Fontan Pathway Growth: A Quantitative Evaluation of Lateral Tunnel and Extracardiac Cavopulmonary Connections Using Serial Cardiac Magnetic Resonance

• Type of Material: Article
• Authors: Maria Restrepo, Georgia Institute of Technology; Lucia Mirabella, Georgia Institute of Technology; Elaine Tang, Georgia Institute of Technology; Christopher M. Haggerty, Georgia Institute of Technology; Reza H. Khiabani, Georgia Institute of Technology; Francis Fynn-Thompson, Boston Children’s Hospital; Anne Marie Valente, Boston Children’s Hospital; Doff B. McElhinney, Boston Children’s Hospital; Mark A. Fogel, Children’s Hospital of Philadelphia; Ajit Yoganathan, Emory University
• Language: English
• Date: 2014-03-01
• Publisher: Elsevier
• Publication Version: Author Accepted Manuscript (After Peer Review)
• Copyright Statement: © 2014 by The Society of Thoracic Surgeons.
• License: Creative Commons Attribution-NonCommercial-NoDerivs 4.0 International
• Final Published Version (URL): http://dx.doi.org/10.1016/j.athoracsur.2013.11.015
• Title of Journal or Parent Work: Annals of Thoracic Surgery
• ISSN: 0003-4975
• Volume: 97
• Issue: 3
• Start Page: 916
• End Page: 923
• Grant/Funding Information: This work was partially supported by the National Heart, Lung, and Blood Institute, grants HL67622 and R01HL098252; and the Pre-Doctoral Fellowship Awards (13PRE14580005 and 10PRE3720002) from the American Heart Association.
• Keywords: RECONSTRUCTION; Life Sciences & Biomedicine; HEART; Cardiovascular System & Cardiology; Cardiac & Cardiovascular Systems; PULMONARY-ARTERY GROWTH; Science & Technology; BODY-SURFACE AREA; OUTCOMES; SHUNT; CONDUIT; Surgery; OPERATION; FOR-PATIENTS-WITH; Respiratory System; CHILDREN
• Research Categories: Engineering, Biomedical; Health Sciences, Medicine and Surgery

Abstract

Background: Typically, a Fontan connection is constructed as either a lateral tunnel (LT) pathway or an extracardiac (EC) conduit. The LT is formed partially by atrial wall and is assumed to have growth potential, but the extent and nature of LT pathway growth have not been well characterized. A quantitative analysis was performed to evaluate this issue. Methods: Retrospective serial cardiac magnetic resonance data were obtained for 16 LT and 9 EC patients at 2 time points (mean time between studies, 4.2 ± 1.6 years). Patient-specific anatomies and flows were reconstructed. Geometric parameters of Fontan pathway vessels and the descending aorta were quantified, normalized to body surface area (BSA), and compared between time points and Fontan pathway types. Results: Absolute LT pathway mean diameters increased over time for all but 2 patients; EC pathway size did not change (2.4 ± 2.2 mm vs 0.02 ± 2.1 mm, p < 0.05). Normalized LT and EC diameters decreased, while the size of the descending aorta increased proportionally to BSA. Growth of other cavopulmonary vessels varied. The patterns and extent of LT pathway growth were heterogeneous. Absolute flows for all vessels analyzed, except for the superior vena cava, proportionally to BSA. Conclusions: Fontan pathway vessel diameter changes over time were not proportional to somatic growth but increases in pathway flows were; LT pathway diameter changes were highly variable. These factors may impact Fontan pathway resistance and hemodynamic efficiency. These findings provide further understanding of the different characteristics of LT and EC Fontan connections and set the stage for further investigation.