Current Practices in Treating Cardiomyopathy and Heart Failure in Duchenne Muscular Dystrophy (DMD): Understanding Care Practices in Order to Optimize DMD Heart Failure Through ACTION

Pediatric Cardiology - Tập 43 - Trang 977-985 - 2022
Chet Villa1, Scott R. Auerbach2, Neha Bansal3, Brian F. Birnbaum4, Jennifer Conway5, Paul Esteso6, Katheryn Gambetta7, E. Kevin Hall8, Beth D. Kaufman9, Sonya Kirmani10, Ashwin K. Lal11, Hugo R. Martinez12, Deipanjan Nandi13, Matthew J. O’Connor14, John J. Parent15, Frank J. Raucci16, Renata Shih17, Svetlana Shugh18, Jonathan H. Soslow19, Hari Tunuguntla20, Carol A. Wittlieb-Weber14, Kathi Kinnett21, Linda Cripe13
1Cincinnati Children’s Hospital Medical Center, University of Cincinnati College of Medicine, Cincinnati, USA
2Department of Pediatrics, Division of Cardiology, University of Colorado, Denver Anschutz Medical Campus, Children’s Hospital Colorado Aurora, Aurora, USA
3Division of Pediatric Cardiology, Children’s Hospital at Montefiore, Albert Einstein College of Medicine, Bronx, USA
4Children’s Mercy Hospital and Clinics, Kansas City, USA
5Stollery Children’s Hospital, Edmonton, Canada
6Boston Children’s Hospital, Boston, USA
7Ann and Robert H. Lurie Children’s Hospital, Chicago, USA
8Yale New Haven Children’s Hospital, Yale University School of Medicine, New Haven, USA
9Department of Pediatrics (Cardiology), Stanford University School of Medicine, Palo Alto, USA
10Duke Children’s Pediatric and Congenital Heart Center, Duke Children’s Hospital, Durham, USA
11Division of Pediatric Cardiology, Primary Children’s Hospital, University of Utah, Salt Lake City, USA
12The Heart Institute at Le Bonheur Children’s Hospital and The University of Tennessee Health Science Center, Memphis, USA
13Nationwide Children’s Hospital, Columbus, USA
14Division of Cardiology, Department of Pediatrics, University of Pennsylvania School of Medicine, Children’s Hospital of Philadelphia, Philadelphia, USA
15Riley Hospital for Children, Indiana University, Indianapolis, USA
16Children’s Hospital of Richmond, Virginia Commonwealth University Health System, Richmond, USA
17Congenital Heart Center, University of Florida, Gainesville, USA
18Heart Institute, Joe DiMaggio Children’s Hospital, Hollywood, USA
19Department of Pediatrics, Thomas P. Graham Division of Pediatric Cardiology, Monroe Carell Jr. Children’s Hospital at Vanderbilt, Nashville, USA
20Department of Pediatrics, Baylor College of Medicine, Houston, USA
21Parent Project Muscular Dystrophy, Hackensack, USA

Tóm tắt

Cardiac disease has emerged as a leading cause of mortality in Duchenne muscular dystrophy in the current era. This survey sought to identify the diagnostic and therapeutic approach to DMD among pediatric cardiologists in Advanced Cardiac Therapies Improving Outcomes Network. Pediatric cardiology providers within ACTION (a multi-center pediatric heart failure learning network) were surveyed regarding their approaches to cardiac care in DMD. Thirty-one providers from 23 centers responded. Cardiac MRI and Holter monitoring are routinely obtained, but the frequency of use and indications for ordering these tests varied widely. Angiotensin converting enzyme inhibitor and aldosterone antagonist are generally initiated prior to onset of systolic dysfunction, while the indications for initiating beta-blocker therapy vary more widely. Seventeen (55%) providers report their center has placed an implantable cardioverter defibrillator in at least 1 DMD patient, while 11 providers (35%) would not place an ICD for primary prevention in a DMD patient. Twenty-three providers (74%) would consider placement of a ventricular assist device (VAD) as destination therapy (n = 23, 74%) and three providers (10%) would consider a VAD only as bridge to transplant. Five providers (16%) would not consider VAD at their institution. Cardiac diagnostic and therapeutic approaches vary among ACTION centers, with notable variation present regarding the use of advanced therapies (ICD and VAD). The network is currently working to harmonize medical practices and optimize clinical care in an era of rapidly evolving outcomes and cardiac/skeletal muscle therapies.

Tài liệu tham khảo

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Pediatric Cardiology

Tập 43

977-985

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