Study of indications for cardiac device implantation and utilisation in Fabry cardiomyopathy
Ravi Vijapurapu1,2,3, Tarekegn Geberhiwot4,5, Ana Jovanovic6
1Department of Cardiology, Queen Elizabeth Hospital Birmingham, Birmingham, UK.
Insights
Cardiovascular implantable electronic devices (CIEDs) are frequently used in Fabry disease patients, but current guidelines for implantation are insufficient. This study highlights the need for Fabry-specific indications for CIEDs due to high arrhythmia burden.
Area of Science:
- Cardiology
- Genetics
- Medical Devices
Background:
- Fabry disease is an X-linked condition causing progressive cardiomyopathy and arrhythmias.
- Arrhythmias significantly worsen prognosis in Fabry disease patients.
- Current guidance for cardiovascular implantable electronic devices (CIEDs) in Fabry disease is limited.
Purpose of the Study:
- To evaluate CIED implantation practices in the UK for Fabry disease.
- To quantify device utilization in Fabry disease patients.
- To identify clinical and imaging markers associated with CIED implantation.
Main Methods:
- Retrospective study of 90 Fabry disease patients with CIEDs (FD-CIED group) and 276 without (FD-control).
- Inclusion of demographic, clinical, and imaging data.
- Comparison of clinical and imaging markers between groups.
Main Results:
- High rates of CIED implantation without a clear class 1 indication, particularly for implantable cardioverter-defibrillators (ICDs).
- 44% of ICDs were implanted for primary prevention outside current guidelines.
- Patients with CIEDs showed higher rates of atrial fibrillation and ventricular arrhythmias, with increased LV mass, scar tissue, and atrial size.
Conclusions:
- Arrhythmias are prevalent in Fabry disease.
- Patients with CIEDs exhibit significant arrhythmia burdens, comparable to hypertrophic cardiomyopathy.
- Fabry-specific indications for CIED implantation are necessary.
Background:
Fabry disease is a treatable X-linked condition leading to progressive cardiomyopathy, arrhythmia and premature death. Atrial and ventricular arrhythmias contribute significantly to adverse prognosis; however, guidance to determine which patients require cardiovascular implantable electronic devices (CIEDs) is sparse. We aimed to evaluate indications for implantation practice in the UK and quantify device utilisation.
Methods:
In this retrospective study, we included demographic, clinical and imaging data from patients in four of the largest UK Fabry centres. Ninety patients with Fabry disease were identified with CIEDs implanted between June 2001 and February 2018 (FD-CIED group). To investigate differences in clinical and imaging markers between those with and without devices, these patients were compared with 276 patients without a CIED (FD-control).
Results:
In the FD-CIED group, 92% of patients with permanent pacemakers but only 28% with implantable cardioverter-defibrillators had a class 1 indication for implantation. A further 44% of patients had defibrillators inserted for primary prevention outside of current guidance. The burden of arrhythmia requiring treatment in the FD-CIED group was high (asymptomatic atrial fibrillation:29%; non-sustained ventricular tachycardia requiring medical therapy alone: 26%; sustained ventricular tachycardia needing anti-tachycardia pacing/defibrillation: 28%). Those with devices were older, had greater LV mass, more scar tissue and larger atrial size.
Conclusions:
Arrhythmias are common in Fabry patients. Those with cardiac devices had high rates of atrial fibrillation requiring anticoagulation and ventricular arrhythmia needing device treatment. These are as high as those in hypertrophic cardiomyopathy, supporting the need for Fabry-specific indications for device implantation.
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