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Published on: February 28, 2012
Clinical utilisation of implantable loop recorders in adults with Fabry disease-a multi-centre snapshot study
Ashwin Roy1,2, Ravi Vijapurapu1,2, Hibba Kurdi3,4
1Institute of Cardiovascular Sciences, University of Birmingham, Birmingham, United Kingdom.
Insights
Implantable loop recorders (ILRs) reveal a high burden of arrhythmia in Fabry disease (FD) patients, suggesting underutilization for risk stratification. Continued monitoring post-ILR is crucial for timely intervention in this pro-arrhythmic condition.
Area of Science:
- Cardiology
- Genetics
- Medical Devices
Background:
- Fabry disease (FD) is an X-linked lysosomal storage disorder caused by alpha-galactosidase-A deficiency.
- Myocardial involvement in FD leads to arrhythmias and sudden death, necessitating improved risk stratification.
- Implantable loop recorders (ILRs) offer prolonged cardiac rhythm monitoring.
Purpose of the Study:
- To evaluate the utilization of ILRs in patients with Fabry disease.
- To quantify the burden of detected arrhythmias and their impact on therapy modification.
- To assess the role of ILRs in risk stratification for device therapy in FD.
Main Methods:
- Retrospective study of 915 Fabry disease patients across three UK specialist centers (2000-2022).
- Analysis of data from 22 patients who underwent clinically indicated ILR implantation.
- Evaluation of arrhythmia detection, interventions, and outcomes post-ILR implantation.
Main Results:
- Only 2.4% of FD patients (22/915) received ILR implantation.
- Arrhythmia requiring intervention was detected in 41% of patients with ILRs (9/22).
- Significant arrhythmias, including heart block and ventricular tachycardia, occurred post-ILR monitoring, necessitating device therapy.
Conclusions:
- Implantable loop recorders uncover a substantial burden of arrhythmias in Fabry disease patients.
- ILR utilization appears low in this pro-arrhythmic population, potentially limited to advanced cardiomyopathy cases.
- Enhanced vigilance and arrhythmia surveillance are recommended, especially after ILR monitoring concludes.
Abstract:
Fabry disease (FD) is an X-linked deficiency of alpha-galactosidase-A, leading to lysosomal storage of sphingolipids in multiple organs. Myocardial accumulation contributes to arrhythmia and sudden death, the most common cause of FD mortality. Therefore, there is a need for risk stratification and prediction to target device therapy. Implantable loop recorders (ILRs) allow for continual rhythm monitoring for up to 3 years. Here, we performed a retrospective study to evaluate current ILR utilisation in FD and quantify the burden of arrhythmia that was detected, which resulted in a modification of therapy. This was a snapshot assessment of 915 patients with FD across three specialist centres in England during the period between 1 January 2000 and 1 September 2022. In total, 22 (2.4%) patients underwent clinically indicated ILR implantation. The mean implantation age was 50 years and 13 (59%) patients were female. Following implantation, nine (41%) patients underwent arrhythmia detection, requiring intervention (six on ILR and three post-ILR battery depletion). Three patients experienced sustained atrial high-rate episodes and were started on anticoagulation. Three had non-sustained tachyarrhythmia and were started on beta blockers. Post-ILR battery depletion, one suffered complete heart block and two had sustained ventricular tachycardia, all requiring device therapy. Those with arrhythmia had a shorter PR interval on electrocardiography. This study demonstrates that ILR implantation in FD uncovers a high burden of arrhythmia. ILRs are likely to be underutilised in this pro-arrhythmic cohort, perhaps restricted to those with advanced FD cardiomyopathy. Following battery depletion in three patients as mentioned above, greater vigilance and arrhythmia surveillance are advised for those experiencing major arrhythmic events post-ILR monitoring. Further work is required to establish who would benefit most from implantation.
