Left Ventricular Assist Device Implantation and Heart Transplantation in a Young Man With Duchenne Muscular Dystrophy.

BACKGROUND

With advances in respiratory care allowing for improved survival, cardiomyopathy has emerged as the leading cause of death in patients with Duchenne Muscular Dystrophy (DMD). As end-stage heart failure emerges as the primary life-limiting complication in DMD patients, their consideration for advanced heart failure therapies, including ventricular assist devices (VADs) and/or heart transplantation (HT), is increasing. To date, however, there are few published reports of HT in DMD patients.

METHODS

We report a case of HT in an 18-year-old young man with DMD, end-stage heart failure, respiratory insufficiency requiring nocturnal noninvasive ventilation, and retained ambulation with the use of a cane, who was declined for VAD and HT at an adult heart transplant center; he then received an LVAD, followed by HT after 362 days of support at a pediatric center.

RESULTS

At 3.5 years of follow-up, the patient has excellent graft function and modestly improved pulmonary mechanics, though has experienced a functional decline from ambulatory status to the need for full assistance with activities of daily living.

CONCLUSIONS

This case underscores the importance of individual consideration for HT in patients with DMD, provides additional insight into the unique risks of VAD support in these patients, and further builds upon the existing experience that acceptable post-HT outcomes are achievable in this population.