Abstract Objective We report our experience with use of LVAD as destination therapy for the management of patients with cardiac end-stage Distrophinopaties Methods From February 2011 to February 2016, 7 patients with Distrophinopaties and dilated cardiomyopathy were assisted with LVAD at our Institution. Median age at surgery was 16.5 years (range 14,2-23,4). A ll patients were pre-operatively evaluated by multidisciplinary team approach. Results All patients survived to hospital discharge. The early post-operative course was characterized by abdominal bleeding (1 patient) and retropharyngeal bleeding (1 patient). Due to abdominal or retropharyngeal bleeding both required post-operative heparin infusion discontinuation for 35 and 33 days respectively. Amongst late complications, one child developed osteolysis and infection at the pedestal site of device which required surgical displacement; one patient required gastrostomy due to poor feeding and another suffered a cerebral stroke treated with percutaneous thrombus aspiration. The last 2 patients did not showed early or late complications. At median FU time of 21.7 months (range 3-45) we have 3 late deaths: one patient died after 45 months for lung infection, the second died for tracheal bleeding after 29 months and the last died for cerebral hemorrhage after 14 months. Conclusions Our experience indicate that the use of VAD as DT in Distrophinopaties patients with end stage DCM is feasible, suggesting that may be a palliative therapy for the treatment of these patients with otherwise no other therapeutic options.