@article {Ollilae001622, author = {Laura Helena Ollila and Kjell Nikus and Hannu Parikka and Sini Weckstr{\"o}m and Heli{\"o} Tiina}, title = {Timing of pacemaker and ICD implantation in LMNA mutation carriers}, volume = {8}, number = {1}, elocation-id = {e001622}, year = {2021}, doi = {10.1136/openhrt-2021-001622}, publisher = {Archives of Disease in childhood}, abstract = {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.No data are available. The data underlying this article cannot be shared publicly due to privacy of the individuals that participated in the study.}, URL = {https://openheart.bmj.com/content/8/1/e001622}, eprint = {https://openheart.bmj.com/content/8/1/e001622.full.pdf}, journal = {Open Heart} }