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      Cardiac device implantation in Fabry disease : A retrospective monocentric study

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          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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          Most cited references7

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          American College of Cardiology/European Society of Cardiology Clinical Expert Consensus Document on Hypertrophic Cardiomyopathy. A report of the American College of Cardiology Foundation Task Force on Clinical Expert Consensus Documents and the European Society of Cardiology Committee for Practice Guidelines.

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            Arrhythmias in Fabry cardiomyopathy.

            Prior studies suggest that the incidence of ventricular arrhythmias is high in patients with Fabry cardiomyopathy. This study evaluated the incidence of significant arrhythmias in a series of patients with Fabry cardiomyopathy. Arrhythmias are important causes of morbidity and mortality in Fabry Cardiomyopathy. This study was a retrospective chart review of 19 patients with known Fabry cardiomyopathy. Device interrogation reports were reviewed for those who had implantable devices. Electrocardiogram, Holter monitor, and event monitors were reviewed in those who did not have implantable devices. Eighteen of nineteen patients were on enzyme replacement therapy (ERT). Nine (47%) out of 19 patients had implantable devices. Implant indications included symptomatic bradycardia, nonsustained ventricular tachycardia, conduction abnormalities, palpitations, and syncope. Mean follow-up in the patients with devices was 50 ± 23 months. Two patients received implantable cardioverter-defibrillator (ICD) shocks, 1 of which was inappropriate for atrial fibrillation. Patients were paced in the atrium 71% ± 37% of the time and paced in the ventricle 49% ± 52% of the time. Four patients with devices were paced more than 95% of the time. Patients with an ICD had lower heart rates prior to ICD implant than the group that did not have devices (60 ± 10 vs 78 ± 16, P = 0.03). Of the patients without devices, only 1 had sudden cardiac death. Patients with implanted devices had higher left ventricular (LV) mass indices compared to patients without implanted devices (136 ± 40 g/m(2) vs 93 ± 19 g/m(2), P = 0.008). Significant ventricular arrhythmias are uncommon in patients with Fabry cardiomyopathy on ERT, but utilization of pacing is high. Sudden cardiac death in Fabry cardiomyopathy may be related to bradycardia. © 2012 Wiley Periodicals, Inc.
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              Enzyme replacement therapy improves cardiac features and severity of Fabry disease.

              Although left ventricular hypertrophy (LVH) in Fabry disease (FD) can improve with enzyme-replacement therapy (ERT), the response is difficult to predict. Furthermore, the response of other cardiac features such as aortic dilatation and ECG changes are poorly understood.
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                Author and article information

                Journal
                Medicine (Baltimore)
                Medicine (Baltimore)
                MEDI
                Medicine
                Wolters Kluwer Health
                0025-7974
                1536-5964
                October 2016
                07 October 2016
                : 95
                : 40
                : e4996
                Affiliations
                [a ]Department of Internal Medicine and Rheumatology, Reference Center for Lysosomal Storage Disorders (CRML, site Avron), Groupe Hospitalier Diaconesses Croix-Saint-Simon
                [b ]Inserm UMRS 974, Université Pierre & Marie Curie
                [c ]Department of Cardiology, Institut Mutualiste Montsouris
                [d ]Department of Clinical Research, Fondation Ophtalmologique Rothschild, Paris
                [e ]Department of Internal Medicine, Centre Hospitalier Jacques Coeur, Bourges
                [f ]Department of Internal Medicine, Hôpital Saint-Antoine, AP-HP, Université Pierre & Marie Curie, Paris
                [g ]Department of Internal Medicine, Centre Hospitalier de Valence, Valence
                [h ]Department of Nephrology, Hôpital Necker, AP-HP, Université René Descartes, Paris
                [i ]Referral Center For Cardiac Hereditary Diseases, Hôpital Pitié-Salpêtrière, AP-HP, Université Versailles-Saint-Quentin, Saint-Quentin-en-Yvelines, France.
                Author notes
                []Correspondence: Olivier Lidove, Service de Médecine Interne-Rhumatologie, Hôpital de la Croix Saint-Simon, 125, rue d’Avron, 75020 Paris, France (e-mail: olidove@ 123456hopital-dcss.org ).
                Article
                04996
                10.1097/MD.0000000000004996
                5059061
                27749559
                a7f042d9-57f9-452d-aead-2aa4866c5d60
                Copyright © 2016 the Author(s). Published by Wolters Kluwer Health, Inc. All rights reserved.

                This is an open access article distributed under the Creative Commons Attribution-ShareAlike License 4.0, which allows others to remix, tweak, and build upon the work, even for commercial purposes, as long as the author is credited and the new creations are licensed under the identical terms. http://creativecommons.org/licenses/by-sa/4.0

                History
                : 6 April 2016
                : 6 September 2016
                : 6 September 2016
                Categories
                3500
                Research Article
                Observational Study
                Custom metadata
                TRUE

                arrhythmia,cardiac device,conduction abnormalities,enzyme replacement therapy,fabry disease (omim 301500),internal cardioverter-defibrillator,pacemaker

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