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We read with great interest the case reported by Stoller and colleagues, regarding the implantation of a centrifugal continuous left ventricular assist device (LVAD) as a long-term support in a patient with Duchenne muscular dystrophy (DMD) presented with cardiogenic shock. The authors addressed an important issue: despite increased awareness among clinicians, heart failure (HF) is increasingly becoming the leading cause of death in DMD patients. Unfortunately, none of the available therapeutic options address the progressive deterioration of ventricular performance. Moreover, DMD has traditionally been considered as a relative contraindication for cardiac transplantation. A possible treatment for end-stage HF in these patients is the use of LVAD as a destination therapy (DT). Nevertheless, caution has to be exercised since the current International Society for Heart & Lung Transplantation (ISHLT) indication, conceived for chronic old patients, excludes patients with important disability. Therefore, the frail status in DMD patients is an important point to be addressed and discussed with every single patient, especially when defining the strategy in accordance with the family’s and caregivers’ expectations. Our group reported in 2012 the first two cases of successful LVAD implantation as DT in adolescents with DMD, using the Jarvik 2000 (Jarvik Heart, Inc, New York), a thumb-sized axial flow impeller pump. Since that time, we have treated seven DMD patients. All patients survived to hospital discharge: this represents the most extensive cohort of patients available. At a median follow-up time of 21.7 months (range, 3–45 months), there have been three deaths: one patient died of a lung infection after 45 months, one died of tracheal bleeding after 29 months, and one died of cerebral haemorrhage after 14 months. Remarkably, a patient survived at a follow-up of 1825 days: to the best of our knowledge, this is the longest follow-up available for a patient with an LVAD affected by DMD. Our current data are consistent with those presented by Stoller et al. and also by other groups, confirming the feasibility of using LVAD as DT in DMD subjects with HF. A multidisciplinary approach with careful evaluation of frailty and co-morbidities is crucial to assess the proper selection of DMD patients. Actually, a shared decision process is needed to obtain a collaborative contact with patient, parents, and caregivers, making this strategy successful. We strongly believe that DMD patients deserve LVAD as DT, and so we encourage other groups to consider this therapeutic strategy and share it with patients, family, and all caregivers as a part of palliative strategy.

esc heart failure

Comment on:  ‘Implantation of a left ventricular assist device to provide long term support for end‐stage Duchenne muscular dystrophy‐associated cardiomyopathy’ by Stoller  et al .