Fontan Geometry and Hemodynamics Are Associated with Quality of Life in Adolescents and Young Adults

• Type of Material: Article
• Authors: Laura Mercer-Rosa, Perelman School of Medicine at the University of Pennsylvania; Mark A. Fogel, Perelman School of Medicine at the University of Pennsylvania; Zhenglun Alan Wei, Emory University; Phillip M. Trusty, Emory University; Michael Tree, Emory University; Elaine Tang, Emory University; Maria Restrepo, Emory University; Kevin K. Whitehead, Perelman School of Medicine at the University of Pennsylvania; Amy Cassedy, University of Cincinnati; Stephen M. Paridon, Perelman School of Medicine at the University of Pennsylvania; Ajit Yoganathan, Emory University; Bradley S. Marino, Cleveland Clinic Foundation
• Language: English
• Date: 2022-02-01
• Publisher: Elsevier
• Publication Version: Author Accepted Manuscript (After Peer Review)
• Copyright Statement: © 2022 by The Society of Thoracic Surgeons
• License: Creative Commons Attribution-NonCommercial-NoDerivs 4.0 International
• Final Published Version (URL): https://doi.org/10.1016%2Fj.athoracsur.2022.01.017
• Title of Journal or Parent Work: The Annals of Thoracic Surgery
• Volume: 114
• Issue: 3
• Start Page: 841
• End Page: 847
• Grant/Funding Information: Sources of Funding: This study was supported by grant NIH R01 HL67622, and by NIH R01 HL098252.; Dr. Mercer-Rosa was supported by grant NIH K01 HL125521, and by Pulmonary Hypertension Association Supplement to K01 HL125521
• Keywords: quality of life; fontan; congenital heart disease; magnetic resonance imaging
• Research Categories: Health Sciences, Medicine and Surgery

Abstract

BACKGROUND: Despite favorable short-term outcomes, Fontan palliation is associated with comorbidities and diminished quality of life (QOL) in the years following completion. We hypothesized that poor Fontan hemodynamics and ventricular function are associated with worse QOL. METHODS: Single center study of Fontan survivors older than 12 years. Subjects completed a cardiac magnetic resonance (CMR) scan and QOL questionnaire. CMR-derived variables included Fontan geometry, and hemodynamics. Computational fluid dynamic (CFD) simulations quantified power loss, pressure drop and total cavo-pulmonary connection (TCPC) resistance across the Fontan. QOL was assessed by completion of the Pediatric Quality of Life Inventory (PedsQL). Longitudinal and cross-sectional comparisons were made between CMR and CFD parameters with patient-reported QOL. RESULTS: We studied 77 Fontan patients, median age 19.7 years (IQR 17.1, 23.6), median time from Fontan completion 16 years (IQR 13, 20). Longitudinal data were available for 48 patients (median time between CMR and QOL of 8.1 years (IQR 7.0, 9.4)). Median patient-reported PedsQL Total score was 80.0 (IQR 67.4, 88.0). Greater power loss and smaller left pulmonary artery diameter at baseline were associated with worse QOL at follow up. Greater pressure drop was associated with worse QOL at the same time point. CONCLUSIONS: In Fontan survivors, measures of CFD hemodynamics and geometry are associated with worse QOL. Interventional strategies targeted at optimizing the Fontan may improve QOL.