The Fontan Patient: Inconsistencies in Medication Therapy Across Seven Pediatric Heart Network Centers |
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Authors: | Page A W Anderson Roger E Breitbart Brian W McCrindle Lynn A Sleeper Andrew M Atz Daphne T Hsu Minmin Lu Renee Margossian Richard V Williams |
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Institution: | (1) Department of Pediatrics, Duke University Medical Center, Durham, NC, USA;(2) Department of Cardiology, Children’s Hospital Boston, 300 Longwood Avenue, Boston, MA 02115, USA;(3) Department of Pediatrics, The Hospital for Sick Children, Toronto, ON, Canada;(4) Center for Statistical Analysis and Research, New England Research Institutes, Watertown, MA, USA;(5) Department of Pediatrics, Medical University of South Carolina, Charleston, SC, USA;(6) Department of Pediatrics, Children’s Hospital of New York, New York, NY, USA;(7) Department of Pediatrics, Primary Children’s Medical Center, Salt Lake City, UT, USA;(8) Present address: The Children’s Hospital at Montifiore, Bronx, NY, USA; |
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Abstract: | Patients who have undergone the Fontan procedure are at risk for thrombosis, ventricular dysfunction, and valve regurgitation,
but data to guide the medical treatment and prevention of these adverse outcomes in this population are lacking. This analysis
examined medication usage among Fontan patients by putative indication and by study center. The medical history and current
medications of 546 Fontan subjects, ages 6–18 years, were assessed in a Pediatric Heart Network multicenter cross-sectional
study. Cardiac imaging was performed within 3 months of enrollment. The majority of the subjects (64%) were taking two or
more medications. Antithrombotics were taken by 86% of those with a history of stroke, thrombosis, or both and 67% of those
without such a history (P = 0.01). Conversely, 14% of those with a history of stroke, thrombosis, or both were taking no antithrombotic. Angiotensin-converting
enzyme inhibitor (ACEi) therapy was independently associated with moderate or severe atrioventricular valve regurgitation
(P = 0.004), right ventricular morphology (P < 0.001), and shorter time since Fontan (P = 0.004) but not with ventricular systolic dysfunction. Glycoside therapy and diuretic therapy each was associated with older
age at Fontan (P = 0.001 and P = 0.023, respectively) and a history of post-Fontan arrhythmia (P < 0.001 and P = 0.003, respectively) but not with ventricular systolic dysfunction. Medication use rates varied widely among the centers,
even with controls for center differences in patient characteristics. Prospective therapeutic trials are needed to guide the
medical treatment of Fontan patients. |
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