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Arrhythmias and fasciculoventricular pathways in patients with Danon disease: A single center experience.
Jhaveri, Simone; Herber, Joshua; Zahka, Kenneth; Boyle, Gerard J; Saarel, Elizabeth V; Aziz, Peter F.
Afiliación
  • Jhaveri S; Department of Pediatric Cardiology, Cleveland Clinic Children's, Cleveland, Ohio.
  • Herber J; Department of Pediatric Cardiology, Cleveland Clinic Children's, Cleveland, Ohio.
  • Zahka K; Department of Pediatric Cardiology, Cleveland Clinic Children's, Cleveland, Ohio.
  • Boyle GJ; Department of Pediatric Cardiology, Cleveland Clinic Children's, Cleveland, Ohio.
  • Saarel EV; Department of Pediatric Cardiology, Cleveland Clinic Children's, Cleveland, Ohio.
  • Aziz PF; Department of Pediatric Cardiology, Cleveland Clinic Children's, Cleveland, Ohio.
J Cardiovasc Electrophysiol ; 30(10): 1932-1938, 2019 10.
Article en En | MEDLINE | ID: mdl-31240821
ABSTRACT

BACKGROUND:

Danon disease is a rare X-linked storage disorder characterized by hypertrophic cardiomyopathy leading to arrhythmias and heart failure. A preexcitation pattern on electrocardiogram (ECG) has been described in these patients, however, invasive studies to distinguish between Wolff-Parkinson-White syndrome syndrome and fasciculoventricular pathways (FVP) are limited.

OBJECTIVES:

The purpose of this study was to delineate the electrophysiological cardiac abnormalities in patients with Danon disease and to describe the presence of FVP in this population.

METHODS:

We performed a retrospective study of all patients with a confirmed diagnosis of Danon disease presenting to a single center from May 2005 to May 2018. Baseline demographics, clinical characteristics, ECG findings, and electrophysiology study (EPS) results were collected.

RESULTS:

Ten patients with Danon disease (30% male, average age 17.4 years) were identified. Seven patients (70%) had tachyarrhythmias including five with atrial arrhythmias and six with nonsustained ventricular tachycardia. Preexcitation pattern on ECG was found in four (40%) patients. Of these, two underwent an EPS which confirmed the presence of an FVP. One patient underwent an adenosine challenge which supported a FVP. Implantable cardioverter defibrillator was placed in five patients for primary prevention with no patients receiving an appropriate discharge. Over a follow-up of 5.3 years, five underwent heart transplantation.

CONCLUSIONS:

This study reports a high incidence of FVP in patients with Danon disease and preexcitation. It underscores an alternate etiology of preexcitation in this population which can potentially be diagnosed without invasive EPS testing. Future multicenter studies are needed to expand this experience.
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Texto completo: 1 Bases de datos: MEDLINE Asunto principal: Arritmias Cardíacas / Cardiomiopatía Hipertrófica / Enfermedad por Depósito de Glucógeno de Tipo IIb / Fascículo Atrioventricular Accesorio Tipo de estudio: Diagnostic_studies / Etiology_studies / Observational_studies / Prognostic_studies / Risk_factors_studies País/Región como asunto: America do norte Idioma: En Revista: J Cardiovasc Electrophysiol Año: 2019 Tipo del documento: Article

Texto completo: 1 Bases de datos: MEDLINE Asunto principal: Arritmias Cardíacas / Cardiomiopatía Hipertrófica / Enfermedad por Depósito de Glucógeno de Tipo IIb / Fascículo Atrioventricular Accesorio Tipo de estudio: Diagnostic_studies / Etiology_studies / Observational_studies / Prognostic_studies / Risk_factors_studies País/Región como asunto: America do norte Idioma: En Revista: J Cardiovasc Electrophysiol Año: 2019 Tipo del documento: Article