Left ventricular assist device implantation in an adult male with Danon disease

Danon disease is an extremely rare inherited disorder characterized by cardiac involvement, myopathy, and intellectual disability. As patients with Danon disease die at an early age, mainly as a result of cardiac involvement, implantation of a left ventricular assist device (LVAD) and/or heart transplantation are essential options. However, various comorbidities associated with Danon disease should be assessed when these patients are being considered as potential heart transplant candidates. We report the case of an adult male patient with dilated-phase hypertrophic cardiomyopathy secondary to Danon disease, who received an LVAD as a bridge to transplantation. <Learning objective: Some patients with Danon disease who underwent heart transplantation have been reported in Japan, but all were female. Male patients with Danon disease have more severe systemic comorbidities than females and heart failure progression is usually too rapid for them to be listed as heart transplant candidates. We present a rare case of an adult male with Danon disease who successfully underwent implantation of a left-ventricular assist device as a bridge to transplantation.>