Implantation of a left ventricular assist device as a destination therapy in Duchenne muscular dystrophy patients with end stage cardiac failure: Management and lessons learned

• Published in: Neuromuscular disorders : NMD 2015-01, Vol.25 (1), p.19-23
• Main Authors: Iodice, Francesca, Testa, Giuseppina, Averardi, Marco, Brancaccio, Gianluca, Amodeo, Antonio, Cogo, Paola
• Format: Article
• Language: English
• Subjects: Adolescent; Cardiomyopathy, Dilated - etiology; Cardiomyopathy, Dilated - surgery; Child; Duchenne muscular dystrophy; Heart Failure - etiology; Heart Failure - surgery; Heart Ventricles - surgery; Heart-Assist Devices; Humans; Left ventricular assist device; Male; Muscular Dystrophy, Duchenne - complications; Neurology; Treatment Outcome
• ISSN: 0960-8966; 1873-2364
• DOI: 10.1016/j.nmd.2014.08.008

Abstract Duchenne muscular dystrophy (DMD) is an X-linked recessive disorder, characterized by progressive skeletal muscle weakness, loss of ambulation, and death secondary to cardiac or respiratory failure. End-stage dilated cardiomyopathy (DCM) is a frequent finding in DMD patients, they are rarely candidates for cardiac transplantation. Recently, the use of ventricular assist devices as a destination therapy (DT) as an alternative to cardiac transplantation in DMD patients has been described. Preoperative planning and patient selection play a significant role in the successful postoperative course of these patients. We describe the preoperative, intraoperative and postoperative management of Jarvik 2000 implantation in 4 DMD pediatric (age range 12–17 years) patients. We also describe the complications that may occur. The most frequent were bleeding and difficulty in weaning from mechanical ventilation. Our standard protocol includes: 1) preoperative multidisciplinary evaluation and selection, 2) preoperative and postoperative non-invasive ventilation and cough machine cycles, 3) intraoperative use of near infrared spectroscopy (NIRS) and transesophageal echocardiography, 4) attention on surgical blood loss, use of tranexamic acid and prothrombin complexes, 5) early extubation and 6) avoiding the use of nasogastric feeding tubes and nasal temperature probes. Our case reports describe the use of Jarvik 2000 as a destination therapy in young patients emphasizing the use of ventricular assist devices as a new therapeutic option in DMD.