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The WATCHMAN Left Atrial Appendage Closure Device for Atrial Fibrillation
Published on: February 28, 2012
Cardiac device implantation in Fabry disease: A retrospective monocentric study
Thomas Sené1, Olivier Lidove, Joel Sebbah
1Department of Internal Medicine and Rheumatology, Reference Center for Lysosomal Storage Disorders (CRML, site Avron), Groupe Hospitalier Diaconesses Croix-Saint-Simon Inserm UMRS 974, Université Pierre & Marie Curie Department of Cardiology, Institut Mutualiste Montsouris Department of Clinical Research, Fondation Ophtalmologique Rothschild, Paris Department of Internal Medicine, Centre Hospitalier Jacques Coeur, Bourges Department of Internal Medicine, Hôpital Saint-Antoine, AP-HP, Université Pierre & Marie Curie, Paris Department of Internal Medicine, Centre Hospitalier de Valence, Valence Department of Nephrology, Hôpital Necker, AP-HP, Université René Descartes, Paris Referral Center For Cardiac Hereditary Diseases, Hôpital Pitié-Salpêtrière, AP-HP, Université Versailles-Saint-Quentin, Saint-Quentin-en-Yvelines, France.
Insights
Arrhythmias and/or conduction abnormalities requiring cardiac device implantation occur in 18% of Fabry disease patients. Delayed diagnosis and older age at follow-up are key risk factors for these cardiac events.
Area of Science:
- Cardiology
- Genetics
- Internal Medicine
Background:
- Fabry disease (FD) is a rare genetic disorder.
- Arrhythmias and/or conduction abnormalities (ACAs) requiring cardiac device (CD) implantation are poorly understood in FD.
- Predictive factors for ACAs in FD are not well-defined.
Purpose of the Study:
- To determine the prevalence, incidence, and factors associated with ACAs requiring CD implantation in a cohort of FD patients.
- To identify risk factors for cardiac device implantation in Fabry disease.
- To assess the need for regular cardiac monitoring in FD patients.
Main Methods:
- Retrospective monocentric study of 49 confirmed Fabry disease patients.
- Analysis of cardiac device implantation events and associated clinical factors.
- Univariate and multivariate analyses to identify predictive factors for ACAs.
Main Results:
- 18% of patients (9/49) experienced ACAs requiring device therapy.
- Annual incidence of CD implantation was 1.90 per 100 person-years.
- Delayed FD diagnosis, delayed enzyme replacement therapy, older age at follow-up, and severe multiorgan phenotype were associated with ACAs. Age at diagnosis and last follow-up were independent predictors.
Conclusions:
- Regular cardiac monitoring is crucial for Fabry disease patients, especially those with late diagnosis or severe phenotypes.
- Early diagnosis and timely intervention are essential to mitigate cardiac risks in FD.
- Holter ECGs, patient education, and emergency cards are vital components of FD patient care.
Abstract:
The incidence and predictive factors of arrhythmias and/or conduction abnormalities (ACAs) requiring cardiac device (CD) implantation are poorly characterized in Fabry disease (FD). The aim of our retrospective study was to determine the prevalence, incidence, and factors associated with ACA requiring CD implantation in a monocentric cohort of patients with confirmed FD who were followed up in a department of internal medicine and reference center for FD.Forty-nine patients (20M, 29F) were included. Nine patients (4M, 5F; 18%) had at least one episode of ACA leading to device therapy. Six patients (4M/2F) required a pacemaker (PM) for sinus node dysfunction (n = 4) or atrioventricular disease (n = 2). One female patient required an internal cardioverter-defibrillator (ICD) to prevent sudden cardiac death because of nonsustained ventricular tachycardia (nSVT). One female patient required PM-ICD for sinus node dysfunction and nSVT. One patient underwent CD implantation before the diagnosis of FD. The annual rate of CD implantation was estimated at 1.90 per 100 person years. On univariate analysis at the end of the follow-up period, the factors associated with ACAs requiring CD implantation were as follows: delayed diagnosis of FD, delayed initiation of enzyme replacement therapy, age at the last follow-up visit, and severe multiorgan phenotype (hypertrophic cardiomyopathy, chronic kidney disease, and/or sensorineural hearing loss). On multivariate analysis, age at diagnosis of FD and age at the last follow-up visit were independently associated with an increased risk of ACAs requiring CD (P < 0.05).Considering the high frequency of ACAs requiring CD implantation and the risk of sudden death in patients with FD, regular monitoring is mandatory, especially in patients with a late diagnosis of FD and/or with a severe phenotype. Regular Holter ECGs, therapeutic education of patients, and deliverance of an emergency card including a phenotype summary are crucial in the care of FD patients.Available guidelines for device therapy and the efficacy of enzyme replacement therapy for arrhythmias or conduction abnormalities are discussed.
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