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      Fate of the Fontan connection: Mechanisms of stenosis and management


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          Stenosis of the venous connections and conduits is a well‐known late complication of the Fontan procedure. Currently, data on the outcomes of percutaneous intervention for the treatment of extra‐ or intracardiac conduits and lateral tunnel baffles obstruction are limited. In an attempt to better define the nature and severity of the stenosis and the results of catheter interventional management, we reviewed Fontan patients with obstructed extra‐ or intracardiac conduits and lateral tunnel baffles.


          Retrospective review of all Fontan patients who had cardiac catheterization from January 2002 to October 2018 was performed. Hemodynamic and angiographic data that assessed extra‐ or intracardiac conduit, or lateral tunnel baffle obstruction/stenosis were evaluated.


          Twenty patients underwent catheter intervention because of conduit stenosis, including calcified homografts, stenotic Gore‐Tex conduits and obstructed lateral tunnels. Six other patients had Fontan obstruction but were referred for surgical revision. After stenting, there was a significant reduction in the connection gradient [2.0 mm Hg (IQR 2; 3) vs 0 mm Hg (IQR 0; 1), P < .0001]. Fontan conduit/connection diameter increased [10.5 mm (IQR 9; 12) vs 18 mm (IQR 14.9; 18); < .0001] and New York Heart Association class [III (IQR II; III) vs I (IQR II; III); P = .03) with stent placement.


          We demonstrated the hemodynamics and angiographic subtypes of conduit stenosis in patients after Fontan, We showed that calcified homografts, stenotic Gore‐Tex conduits and lateral tunnels pathways can be safely and effectively stented to eliminate obstruction. Percutaneous stenting is associated with a decrease in connection gradients and improvement in functional capacity.

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          Total cavopulmonary connection: a logical alternative to atriopulmonary connection for complex Fontan operations. Experimental studies and early clinical experience.

          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.
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            Liver Disease in Patients After the Fontan Operation.

            We reviewed records of all patients with an initial Fontan operation or revision from 1973 to 2012 at our institution (n = 1,138); 195 patients had postoperative liver data available. Cirrhosis was identified by histopathology or characteristic findings on imaging with an associated diagnosis of cirrhosis by a hepatologist. Of 195 patients with biopsy or imaging, 10-, 20-, and 30-year freedom from cirrhosis was 99%, 94%, and 57%, respectively. There were 40 of 195 patients (21%) diagnosed with cirrhosis (mean age at Fontan 10.7 ± 8 years). On multivariate analysis, hypoplastic left heart syndrome was associated with increased risk of cirrhosis (n = 2 of 16, p = 0.0133), whereas preoperative sinus rhythm was protective (p = 0.009). Survival after diagnosis of cirrhosis was 57% and 35%, at 1, and 5 years, respectively. The cause of death was known for 9 patients (5 multiorgan failure, 2 liver failure, and 2 heart failure). In conclusion, there is an incremental occurrence of cirrhosis after the Fontan, which should be considered when designing follow-up protocols for patients after Fontan operation.
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              Optimal conduit size of the extracardiac Fontan operation based on energy loss and flow stagnation.

              In the extracardiac Fontan operation, larger conduits are used when considering the patients' growth rate. However, larger conduits may cause inefficient flow due to turbulence or stagnation, resulting in late problems such as thrombosis or stenosis. Our objective was to reveal the physiologic effects of respiration and exercise using numerical models, based on the energy loss and flow stagnation, and to determine optimal conduit size. For the Fontan operation, a conduit from 14 to 22 mm was created based on angiographic data from 17 Fontan patients (mean age, 36.0 months; mean body surface area, 0.53 m(2)). Respiratory-driven flow of the superior and inferior vena cava was determined at rest and during exercise on two levels (0.5 and 1.0 W/kg) by magnetic resonance imaging flow studies. Flow stagnation was defined as the volume of the region where flow velocity was less than 0.01 m/second at both the expiratory and inspiratory phases. In larger conduits, backward flow at the expiratory phase was prominent. Energy loss was small even during exercise, but the change was slightly larger between 14 and 16 mm than other conduit sizes (14 mm, 5.759 mW; 16 mm, 4.881 mW; and 22 mm, 4.199 mW during 1.0 W/kg exercise). Stagnation volume at the expiratory phase increased with an increase of conduit size (14 mm, 9.20% vs 22 mm, 33.9% conduit volume at rest). Fontan circulation is a low-energy system even during exercise. Larger conduits were proven to have redundant spaces, thus 16 and 18 mm conduits were optimal.

                Author and article information

                Congenit Heart Dis
                Congenit Heart Dis
                Congenital Heart Disease
                John Wiley and Sons Inc. (Hoboken )
                25 February 2019
                Jul-Aug 2019
                : 14
                : 4 ( doiID: 10.1111/chd.v14.4 )
                : 571-581
                [ 1 ] Division of Pediatric Cardiology, Department of Pediatric and Adolescent Medicine Mayo Clinic College of Medicine Rochester Minnesota
                [ 2 ] Department of Cardiovascular Diseases Mayo Clinic College of Medicine Rochester Minnesota
                [ 3 ] Department of Cardiovascular Surgery Mayo Clinic College of Medicine Rochester Minnesota
                Author notes
                [*] [* ] Correspondence

                Donald J. Hagler, MD, FSCAI, Division of Pediatric Cardiology, Department of Pediatric and Adolescent Medicine, Mayo Clinic College of Medicine, Rochester, MN 55905.

                Email: hagler.donald@ 123456mayo.edu

                © 2019 The Authors. Congenital Heart Disease Published by Wiley Periodicals, Inc.

                This is an open access article under the terms of the http://creativecommons.org/licenses/by-nc-nd/4.0/ License, which permits use and distribution in any medium, provided the original work is properly cited, the use is non‐commercial and no modifications or adaptations are made.

                Page count
                Figures: 7, Tables: 2, Pages: 11, Words: 7538
                Original Article
                Original Articles
                Custom metadata
                July/August 2019
                Converter:WILEY_ML3GV2_TO_JATSPMC version:5.7.1 mode:remove_FC converted:12.11.2019

                Cardiovascular Medicine
                Cardiovascular Medicine
                cirrhosis, conduit, stenosis, stenting


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