Gianluigi Perri1, Sergio Filippelli2, Rachele Adorisio2, Roberta Iacobelli2, Francesca Iodice2, Giuseppina Testa2, Maria Giovanna Paglietti3, Domenico D'Amario4, Massimo Massetti4, Antonio Amodeo2. 1. Department of Pediatric Cardiology and Cardiac Surgery, Bambino Gesù Children Hospital, IRCCS, Rome, Italy; Pneumology Unit - University Hospital Pediatric Department, Bambino Gesù Children Hospital, IRCCS, Rome, Italy; Cardiovascular Science, Cardio-Thoracic Surgery Unit, Catholic University, A. Gemelli Hospital, Rome, Italy. Electronic address: dr.gianluigiperri@gmail.com. 2. Department of Pediatric Cardiology and Cardiac Surgery, Bambino Gesù Children Hospital, IRCCS, Rome, Italy. 3. Pneumology Unit - University Hospital Pediatric Department, Bambino Gesù Children Hospital, IRCCS, Rome, Italy. 4. Cardiovascular Science, Cardio-Thoracic Surgery Unit, Catholic University, A. Gemelli Hospital, Rome, Italy.
Abstract
OBJECTIVE: We report our experience with the use of a left ventricular assist device (LVAD) as destination therapy (DT) for the management of patients with cardiac end-stage dystrophinopathies. METHODS: From February 2011 to February 2016, 7 patients with dystrophinopathies and dilated cardiomyopathy (DCM) were treated with LVADs at our institution. Median age at surgery was 16.5 years (range, 14.2-23.4 years). All patients were preoperatively evaluated by a multidisciplinary team approach. RESULTS: All patients survived to hospital discharge. The early postoperative course was characterized by abdominal bleeding (1 patient) and retropharyngeal bleeding (1 patient). Because of abdominal or retropharyngeal bleeding, both required postoperative heparin infusion discontinuation for 35 and 33 days, respectively. Among the late complications, 1 child developed osteolysis and infection at the pedestal site of the device, which required surgical displacement; 1 patient required gastrostomy as a result of poor feeding, and another had a cerebral stroke, which was treated with percutaneous thrombus aspiration. The other 2 patients did not show early or late complications. At a median follow-up time of 21.7 months (range, 3-45 months) there have been 3 deaths: 1 patient died of a lung infection after 45 months, 1 died of tracheal bleeding after 29 months, and 1 died of cerebral hemorrhage after 14 months. CONCLUSIONS: Our experience indicates that the use of an LVAD as DT in patients with dystrophinopathies with end-stage DCM is feasible, suggesting that it may be suitable as a palliative therapy for the treatment of these patients with no other therapeutic options. Copyright Â
OBJECTIVE: We report our experience with the use of a left ventricular assist device (LVAD) as destination therapy (DT) for the management of patients with cardiac end-stage dystrophinopathies. METHODS: From February 2011 to February 2016, 7 patients with dystrophinopathies and dilated cardiomyopathy (DCM) were treated with LVADs at our institution. Median age at surgery was 16.5 years (range, 14.2-23.4 years). All patients were preoperatively evaluated by a multidisciplinary team approach. RESULTS: All patients survived to hospital discharge. The early postoperative course was characterized by abdominal bleeding (1 patient) and retropharyngeal bleeding (1 patient). Because of abdominal or retropharyngeal bleeding, both required postoperative heparin infusion discontinuation for 35 and 33 days, respectively. Among the late complications, 1 child developed osteolysis and infection at the pedestal site of the device, which required surgical displacement; 1 patient required gastrostomy as a result of poor feeding, and another had a cerebral stroke, which was treated with percutaneous thrombus aspiration. The other 2 patients did not show early or late complications. At a median follow-up time of 21.7 months (range, 3-45 months) there have been 3 deaths: 1 patient died of a lung infection after 45 months, 1 died of tracheal bleeding after 29 months, and 1 died of cerebral hemorrhage after 14 months. CONCLUSIONS: Our experience indicates that the use of an LVAD as DT in patients with dystrophinopathies with end-stage DCM is feasible, suggesting that it may be suitable as a palliative therapy for the treatment of these patients with no other therapeutic options. Copyright Â
Authors: Carol A Wittlieb-Weber; Chet R Villa; Jennifer Conway; Matthew J Bock; Katheryn E Gambetta; Jonathan N Johnson; Ashwin K Lal; Kurt R Schumacher; Sabrina P Law; Shriprasad R Deshpande; Shawn C West; Joshua M Friedland-Little; Irene D Lytrivi; Michael A McCulloch; Ryan J Butts; David R Weber; Kenneth R Knecht Journal: Prog Pediatr Cardiol Date: 2019-01-11
Authors: Chet Villa; Scott R Auerbach; Neha Bansal; Brian F Birnbaum; Jennifer Conway; Paul Esteso; Katheryn Gambetta; E Kevin Hall; Beth D Kaufman; Sonya Kirmani; Ashwin K Lal; Hugo R Martinez; Deipanjan Nandi; Matthew J O'Connor; John J Parent; Frank J Raucci; Renata Shih; Svetlana Shugh; Jonathan H Soslow; Hari Tunuguntla; Carol A Wittlieb-Weber; Kathi Kinnett; Linda Cripe Journal: Pediatr Cardiol Date: 2022-01-13 Impact factor: 1.838