Left ventricular assist device in Duchenne cardiomyopathy: can we change the natural history of cardiac disease?

End stage dilated cardiomyopathy (DCM) is currently one of the most challenging elements in the management of patients affected by Duchenne muscular dystrophy [1]. DCM is a complication of Duchenne muscular dystrophy, and leads to advanced heart failure and premature death [2,3]. Until the last decade, cardiomyopathy inDuchennemuscular dystrophy accounted for only 20% of deaths because respiratory failure occurred earlier than cardiac failure. Due to recent technological advances, respiratory care has greatly improved and life expectancy has increased to 30–40 years. The efficacy of standard heart failure treatment for improving the clinical outcome of these patients has been proven, but neverthelessmore than40%will die of heart failure [2]. Duchenne syndrome has generally been considered a contraindication for cardiac transplantation due to the associated progressive skeletal myopathy leading to limited functional capacity [4]. This concern has resulted in a reluctance to offer cardiac transplantation to these patients in an era of donor shortage. The recent advances in left ventricular assist devices, used as destination therapy, have made feasible the use of such devices for the treatment of DCM in Duchenne patients. We present two adolescents with Duchenne muscular dystrophy admitted to our department because of acute heart failure, for which a ventricular assistant device (VAD) was decided as destination therapy. Case 1. A 15 year-old boy who, during the last year, experienced three episodes of acute cardiac failure despite maximal medical treatment, was admitted to our ICU with intractable cardiac failure unresponsive to high dosage inotropes. We elected to implant, as destination therapy, a left VAD (Jarvik 2000) with the pedestal inserted into the skull. The patient needed daily treatmentwith a coughmachine and