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Left Ventricular Assist Device Support for Fabry Cardiomyopathy: A Case Series
M D Omid Kiamanesh1,2,3, Yasbanoo Moayedi2,3, Natasha Aleksova2,3
1Department of Cardiac Sciences, University of Calgary, Calgary, Alberta, Canada.
CJC Open
|March 1, 2021
Summary
Left ventricular assist device (LVAD) support is feasible for patients with Fabry disease cardiomyopathy, improving survival and quality of life. Careful management is crucial to mitigate risks and initiate enzyme replacement therapy.
Area of Science:
- Cardiology
- Genetics
- Medical Devices
Background:
- Fabry disease causes restrictive cardiomyopathy, often excluding patients from left ventricular assist device (LVAD) support due to small left ventricular (LV) cavities and suction risk.
- Limited data exists on LVAD use in Fabry disease patients.
Observation:
- This study presents the first case series of LVAD support in patients with Fabry disease.
- Patients with Fabry disease-related restrictive cardiomyopathy were evaluated for LVAD implantation.
- The study observed outcomes for both bridging to transplant and destination therapy.
Findings:
- LVAD therapy demonstrated potential to improve survival and quality of life in this patient cohort.
- Clinical stability achieved with LVAD support facilitated the initiation of enzyme replacement therapy.
- Specific surgical precautions, including fever management to prevent Fabry crises and multidisciplinary team involvement, were highlighted.
Implications:
- LVAD support may be a feasible therapeutic option for select Fabry disease patients with advanced cardiomyopathy.
- Early rehabilitation and multidisciplinary care are essential for optimizing LVAD outcomes in this population.
- This case series expands the understanding of LVAD candidacy and management in rare genetic cardiomyopathies.
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