Cardiac device implantation and device usage in Fabry and hypertrophic cardiomyopathy

Area of Science:

• Cardiology
• Genetics
• Electrophysiology

Background:

• Fabry disease (FD) is an X-linked condition causing progressive cardiac issues and early mortality.
• Awareness of arrhythmogenicity in Fabry cardiomyopathy is limited.
• This study compares implantable cardioverter-defibrillator (ICD) usage in FD and hypertrophic cardiomyopathy (HCM).

Purpose of the Study:

• To increase awareness of the arrhythmogenic potential of Fabry cardiomyopathy.
• To compare device usage and arrhythmia burden in FD and HCM patients.
• To evaluate the effectiveness of current risk models in managing cardiac involvement in FD.

Main Methods:

• Retrospective analysis of all UK patients with FD receiving an ICD over 17 years.
• Inclusion of a comparator group of HCM patients with primary prevention ICD implantation from a regional registry.
• Comparison of arrhythmia occurrence, type, and ICD therapy delivery between FD and HCM cohorts.

Main Results:

• Indications for ICD implantation in FD varied, with 72% for primary prevention.
• Arrhythmias occurred more frequently in FD patients compared to HCM, even with primary prevention ICDs (HR 4.2, p<0.001).
• Ventricular tachycardia (VT) requiring therapy (HR 4.5, p=0.002), immediate shock therapy (HR 2.5, p<0.001), atrial fibrillation (AF) (HR 6.2, p=0.004), and non-sustained VT (NSVT) (HR 3.1, p<0.001) were more common in FD.

Conclusions:

• Fabry cardiomyopathy exhibits a high burden of arrhythmias and significant ICD utilization.
• The findings suggest Fabry cardiomyopathy is more arrhythmogenic than previously understood.
• Current risk stratification models may not be universally applicable to Fabry patients with cardiac involvement, warranting further investigation.