Long-term arrhythmia follow-up of patients with myotonic dystrophy.
J Cardiovasc Electrophysiol
; 26(3): 305-10, 2015 Mar.
Article
en En
| MEDLINE
| ID: mdl-25546341
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.Palabras clave
Texto completo:
1
Base de datos:
MEDLINE
Asunto principal:
Arritmias Cardíacas
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Muerte Súbita Cardíaca
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Desfibriladores Implantables
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Distrofia Miotónica
Tipo de estudio:
Observational_studies
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Prognostic_studies
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Risk_factors_studies
Límite:
Adult
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Aged
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Female
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Humans
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Male
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Middle aged
Idioma:
En
Revista:
J Cardiovasc Electrophysiol
Asunto de la revista:
ANGIOLOGIA
/
CARDIOLOGIA
/
FISIOLOGIA
Año:
2015
Tipo del documento:
Article
País de afiliación:
Estados Unidos