scholarly journals Study of indications for cardiac device implantation and utilisation in Fabry cardiomyopathy

Heart ◽  
2019 ◽  
Vol 105 (23) ◽  
pp. 1825-1831 ◽  
Author(s):  
Ravi Vijapurapu ◽  
Tarekegn Geberhiwot ◽  
Ana Jovanovic ◽  
Shanat Baig ◽  
Sabrina Nordin ◽  
...  

BackgroundFabry disease is a treatable X-linked condition leading to progressive cardiomyopathy, arrhythmia and premature death. Atrial and ventricular arrhythmias contribute significantly to adverse prognosis; however, guidance to determine which patients require cardiovascular implantable electronic devices (CIEDs) is sparse. We aimed to evaluate indications for implantation practice in the UK and quantify device utilisation.MethodsIn this retrospective study, we included demographic, clinical and imaging data from patients in four of the largest UK Fabry centres. Ninety patients with Fabry disease were identified with CIEDs implanted between June 2001 and February 2018 (FD-CIED group). To investigate differences in clinical and imaging markers between those with and without devices, these patients were compared with 276 patients without a CIED (FD-control).ResultsIn the FD-CIED group, 92% of patients with permanent pacemakers but only 28% with implantable cardioverter-defibrillators had a class 1 indication for implantation. A further 44% of patients had defibrillators inserted for primary prevention outside of current guidance. The burden of arrhythmia requiring treatment in the FD-CIED group was high (asymptomatic atrial fibrillation:29%; non-sustained ventricular tachycardia requiring medical therapy alone: 26%; sustained ventricular tachycardia needing anti-tachycardia pacing/defibrillation: 28%). Those with devices were older, had greater LV mass, more scar tissue and larger atrial size.ConclusionsArrhythmias are common in Fabry patients. Those with cardiac devices had high rates of atrial fibrillation requiring anticoagulation and ventricular arrhythmia needing device treatment. These are as high as those in hypertrophic cardiomyopathy, supporting the need for Fabry-specific indications for device implantation.

2019 ◽  
Vol 8 (1) ◽  
pp. 60-65
Author(s):  
Luiz Wellington Pinto ◽  
Silvia Veloso Gandra ◽  
Matheus de Carvalho Alves ◽  
Isabel Gomes ◽  
Eduardo Back Sternick

Abstract Current guidelines do not recommend bioelectrical impedance analysis (BIA) in patients with implanted cardiac devices. There is no data on the influence of such devices over the parameters assessed by BIA. We aimed to assess the influence of cardiac devices on the parameters assessed by BIA as well as to evaluate the likelihood of electromagnetic interference of BIA in patients with implanted cardiac devices. Sixty-two consecutive patients over 18 years of age who underwent single (PM) or multisite (CRT) pacemaker or defibrillator (ICD) implantation were included. Body composition assessment was done using a single frequency device, on both right and left sides, before and after cardiac device implantation. During BIA analysis after device implantation, we did real-time telemetry to assess electromagnetic interference. Patients were 67+14 years old and 51.6% male. PM was implanted in 52 patients (83.9%), ICD in 7 (11.3%), ICD with CRT in 2 (3.2%) and CRT in 1 (1.6%). During real-time telemetry, there was no electromagnetic interference including interruption of telemetry. Default device programming did not change after BIA assessment. After surgery, resistance and fat mass were smaller, while cellular mass, fat-free mass, metabolic rate and total body water/ body weight increased, on right and left sides measurements. We concluded that decreased resistance and related parameters after device implantation were probably influenced to a change in hydration status, regardless of the implanted device. Bioimpedance analysis is safe in patients with an implanted cardiac device.


2019 ◽  
Vol 8 (2) ◽  
pp. 138-142 ◽  
Author(s):  
Mark Elliott ◽  
Sheikh Momin ◽  
Barnaby Fiddes ◽  
Fahad Farooqi ◽  
SM Afzal Sohaib

The need for cardiac device implantation in patients receiving deep brain stimulation (DBS) is increasing. Despite the theoretical risk of the two systems interacting, there are no clear guidelines for cardiologists carrying out cardiac device implantation in this population. We performed a review of the literature and describe 13 case reports in which patients have both DBS and a cardiac pacemaker or ICD implanted. Except for one early study, in which an ICD shock reset the deep brain stimulator, no significant interactions have been reported. We discuss the potential interactions between DBS and cardiac devices, and provide practical advice for implanting cardiologists. We conclude that, provided that specific precautions are taken, cardiac device implantation is likely to be safe in patients with DBS.


Author(s):  
Kim Rajappan

The term ‘device therapy’ is used in cardiology to refer to three different types of implantable cardiac-rhythm-management devices: pacemakers, implantable cardioverter defibrillators (also known as ICDs), and cardiac resynchronization therapy devices (also known as CRT devices). There has been a steady increase in the number of patients receiving these cardiac devices; in relation to CRT devices, the increase has been almost exponential.


Author(s):  
Agnieszka Smoczyńska ◽  
Vera Loen ◽  
David J. Sprenkeler ◽  
Anton E. Tuinenburg ◽  
Henk J. Ritsema van Eck ◽  
...  

Background Short‐term variability of the QT interval (STV QT ) has been proposed as a novel electrophysiological marker for the prediction of imminent ventricular arrhythmias in animal models. Our aim is to study whether STV QT can predict imminent ventricular arrhythmias in patients. Methods and Results In 2331 patients with primary prophylactic implantable cardioverter defibrillators, 24‐hour ECG Holter recordings were obtained as part of the EU‐CERT‐ICD (European Comparative Effectiveness Research to Assess the Use of Primary Prophylactic Implantable Cardioverter Defibrillators) study. ECG Holter recordings showing ventricular arrhythmias of >4 consecutive complexes were selected for the arrhythmic groups (n=170), whereas a control group was randomly selected from the remaining Holter recordings (n=37). STV QT was determined from 31 beats with fiducial segment averaging and calculated as , where D n represents the QT interval. STV QT was determined before the ventricular arrhythmia or 8:00  am in the control group and between 1:30 and 4:30  am as baseline. STV QT at baseline was 0.84±0.47 ms and increased to 1.18±0.74 ms ( P <0.05) before the ventricular arrhythmia, whereas the STV QT in the control group remained unchanged. The arrhythmic patients were divided into three groups based on the severity of the arrhythmia: (1) nonsustained ventricular arrhythmia (n=32), (2) nonsustained ventricular tachycardia (n=134), (3) sustained ventricular tachycardia (n=4). STV QT increased before nonsustained ventricular arrhythmia, nonsustained ventricular tachycardia, and sustained ventricular tachycardia from 0.80±0.43 ms to 1.18±0.78 ms ( P <0.05), from 0.90±0.49 ms to 1.14±0.70 ms ( P <0.05), and from 1.05±0.22 ms to 2.33±1.25 ms ( P <0.05). This rise in STV QT was significantly higher in sustained ventricular tachycardia compared with nonsustained ventricular arrhythmia (+1.28±1.05 ms versus +0.24±0.57 ms [ P <0.05]) and compared with nonsustained ventricular arrhythmia (+0.34±0.87 ms [ P <0.05]). Conclusions STV QT increases before imminent ventricular arrhythmias in patients, and the extent of the increase is associated with the severity of the ventricular arrhythmia.


2021 ◽  
Vol 10 (8) ◽  
pp. 1618
Author(s):  
Andrea Matteucci ◽  
Michela Bonanni ◽  
Marco Centioni ◽  
Federico Zanin ◽  
Francesco Geuna ◽  
...  

Background: The in-hospital management of patients with cardiac implantable electronic devices (CIEDs) changed early in the COVID-19 pandemic. Routine in-hospital controls of CIEDs were converted into remote home monitoring (HM). The aim of our study was to investigate the impact of the lockdown period on CIEDs patients and its influence on in-hospital admissions through the analysis of HM data. Methods: We analysed data recorded from 312 patients with HM during the national quarantine related to COVID-19 and then compared data from the same period of 2019. Results: We observed a reduction in the number of HM events in 2020 when compared to 2019. Non-sustained ventricular tachycardia episodes decreased (18.3% vs. 9.9% p = 0.002) as well as atrial fibrillation episodes (29.2% vs. 22.4% p = 0.019). In contrast, heart failure (HF) alarm activation was lower in 2019 than in 2020 (17% vs. 25.3% p = 0.012). Hospital admissions for critical events recorded with CIEDs dropped in 2020, including those for HF. Conclusions: HM, combined with telemedicine use, has ensured the surveillance of CIED patients. In 2020, arrhythmic events and hospital admissions decreased significantly compared to 2019. Moreover, in 2020, patients with HF arrived in hospital in a worse clinical condition compared to previous months.


Heart Asia ◽  
2019 ◽  
Vol 11 (2) ◽  
pp. e011166
Author(s):  
Takashi Nakagawa ◽  
Hisao Hara ◽  
Masaya Yamamoto ◽  
Yumi Matsushita ◽  
Yukio Hiroi

ObjectiveParoxysmal atrial fibrillation could progress to permanent atrial fibrillation. Whether the transmitral inflow waves could be used to predict progression from paroxysmal atrial fibrillation to permanent atrial fibrillation is unknown. Therefore, we investigated the association between the transmitral inflow waves and progression of paroxysmal atrial fibrillation.MethodWe performed a retrospective study by analysing clinical and echocardiographic data from 88 patients with paroxysmal atrial fibrillation. We excluded patients who had structural heart disease, significant valvular disease, cardiomyopathy, cardiac device implantation or a left ventricular ejection fraction <50%.ResultThe patients with progression to permanent atrial fibrillation were more likely to be male and had lower peak A velocity than those without progression. After adjusting for covariates, lower peak A velocity remained the independent predictor of progression to permanent atrial fibrillation (p=0.025).ConclusionThe A velocity could be useful for predicting progression to permanent atrial fibrillation in Asian people.


2020 ◽  
Vol 81 (10) ◽  
pp. 92-103
Author(s):  
Adam SC Dennis ◽  
Jonathan M Behar

This article provides an overview of current cardiac device management, complications, and future areas for development. The last 70 years have seen huge advances in the field of implantable cardiac devices, from diagnostic tools to electrical therapies for bradycardia, ventricular arrhythmia and cardiac resynchronisation. While out-of-hours specialist cardiology cover and regional arrhythmia pathways are increasingly established, they are not universal, and the management of arrhythmia remains an important facet of clinical medicine for the general physician. This article discusses core recommendations from international guidelines with respect to heart rhythm diagnostics, pacing for bradycardia, cardiac resynchronisation and implantable cardioverter defibrillators, along with common complications. Finally, future innovations such as the diagnostic potential of portable technologies, antibiotic envelopes for cardiac devices and the increasing use of leadless pacemakers are described.


2022 ◽  
Vol 17 (1) ◽  
Author(s):  
Ravi Vijapurapu ◽  
William Bradlow ◽  
Francisco Leyva ◽  
James C. Moon ◽  
Abbasin Zegard ◽  
...  

Abstract Background Fabry disease (FD) is a treatable X-linked condition leading to progressive cardiac disease, arrhythmia and premature death. We aimed to increase awareness of the arrhythmogenicity of Fabry cardiomyopathy, by comparing device usage in patients with Fabry cardiomyopathy and sarcomeric HCM. All Fabry patients with an implantable cardioverter defibrillator (ICD) implanted in the UK over a 17 year period were included. A comparator group of HCM patients, with primary prevention ICD implantation, were captured from a regional registry database. Results Indications for ICD in FD varied with 72% implanted for primary prevention based on multiple potential risk factors. In FD and HCM primary prevention devices, arrhythmia occurred more frequently in FD over shorter follow-up (HR 4.2, p < 0.001). VT requiring therapy was more common in FD (HR 4.5, p = 0.002). Immediate shock therapy for sustained VT was also more common (HR 2.5, p < 0.001). There was a greater burden of AF needing anticoagulation and NSVT in FD (AF: HR 6.2, p = 0.004, NSVT: HR 3.1, p < 0.001). Conclusion This study demonstrates arrhythmia burden and ICD usage in FD is high, suggesting that Fabry cardiomyopathy may be more ‘arrhythmogenic’ than previously thought. Existing risk models cannot be mutually applicable and further research is needed to provide clarity in managing Fabry patients with cardiac involvement.


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