Health Care Use of Cardiac Specialty Care in Children With Muscular Dystrophy in the United States

Erika J. Mejia*, Kimberly Y. Lin, Oluwatimilehin Okunowo, Katherine A. Iacobellis, Susan E. Matesanz, John F. Brandsema, Carol A. Wittlieb-Weber, Hannah Katcoff, Heather Griffis, Jonathan B. Edelson

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

Abstract

BACKGROUND: Duchenne and Becker muscular dystrophy are progressive disorders associated with cardiac mortality. Guidelines recommend routine surveillance; we assess cardiac resource use and identify gaps in care delivery. METHODS AND RESULTS: Male patients, aged 1 to 18 years, with Duchenne and Becker muscular dystrophy between January 2013 and December 2017 were identified in the IBM MarketScan Research Database. The cohort was divided into <10 and 10 to 18 years of age. The primary outcome was rate of annual health care resource per person year. Resource use was assessed for place of service, cardiac testing, and medications. Adjusted incidence rate ratios (IRRs) were estimated using a Poisson regression model. Medication use was measured by proportion of days covered. There were 1386 patients with a median follow-up time of 3.0 years (interquartile range, 1.9–4.7 years). Patients in the 10 to 18 years group had only 0.40 (95% CI, 0.35–0.45) cardiology visits per person year and 0.66 (95% CI, 0.62–0.70) echocardiography/magnetic resonance imaging per person year. Older patients had higher rates of inpatient admissions (IRR, 1.46; 95% CI, 1.03–2.09), outpatient cardiology visits (IRR, 2.0; 95% CI, 1.66–2.40), cardiac imaging (IRR, 1.59; 95% CI, 1.40–1.80), and Holter monitoring (IRR, 3.33; 95% CI, 2.35–4.73). A proportion of days covered >80% for angiotensin-converting enzyme inhibitors/angiotensin receptor blockers was observed in 13.6% (419/3083) of total person years among patients in the 10 to 18 years group. CONCLUSIONS: Children 10 to 18 years of age have higher rates of cardiac resource use compared with those <10 years of age. However, rates in both age groups fall short of guidelines. Opportunities exist to identify barriers to resource use and optimize cardiac care for patients with Duchenne and Becker muscular dystrophy.

Original languageEnglish (US)
Article numbere024722
JournalJournal of the American Heart Association
Volume11
Issue number8
DOIs
StatePublished - Apr 19 2022

Keywords

  • Becker muscular dystrophy
  • Duchenne muscular dystrophy
  • heart failure
  • resource use

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine

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