HeartMate 3 Left Ventricular Assist Device Implantation in a Pediatric Patient With Limb-Girdle Muscular Dystrophy.

Background: The development of cardiac complications is common in patients with muscular dystrophy. However, advanced heart failure therapies such as implantation of durable ventricular assist devices and transplant are infrequently used in patients who develop cardiomyopathy, often because of comorbid impairments in mobility and respiratory function.

Case report: A 16-year-old male with limb-girdle muscular dystrophy type R4 presented with chronic decompensated heart failure. Recent worsening of his respiratory function and mobility were confounded by severe heart failure. In addition to our core advanced heart failure team, multidisciplinary assessment preoperatively included Neurology, Pulmonology, Genetics, and Physical Medicine and Rehabilitation. The patient underwent implantation of a HeartMate 3 left ventricular assist device and had an uneventful postoperative course. After intensive inpatient physical and occupational therapy, he was discharged home on postoperative day 16 with minimal residual heart failure symptoms and plans to continue robust outpatient physical therapy.

Conclusion: Patients with muscular dystrophy often have cardiac involvement; however, certain subtypes of muscular dystrophy are associated with an earlier presentation of severe life-limiting cardiomyopathy. Pediatric patients with muscular dystrophy should be considered for advanced heart failure therapies such as implantation of a durable left ventricular assist device at an appropriate center. Carefully selected patients may experience substantial improvements in their quality of life. Given the variable disease progression and life expectancy of patients with subtypes of muscular dystrophy, a thorough assessment by a multidisciplinary team is critical.