Timing of pacemaker and ICD implantation in LMNA mutation carriers

Aims LMNA-cardiomyopathy is often associated with pathology in the cardiac conduction system necessitating device implantations. The aim was to study the timing and types of device implantations and need for re-implantations in LMNA mutation carriers.Methods We studied the hospital records of 60 LMN...

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Main Authors: Kjell Nikus, Laura Helena Ollila, Hannu Parikka, Sini Weckström, Heliö Tiina
Format: Article
Language:English
Published: BMJ Publishing Group 2021-02-01
Series:Open Heart
Online Access:https://openheart.bmj.com/content/8/1/e001622.full
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author Kjell Nikus
Laura Helena Ollila
Hannu Parikka
Sini Weckström
Heliö Tiina
author_facet Kjell Nikus
Laura Helena Ollila
Hannu Parikka
Sini Weckström
Heliö Tiina
author_sort Kjell Nikus
collection DOAJ
description Aims LMNA-cardiomyopathy is often associated with pathology in the cardiac conduction system necessitating device implantations. The aim was to study the timing and types of device implantations and need for re-implantations in LMNA mutation carriers.Methods We studied the hospital records of 60 LMNA mutation carriers concerning device implantations and re-implantations and their indications. Data were collected until April 2019.Results The median follow-up time from the first ECG recording to the last clinical follow-up, transplantation, or death was 7.7 (IQR=9.1) years. Altogether 61.7% (n=37) of the LMNA mutation carriers received a pacemaker or an implantable cardioverter defibrillator (ICD), and of them 27.0% (n=10) needed a device upgrade. Notably, in some patients the upgrade took place very soon after the first implantation. The first device was implanted at an average age of 47.9 years (SD=9.5), whereas the upgrade took place at an average age of 50.3 years (SD=8.1). Most upgrades were ICD implantations. Male patients underwent device upgrade more often and at a younger age than women. By the end of follow-up, 35.0% (n=21) of the patients fulfilled echocardiographic criteria for dilated cardiomyopathy, and 90.5% of them (n=19) needed pacemaker implantation.Conclusion Most LMNA mutation carriers underwent pacemaker implantation in this study. Due to the progressive nature of LMNA-cardiomyopathy, device upgrades are quite common. An ICD should be considered when the initial device implantation is planned in an LMNA mutation carrier.
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spelling doaj-art-0768e3cba03a45619a3cd9869d900dff2024-11-12T02:00:07ZengBMJ Publishing GroupOpen Heart2053-36242021-02-018110.1136/openhrt-2021-001622Timing of pacemaker and ICD implantation in LMNA mutation carriersKjell Nikus0Laura Helena Ollila1Hannu Parikka2Sini Weckström3Heliö Tiina4Heart Centre, Tampere University Hospital, Tampere, FinlandHeart and Lung Centre, Helsinki University Central Hospital, Helsinki, FinlandHeart and Lung Centre, Helsinki University Central Hospital, Helsinki, FinlandHeart and Lung Centre, Helsinki University Central Hospital, Helsinki, FinlandHeart and Lung Centre, Helsinki University Central Hospital, Helsinki, FinlandAims LMNA-cardiomyopathy is often associated with pathology in the cardiac conduction system necessitating device implantations. The aim was to study the timing and types of device implantations and need for re-implantations in LMNA mutation carriers.Methods We studied the hospital records of 60 LMNA mutation carriers concerning device implantations and re-implantations and their indications. Data were collected until April 2019.Results The median follow-up time from the first ECG recording to the last clinical follow-up, transplantation, or death was 7.7 (IQR=9.1) years. Altogether 61.7% (n=37) of the LMNA mutation carriers received a pacemaker or an implantable cardioverter defibrillator (ICD), and of them 27.0% (n=10) needed a device upgrade. Notably, in some patients the upgrade took place very soon after the first implantation. The first device was implanted at an average age of 47.9 years (SD=9.5), whereas the upgrade took place at an average age of 50.3 years (SD=8.1). Most upgrades were ICD implantations. Male patients underwent device upgrade more often and at a younger age than women. By the end of follow-up, 35.0% (n=21) of the patients fulfilled echocardiographic criteria for dilated cardiomyopathy, and 90.5% of them (n=19) needed pacemaker implantation.Conclusion Most LMNA mutation carriers underwent pacemaker implantation in this study. Due to the progressive nature of LMNA-cardiomyopathy, device upgrades are quite common. An ICD should be considered when the initial device implantation is planned in an LMNA mutation carrier.https://openheart.bmj.com/content/8/1/e001622.full
spellingShingle Kjell Nikus
Laura Helena Ollila
Hannu Parikka
Sini Weckström
Heliö Tiina
Timing of pacemaker and ICD implantation in LMNA mutation carriers
Open Heart
title Timing of pacemaker and ICD implantation in LMNA mutation carriers
title_full Timing of pacemaker and ICD implantation in LMNA mutation carriers
title_fullStr Timing of pacemaker and ICD implantation in LMNA mutation carriers
title_full_unstemmed Timing of pacemaker and ICD implantation in LMNA mutation carriers
title_short Timing of pacemaker and ICD implantation in LMNA mutation carriers
title_sort timing of pacemaker and icd implantation in lmna mutation carriers
url https://openheart.bmj.com/content/8/1/e001622.full
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AT laurahelenaollila timingofpacemakerandicdimplantationinlmnamutationcarriers
AT hannuparikka timingofpacemakerandicdimplantationinlmnamutationcarriers
AT siniweckstrom timingofpacemakerandicdimplantationinlmnamutationcarriers
AT heliotiina timingofpacemakerandicdimplantationinlmnamutationcarriers