Michael L. O’Byrne, Jennifer A. Faerber, Hannah Katcoff, Jing Huang, Jonathan B. Edelson, David M. Finkelstein, Bethan Lemley, Christopher M. Janson, Catherine M. Avitabile, Andrew C. Glatz, David J. Goldberg
Children’s Hospital of Philadelphia and Perelman School of Medicine at the University of Pennsylvania.
United States
American Heart Journal
Am Heart J 2022; 243: 158-166
DOI: 10.1016/j.ahj.2021.09.012
Abstract
Background: Survivors of Fontan palliation are at life-long risk of thrombosis, arrhythmia, and circulatory failure. To our knowledge, no studies have evaluated current United States pharmaceutical prescription practice in this population.
Methods: A retrospective observational study evaluating the prevalent use of prescription medications in children and adolescents with hypoplastic left heart syndrome or tricuspid atresia after Fontan completion (identified using ICD9/10 codes) was performed using data contained in the MarketScan Commercial and Medicaid databases for the years 2013 through 2018. Cardiac pharmaceuticals were divided by class. Anticoagulant agents other than platelet inhibitors, which are not uniformly a prescription medication, were also studied. Associations between increasing age and the likelihood of a filled prescription for each class of drug were evaluated. Annualized retail costs of pharmaceutical regimens were calculated.
Results: A cohort of 4,056 subjects (median age 12 years [interquartile range: 8-16], 61% male, 60% commercial insurance) was identified. Of the cohort, 50% received no prescription medications. Angiotensin converting enzyme inhibitors/angiotensin receptor blockers (ACEi/ARB) (38%), diuretics (15%), and mineralocorticoid receptor antagonists (8%) were prescribed with the highest frequency. Pulmonary vasodilators were received by 6% of subjects. Older age was associated with increased likelihood of filled prescriptions for anticoagulants (P = .008), antiarrhythmic agents, digoxin, ACEi/ARB, and beta blockers (each P < .0001), but also lower likelihood of filled prescriptions for pulmonary vasodilators, conventional diuretics (both P < .0001), and mineralocorticoid receptor antagonists (P = .02).
Conclusions: Pharmaceuticals typically used to treat heart failure and pulmonary hypertension are the most commonly prescribed medications following Fontan palliation. While the likelihood of treatment with a particular class of medication is associated with the age of the patient, determining the optimal regimen for individual patients and the population at large is an important knowledge gap for future research.
Category
Class I. Pulmonary Hypertension Associated with Congenital Cardiovascular Disease
Medical Therapy. Efficacy or Lack of Efficacy
Age Focus: Pediatric Pulmonary Vascular Disease or Adult Pulmonary Vascular Disease
Fresh or Filed Publication: Filed (PHiled). Greater than 1-2 years since publication
Article Access
Free PDF File or Full Text Article Available Through PubMed or DOI: Yes