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      The Influence of Respiration on Blood Flow in the Fontan Circulation: Insights for Imaging-Based Clinical Evaluation of the Total Cavopulmonary Connection

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          Abstract

          Congenital heart disease is the most common birth defect and functionally univentricular heart defects represent the most severe end of this spectrum. The Fontan circulation provides an unique solution for single ventricle patients, by connecting both caval veins directly to the pulmonary arteries. As a result, the pulmonary circulation in Fontan palliated patients is characterized by a passive, low-energy circulation that depends on increased systemic venous pressure to drive blood toward the lungs. The absence of a subpulmonary ventricle led to the widely believed concept that respiration, by sucking blood to the pulmonary circulation during inspiration, is of great importance as a driving force for antegrade blood flow in Fontan patients. However, recent studies show that respiration influences pulsatility, but has a limited effect on net forward flow in the Fontan circulation. Importantly, since MRI examination is recommended every 2 years in Fontan patients, clinicians should be aware that most conventional MRI flow sequences do not capture the pulsatility of the blood flow as a result of the respiration. In this review, the unique flow dynamics influenced by the cardiac and respiratory cycle at multiple locations within the Fontan circulation is discussed. The impact of (not) incorporating respiration in different MRI flow sequences on the interpretation of clinical flow parameters will be covered. Finally, the influence of incorporating respiration in advanced computational fluid dynamic modeling will be outlined.

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          Most cited references67

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          Heart Disease and Stroke Statistics—2015 Update: A Report From the American Heart Association

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            4D flow cardiovascular magnetic resonance consensus statement

            Pulsatile blood flow through the cavities of the heart and great vessels is time-varying and multidirectional. Access to all regions, phases and directions of cardiovascular flows has formerly been limited. Four-dimensional (4D) flow cardiovascular magnetic resonance (CMR) has enabled more comprehensive access to such flows, with typical spatial resolution of 1.5×1.5×1.5 – 3×3×3 mm3, typical temporal resolution of 30–40 ms, and acquisition times in the order of 5 to 25 min. This consensus paper is the work of physicists, physicians and biomedical engineers, active in the development and implementation of 4D Flow CMR, who have repeatedly met to share experience and ideas. The paper aims to assist understanding of acquisition and analysis methods, and their potential clinical applications with a focus on the heart and greater vessels. We describe that 4D Flow CMR can be clinically advantageous because placement of a single acquisition volume is straightforward and enables flow through any plane across it to be calculated retrospectively and with good accuracy. We also specify research and development goals that have yet to be satisfactorily achieved. Derived flow parameters, generally needing further development or validation for clinical use, include measurements of wall shear stress, pressure difference, turbulent kinetic energy, and intracardiac flow components. The dependence of measurement accuracy on acquisition parameters is considered, as are the uses of different visualization strategies for appropriate representation of time-varying multidirectional flow fields. Finally, we offer suggestions for more consistent, user-friendly implementation of 4D Flow CMR acquisition and data handling with a view to multicenter studies and more widespread adoption of the approach in routine clinical investigations.
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              Evaluation and Management of the Child and Adult With Fontan Circulation: A Scientific Statement From the American Heart Association

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                Author and article information

                Contributors
                Journal
                Front Cardiovasc Med
                Front Cardiovasc Med
                Front. Cardiovasc. Med.
                Frontiers in Cardiovascular Medicine
                Frontiers Media S.A.
                2297-055X
                05 August 2021
                2021
                : 8
                : 683849
                Affiliations
                [1] 1Department of Cardiology, Biomedical Engineering, Biomechanics Laboratory , Rotterdam, Netherlands
                [2] 2Department of Cardiothoracic Surgery, Leiden University Medical Center , Leiden, Netherlands
                [3] 3Department of Anatomy, Embryology and Cardiology, Leiden University Medical Center , Leiden, Netherlands
                [4] 4Department of Chemical Engineering, Faculty of Applied Sciences, Delft University of Technology and J. M. Burgerscentrum Research School for Fluid Mechanics , Delft, Netherlands
                [5] 5Department of Radiology, Leiden University Medical Center , Leiden, Netherlands
                [6] 6Department of Pediatric Cardiology, Leiden University Medical Center , Leiden, Netherlands
                Author notes

                Edited by: Yves D'Udekem, Children's National Hospital, United States

                Reviewed by: Sezen Ugan Atik, Mehmet Akif Ersoy Thoracic and Cardiovascular Surgery Training and Research Hospital, Turkey; Joris Lemson, Radboud University Nijmegen, Netherlands

                *Correspondence: Friso M. Rijnberg f.m.rijnberg@ 123456lumc.nl

                This article was submitted to Pediatric Cardiology, a section of the journal Frontiers in Cardiovascular Medicine

                †These authors share first authorship

                Article
                10.3389/fcvm.2021.683849
                8374887
                34422920
                03aad243-78ca-4395-8362-77dbb0611225
                Copyright © 2021 van der Woude, Rijnberg, Hazekamp, Jongbloed, Kenjeres, Lamb, Westenberg, Roest and Wentzel.

                This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.

                History
                : 22 March 2021
                : 05 July 2021
                Page count
                Figures: 4, Tables: 1, Equations: 0, References: 68, Pages: 12, Words: 8509
                Funding
                Funded by: Hartstichting 10.13039/501100002996
                Categories
                Cardiovascular Medicine
                Review

                fontan,total cavopulmonary connection,respiration - physiology,flow imaging,mri,hepatic veins,blood flow,extracardiac conduit fontan

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