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Initial multicenter experience with ventricular assist devices in children and young adults with muscular dystrophy: An ACTION registry analysis

Authors :
Deipanjan Nandi
Scott R. Auerbach
Neha Bansal
Holger Buchholz
Jennifer Conway
Paul Esteso
Beth D. Kaufman
Ashwin K. Lal
Sabrina P. Law
Angela Lorts
Lindsay J. May
Mary Mehegan
Deepa Mokshagundam
David L.S. Morales
Matthew J. O'Connor
David N. Rosenthal
Muhammad F. Shezad
Kathleen E. Simpson
David L. Sutcliffe
Christina Vanderpluym
Carol A. Wittlieb-Weber
Farhan Zafar
Linda Cripe
Chet R. Villa
Source :
The Journal of heart and lung transplantation : the official publication of the International Society for Heart Transplantation.
Publication Year :
2022

Abstract

Cardiac disease results in significant morbidity and mortality in patients with muscular dystrophy (MD). Single centers have reported their ventricular assist device (VAD) experience in specific MDs and in limited numbers. This study sought to describe the outcomes associated with VAD therapy in an unselected population across multiple centers.We examined outcomes of patients with MD and dilated cardiomyopathy implanted with a VAD at Advanced Cardiac Therapies Improving Outcomes Network (ACTION) centers from 9/2012 to 9/2020.A total of 19 VADs were implanted in 18 patients across 12 sites. The majority of patients had dystrophinopathy (66%) and the median age at implant was 17.2 years (range 11.7-29.5). Eleven patients were non-ambulatory (61%) and 6 (33%) were on respiratory support pre-VAD. Five (28%) patients were implanted as a bridge to transplant, 4 of whom survived to transplant. Of 13 patients implanted as bridge to decision or destination therapy, 77% were alive at 1 year and 69% at 2 years. The overall frequencies of positive outcome (transplanted or alive on device) at 1 year and 2 years were 84% and 78%, respectively. Two patients suffered a stroke, 2 developed sepsis, 1 required tracheostomy, and 1 experienced severe right heart failure requiring right-sided VAD.This study demonstrates the potential utility of VAD therapies in patients with muscular dystrophy. Further research is needed to further improve outcomes and better determine which patients may benefit most from VAD therapy in terms of survival and quality of life.

Details

ISSN :
15573117
Database :
OpenAIRE
Journal :
The Journal of heart and lung transplantation : the official publication of the International Society for Heart Transplantation
Accession number :
edsair.doi.dedup.....cb4ea805c74847484c2b804e6dde0996