Prevalent pharmacotherapy of US Fontan survivors: A study utilizing data from the MarketScan Commercial and Medicaid claims databases

Michael L. O'Byrne*, Jennifer A. Faerber, Hannah Katcoff, Jing Huang, Jonathan B. Edelson, David M. Finkelstein, Bethan A. Lemley, Christopher M. Janson, Catherine M. Avitabile, Andrew C. Glatz, David J. Goldberg

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

3 Scopus citations


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.

Original languageEnglish (US)
Pages (from-to)158-166
Number of pages9
JournalAmerican heart journal
StatePublished - Jan 2022

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine


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