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Timing of pacemaker and ICD implantation in LMNA mutation carriers
Laura Helena Ollila1, Kjell Nikus2,3, Hannu Parikka4
1Heart and Lung Centre, Helsinki University Central Hospital, Helsinki, Finland laura.ollila@hus.fi.
Insights
Most LMNA mutation carriers require pacemaker or ICD implantation due to progressive LMNA-cardiomyopathy. Device upgrades are common, with males needing them more frequently and at a younger age. Consider ICDs for initial implantation.
Area of Science:
- Cardiology
- Genetics
- Medical Devices
Background:
- LMNA-cardiomyopathy frequently affects the cardiac conduction system, often requiring device implantation.
- Understanding device implantation patterns in LMNA mutation carriers is crucial for patient management.
Purpose of the Study:
- To investigate the timing and types of device implantations in LMNA mutation carriers.
- To determine the rate and reasons for device re-implantations in this population.
Main Methods:
- Retrospective analysis of hospital records for 60 LMNA mutation carriers.
- Data collection on device implantations, re-implantations, and indications until April 2019.
Main Results:
- 61.7% of LMNA mutation carriers received a pacemaker or implantable cardioverter defibrillator (ICD).
- 27.0% required a device upgrade, often soon after initial implantation; males underwent upgrades more frequently and at a younger age.
- 35.0% developed dilated cardiomyopathy, with 90.5% needing pacemaker implantation.
Conclusions:
- Pacemaker implantation is common in LMNA mutation carriers.
- Device upgrades are frequent due to the progressive nature of LMNA-cardiomyopathy.
- An ICD should be considered for initial device 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 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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