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Cardiac device implantation in Fabry disease: A retrospective monocentric study.

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?

SUBMITTER: Sene T

PROVIDER: S-EPMC5059061 | biostudies-literature | 2016 Oct

REPOSITORIES: biostudies-literature

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Cardiac device implantation in Fabry disease: A retrospective monocentric study.

Sené Thomas T Lidove Olivier O Sebbah Joel J Darondel Jean-Marc JM Picard Hervé H Aaron Laurent L Fain Olivier O Zenone Thierry T Joly Dominique D Charron Philippe P Ziza Jean-Marc JM

Medicine 20161001 40

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 inclu ...[more]

PMID: 27749559

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Project description:The use of temporary cardiac pacing is frequent in critical care units for severe bradycardia or electrical storm, but may be associated with frequent and potentially severe complications, especially when indwelling for several days. In some cases, transient indication or ongoing contraindication for a permanent pacemaker justifies prolonged temporary pacing. In that case, the implantation of an active-fixation lead connected to an externalized pacemaker represents a valuable option to increase safety and patient comfort. Yet, evidence remains scarce. We aimed to describe the population receiving prolonged temporary cardiac pacing (PTCP) and their outcomes. We retrospectively included all consecutive patients, admitted to our hospital from 2016 to 2021, who underwent PTCP. We collected in-hospital and six-month outcomes. Forty-six patients (median age of 73, 63% male) were included, and twenty-nine (63%) had prior heart disease. Indications for PTCP were found: seventeen (37%) potentially reversible high-grade conduction disorders, fourteen (30%) indications for permanent pacemaker but ongoing infection, seven (15%) cardiac implantable electronic device infections requiring extraction in pacing-dependent patients, seven (15%) severe vagal hyperreactivity in prolonged critical care hospitalizations, and one (2%) recurrent sustained ventricular tachycardia requiring overdrive pacing. The median PTCP duration was nine (5-13) days. Ten (22%) patients exhibited at least one complication during hospitalization. Twenty-six (56.5%) patients required definite device implantation (twenty-five pacemakers and one cardioverter-defibrillator) and twenty (43.5%) did not (fifteen PTCP device removal for recovery and five deaths under PTCP). At six months, two (5%) deaths and two (5%) new infections of a definite implanted device occurred, all in patients with initial active infection. The use of prolonged temporary cardiac pacing, with an active -fixation lead connected to an externalized pacemaker, is possible and reasonable; this would allow for the possible recovery or resolution of contraindication for definite device implantation.

Dataset Information

Cardiac device implantation in Fabry disease: A retrospective monocentric study.

Publications

Cardiac device implantation in Fabry disease: A retrospective monocentric study.

Similar Datasets

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