Abstract

Background: Patients who have undergone Fontan palliation are at life-long risk of significant morbidities including thrombosis, heart failure, and arrhythmia. At present there is limited evidence to guide pharmacotherapy and no recent studies have evaluated US pharmaceutical 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 from the Marketscan® Commercial and Medicaid databases. Cardiac pharmaceuticals were divided by class and function. Anticoagualants, other than aspirin, which is 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. Results: A cohort of 4056 subjects [median age 12 years (IQR: 8-16), 61% male, 60% commercial insurance] was identified. The most common medication classes were angiotensin converting enzyme inhibitors/angiotensin receptor blockers (ACEI/ARB), diuretics, cardiac glycosides, and pulmonary vasodilators (Table), although 50% received no prescription medications. Older age was associated with increased likelihood of receiving anticoagulants (p=0.008), antiarrhythmic agents, digoxin, ACEI/ARB, and beta blockers (each p <0.0001 ), but also lower likelihood of receiving pulmonary vasodilators, diuretics (both p <0.0001), and potassium sparing diuretics ( p =0.02). Conclusion: Pharmacotherapy typically used to treat adult heart failure and pulmonary hypertension are the most commonly prescribed medications following Fontan palliation. Determining the optimal regimen for individual patients and the population at large is an important knowledge gap for future research.

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