Prescription Patterns of Cardiovascular Medications in the Adolescent and Adult Population With Fontan Circulation—A Report From the Multicenter Single Ventricle Outcomes Network

BACKGROUND: There are limited data on prescription patterns in adolescents and adults with Fontan circulation. METHODS: We conducted a retrospective analysis of patients 13 years of age and older enrolled between 2022 and 2024 in the Fontan Outcomes Network (now SV-ONE [Single Ventricle Outcomes Network]) who had undergone Fontan operation without subsequent Fontan takedown or heart transplantation. Demographics, clinical variables, and medications prescribed were assessed. Logistic regression was used to identify factors associated with prescribed cardiac medications. RESULTS: There were 639 patients (age range 13-67 years, 55.6% male, 81.1% White race, 46.8% dominant left ventricle) enrolled at 16 centers. The most frequently prescribed medication classes were antiplatelets (75.6%) and renin-angiotensin system inhibitors (55.9%). Direct oral anticoagulant prescription (15.0%) exceeded that of warfarin (11.6%). SGLT2 (sodium glucose cotransporter 2) inhibitors were prescribed to 6.3% of patients, and angiotensin receptor/neprilysin inhibitors were prescribed to 2.2%. Moderate or severely decreased systolic ventricular function was most strongly associated with prescription of digoxin (odds ratio [OR], 4.2 [95% CI, 2.1-8.3]). Protein losing enteropathy was most strongly associated with prescription of diuretics (OR, 22.9 [95% CI, 8.1-64.9]), mineralocorticoid receptor agonists (OR, 5.3 [95% CI, 2.4-11.8]), and pulmonary vasodilators (OR, 5.2 [95% CI, 2.5-10.8]). CONCLUSIONS: In a multicenter cohort of adolescents and adults with Fontan circulation, medications indicated for biventricular heart failure with reduced ejection fraction are often used despite an absence of supportive evidence in this distinct circulation. Future prospective analysis may provide further insight into shifting prescribing patterns and response to therapy.