Left Ventricular Assist Device Support for Fabry Cardiomyopathy: A Case SeriesNovel Teaching Points
M.D. Omid Kiamanesh,
Yasbanoo Moayedi, MD,
Natasha Aleksova, MD,
Juan Duero Posada, MD, MSc,
Ariel Gershon, MD,
Michael A. Seidman, MD, PhD,
Vivek Rao, MD, PhD,
Filio Billia, MD, PhD
Affiliations
M.D. Omid Kiamanesh
Department of Cardiac Sciences, University of Calgary, Calgary, Alberta, Canada; Peter Munk Cardiac Centre, University Health Network, Toronto, Ontario, Canada; Ted Roger's Center for Heart Research, Toronto, Ontario, Canada
Yasbanoo Moayedi, MD
Peter Munk Cardiac Centre, University Health Network, Toronto, Ontario, Canada; Ted Roger's Center for Heart Research, Toronto, Ontario, Canada
Natasha Aleksova, MD
Peter Munk Cardiac Centre, University Health Network, Toronto, Ontario, Canada; Ted Roger's Center for Heart Research, Toronto, Ontario, Canada
Juan Duero Posada, MD, MSc
Peter Munk Cardiac Centre, University Health Network, Toronto, Ontario, Canada; Division of Cardiology, University of Toronto, Toronto, Ontario, Canada; Ted Roger's Center for Heart Research, Toronto, Ontario, Canada
Ariel Gershon, MD
Department of Laboratory Medicine and Pathobiology, University of Toronto, Toronto, Ontario, Canada; Laboratory Medicine Program, University Health Network, Toronto, Ontario, Canada
Michael A. Seidman, MD, PhD
Department of Laboratory Medicine and Pathobiology, University of Toronto, Toronto, Ontario, Canada; Laboratory Medicine Program, University Health Network, Toronto, Ontario, Canada
Vivek Rao, MD, PhD
Peter Munk Cardiac Centre, University Health Network, Toronto, Ontario, Canada; Division of Cardiac Surgery, University of Toronto, Toronto, Ontario, Canada; Ted Roger's Center for Heart Research, Toronto, Ontario, Canada
Filio Billia, MD, PhD
Peter Munk Cardiac Centre, University Health Network, Toronto, Ontario, Canada; Division of Cardiology, University of Toronto, Toronto, Ontario, Canada; Ted Roger's Center for Heart Research, Toronto, Ontario, Canada; Corresponding author: Dr Filio Billia, University Health Network, 200 Elizabeth St, Toronto, Ontario M5G 2N2, Canada. Tel.: +1-416-340 4800 x 6805; fax: +1-416-340-4012.
Patients with restrictive cardiomyopathy due to Fabry disease are often deemed ineligible for left ventricular assist device (LVAD) support due to the risk of suction events with a small LV cavity. We describe the first case series of LVAD support for Fabry disease. LVAD therapy can improve survival, quality of life, and provide clinical stability to start enzyme replacement therapy. Precautions at the time of surgery include rapid treatment of fever to avoid Fabry crises, involvement of a multidisciplinary team, and early initiation of rehabilitation. We describe LVAD support for both bridging and destination therapy. Résumé: Les patients qui ont une cardiomyopathie restrictive due à la maladie de Fabry sont souvent considérés non admissibles aux dispositifs d’assistance ventriculaire gauche (DAVG) en raison du risque de succion lié à la petite cavité VG. Nous décrivons la première série de cas liée aux DAVG en présence de la maladie de Fabry. La thérapie par DAVG peut contribuer à l’amélioration de la survie, de la qualité de vie et offrir une stabilité clinique pour débuter l’enzymothérapie de substitution. Parmi les précautions à prendre au moment de l’intervention chirurgicale figurent le traitement rapide de la fièvre pour éviter les crises de Fabry, la participation de l’équipe multidisciplinaire et la mise en place précoce de la réadaptation. Nous décrivons l’utilisation du DAVG à titre de pont jusqu’à la transplantation et à titre de thérapie définitive.
