient with MD2 (8.3%). Patients with MD1 were more likely to have evidence of conduction disease abnormalities (40% vs. 8.3%, P = ns) and had a higher all-cause mortality (16% vs. 0%) than those with MD2. Criteria for recommending ICD implantation were based on sudden death risk factors suggested by published literature. Eleven patients were offered an ICD, 2 refused and died within the next year. Of the 9 patients who received an ICD, 8 had MD1. Three patients received appropriate shocks, 2 for monomorphic VT, and one for polymorphic VT.

CONCLUSION:

The presence of AV conduction disturbance in MD patients is associated with a greater risk for ventricular arrhythmias. MD1 was more likely to be associated with cardiac arrhythmias than MD2. The incidence of ventricular arrhythmias among those who received a primary prevention ICD was 33% over 22 months, with 2 patients experiencing monomorphic VT and one experiencing polymorphic VT.

KEYWORDS:

implantable cardioverter defibrillator; myopathy; myotonic dystrophy; sudden death; ventricular fibrillation; ventricular tachycardia

PMID:
25546341
DOI:
10.1111/jce.12604
[Indexed for MEDLINE]
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