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      Providing a framework of principles for conceptualising the Fontan circulation

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          Abstract

          The Fontan operation remains the final palliation for thousands of patients with complex congenital heart disease. By creating a Fontan circuit, control of cardiac output and congestion is wrested away from the ventricle and new haemodynamic forces take control. Understanding how to control the flow in the Fontan circuit will enable clinicians to improve patient management and possibly prevent future complications.

          Conclusion

          This review proposes a framework of principles to conceptualise the functionality and limitations of a Fontan circulation.

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          Evaluation and Management of the Child and Adult With Fontan Circulation: A Scientific Statement From the American Heart Association

          Jack Rychik, Andrew M Atz, David Celermajer … (2019)
          Article 0 comments Cited 155 times – based on 0 reviews     Review now
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            The Fontan circulation after 45 years: update in physiology

            Marc Gewillig, Stephen C Brown (2016)
            The Fontan operation was first performed in 1968. Since then, this operation has been performed on thousands of patients worldwide. Results vary from very good for many decades to very bad with a pleiad of complications and early death. A good understanding of the physiology is necessary to further improve results. The Fontan connection creates a critical bottleneck with obligatory upstream congestion and downstream decreased flow; these two features are the basic cause of the majority of the physiologic impairments of this circulation. The ventricle, while still the engine of the circuit, cannot compensate for the major flow restriction of the Fontan bottleneck: the suction required to compensate for the barrier effect cannot be generated, specifically not in a deprived heart. Except for some extreme situations, the heart therefore no longer controls cardiac output nor can it significantly alter the degree of systemic venous congestion. Adequate growth and development of the pulmonary arteries is extremely important as pulmonary vascular impedance will become the major determinant of Fontan outcome. Key features of the Fontan ventricle are early volume overload and overgrowth, but currently chronic preload deprivation with increasing filling pressures. A functional decline of the Fontan circuit is expected and observed as pulmonary vascular resistance and ventricular filling pressure increase with time. Treatment strategies will only be successful if they open up or bypass the critical bottleneck or act on immediate surroundings (impedance of the Fontan neoportal system, fenestration, enhanced ventricular suction).
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              Total cavopulmonary connection: a logical alternative to atriopulmonary connection for complex Fontan operations. Experimental studies and early clinical experience.

              P Kilner, M Gewillig, Simon Bull … (1988)
              To understand better the contribution of a right atrium in a valveless atriopulmonary connection, we performed some basic hydrodynamic studies. Pulsation of a valveless chamber in a simple continuous flow circuit was found to generate turbulence and thereby to increase resistance to net forward flow. Visualization of flow through cavities and around corners and measurements of energy losses across nonpulsatile cavities, corners, and stenoses indicated the importance of streamlining. These studies suggested ways in which hydrodynamic designs of the Fontan circulation might be improved. In parallel with these in vitro studies, we have developed a modified approach to Fontan reconstruction that entails exclusion of most or all of the right atrium (total cavopulmonary connection). The operation consists of three parts: (1) end-to-side anastomosis of the superior vena cava to the undivided right pulmonary artery; (2) construction of a composite intraatrial tunnel with the use of the posterior wall of the right atrium; and (3) use of a prosthetic patch to channel the inferior vena cava to the enlarged orifice of the transected superior vena cava that is anastomosed to the main pulmonary artery. The operation was performed in 20 patients between March 1987 and March 1988. The diagnoses were double-inlet ventricle (11 patients), hypoplastic systemic or pulmonary ventricle (seven patients), and absent right atrioventricular connection (two patients). There were two early deaths and one late death. None of the deaths was related to the actual procedure but rather to increased pulmonary vascular resistance (two patients) or systemic ventricular failure (one patient). Total cavopulmonary connections have the following advantages: (1) They are technically simple and reproducible in any atrioventricular arrangement and are away from the atrioventricular node; (2) most of the right atrial chamber remains at low pressure, which reduces the risk of early or late arrhythmias; (3) reduction of turbulence prevents energy losses and should minimize the risk of atrial thrombosis; (4) postoperative cardiac catheterization performed in 10 patients confirmed these favorable flow patterns with minimal gradients throughout the connections. These encouraging early results support the continuing use of total cavopulmonary connection, at least for patients with a nonhypertrophied right atrium.
              Article 0 comments Cited 71 times – based on 0 reviews     Review now
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                Author and article information

                Contributors
                Marc Gewillig:
                ORCID: https://orcid.org/0000-0002-4595-5922
                marc.gewillig@uzleuven.be
                Journal
                Journal ID (nlm-ta): Acta Paediatr
                Journal ID (iso-abbrev): Acta Paediatr
                Journal ID (doi): 10.1111/(ISSN)1651-2227
                Journal ID (publisher-id): APA
                Title: Acta Paediatrica (Oslo, Norway : 1992)
                Publisher: John Wiley and Sons Inc. (Hoboken )
                ISSN (Print): 0803-5253
                ISSN (Electronic): 1651-2227
                Publication date (Electronic): 08 January 2020
                Publication date (Print): April 2020
                Volume: 109
                Issue: 4 ( doiID: 10.1111/apa.v109.4 )
                Pages: 651-658
                Affiliations
                [ 1 ] Pediatric Cardiology University Hospitals Leuven Leuven Belgium
                [ 2 ] Pediatric Cardiology University of the Free State Bloemfontein South Africa
                [ 3 ] Congenital Cardiology University Hospital Leuven Leuven Belgium
                [ 4 ] Pediatric Cardiology The Children's Hospital of Philadelphia Philadelphia PA USA
                Author notes
                [*] [* ] Correspondence

                Marc Gewillig, Pediatric Cardiology, University Hospital Leuven, B ‐ 3000 Leuven, Belgium.

                Email: marc.gewillig@ 123456uzleuven.be

                Author information
                https://orcid.org/0000-0002-4595-5922
                Article
                Publisher ID: APA15098
                DOI: 10.1111/apa.15098
                PMC ID: 7155129
                PubMed ID: 31737940
                SO-VID: 727b336f-3f7d-423a-bfdc-4f1b85766a22
                Copyright © © 2019 The Authors. Acta Paediatrica published by John Wiley & Sons Ltd on behalf of Foundation Acta Paediatrica
                License:

                This is an open access article under the terms of the http://creativecommons.org/licenses/by-nc/4.0/ License, which permits use, distribution and reproduction in any medium, provided the original work is properly cited and is not used for commercial purposes.

                History
                Date received : 22 September 2019
                Date revision received : 14 November 2019
                Date accepted : 15 November 2019
                Page count
                Figures: 7, Tables: 0, Pages: 8, Words: 5468
                Funding
                Funded by: Eddy Merckx Research Foundation
                Funded by: de Kleine Hartjes
                Categories
                Subject: Review Article
                Subject: Review Articles
                Custom metadata
                source-schema-version-number 2.0
                cover-date April 2020
                details-of-publishers-convertor Converter:WILEY_ML3GV2_TO_JATSPMC version:5.8.0 mode:remove_FC converted:14.04.2020

                ScienceOpen disciplines: Pediatrics
                Keywords: circulation failure,diastolic function,fontan,pulmonary vasculature,pulmonary vascular resistance
                Data availability:
                ScienceOpen disciplines: Pediatrics
                Keywords: circulation failure, diastolic function, fontan, pulmonary vasculature, pulmonary vascular resistance

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