Timing of pacemaker and ICD implantation in mutation carriers.

AIMS

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 mutation carriers.

METHODS

We studied the hospital records of 60 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 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 mutation carriers underwent pacemaker implantation in this study. Due to the progressive nature of -cardiomyopathy, device upgrades are quite common. An ICD should be considered when the initial device implantation is planned in an mutation carrier.