Objective: In muscular dystrophies (MD) patients with end-stage heart failure (HF), continuous flow left ventricular assist device (cf-LVAD) therapy is still controversial due to a progressive nature of MD-associated muscle weakness.
Methods: All the MD patients who had cf- VAD implants between March 2013 and August 2019 in our hospital were retrospectively studied. Study end points were death, major LVAD-associated complications or respiratory dysfunction caused by muscular weakness.
Results: A total of 11 MD patients (Becker type: n = 6; Emery-Dreifuss Myodystrophy: n = 2; Fukuyama subtype: n = 1; Limb-girdle 1B: n = 2) were enrolled.
Demographics: median age 41 years (IQR; 29-47); median Japanese Registry for Mechanically Assisted Circulatory Support: level 3 (2-3); a median interval between MD diagnosis and LVAD implantation 9 years (6-18). The pulmonary function test at LVAD implantation showed a median of %VC; 62% (45-82), FEV1%, 82% (81-88). Survival to discharge was 100% without pulmonary complication and early VAD-related complications. During a median follow-up of 38 months (27-53), re-admissions were needed due to device infection (n = 2), cerebrovascular accidents (disabling, n = 2 and non-disabling, n = 2), ventricular tachycardia (n = 4), and right HF (n = 3), respectively. 7 patients received successful heart transplant after a median waiting time of 44 months (34-61); 3 patients are still on the waiting list (waiting time: 21, 38, and 39 months). One patient died of right HF 15 months after VAD implantation. No one had overt pulmonary dysfunction during LVAD support.
Conclusion: In selected MD patients with end-stage HF, cf-LVAD therapy is a viable therapeutic option as bridge to heart transplant.
Keywords: Heart failure; Left ventricle assist device; Muscle dystrophy; Pulmonary disorders.
© 2022. The Author(s), under exclusive licence to The Japanese Association for Thoracic Surgery.