Current Management of Late Failure After Classic Fontan Modifications: Fontan Conversions[1]

Abstract

Background: Re-establisment of Fontan circulation by eliminating the drawbacks of classic Fontan modifications has been proposed recently to improve the functional class and quality of life of patients with failed Fontan circulation. Methods: Five patients underwent extracardiac and lateral tunnel Fontan conversion due to failure of the Fontan circulation, after classic Fontan modifications. All of the patients were female and mean age was 10.2 ± 2.2 years (range: 8 - 14 years). Previous Fontan modifications were atriopulmonary anastomosis in 3 and Kawashima operation in 2 patients. Time to re-operation for Fontan conversion was 6 ± 1.5 years (range: 4 - 8 years). Indications for re-operation were right heart failure, right atrial dilatation, and intractable atrial arrhythmias in patients with previous atriopulmonary anastomosis, progressive hypoxia and exercise intolerance in patients with previous Kawashima operation due to pulmonary arteriovenous fistulas. Previous atriopulmonary anastomoses were converted to intra-atrial or lateral tunnel Fontan operation with modified right atrial Maze procedure. Extracardiac Fontan completion was carried out after previous Kawashima operations by redirection of hepatic veins to the lungs. Results: There was no mortality and no major morbidity. All patients were discharged from the hospital in good condition and in sinus rhythm. No prolonged or recurrent effusions were observed. On follow-up, all patients were in sinus rhythm and had NYHA class I functional capacity. In two Kawashima patients, SpO₂ gradually increased from 60 % to 90 % six months after the operation. Discussion: We suggest that Fontan conversion should be considered in patients with previous atriopulmonary anastomosis, when right atrial dilatation or intractable atrial arrhythmias with deteriorating functional status develops. Redirection of hepatic venous flow to lungs induces regression of pulmonary arteriovenous fistulas and improves arterial saturation in patients with previous Kawashima operation.

Fontan conversion - atriopulmonary anastomosis - Kawashima procedure - Fontan modifications

References

• 1 Fontan F, Baudet E. Surgical repair of tricuspid atresia.  Thorax. 1971;  26 240-248
• 2 Ensley A E, Lynch P, Chatzimavroudis G P, Lucas C, Sharma S, Yoganathan A P. Toward designing the optimal total cavopulmonary connection: An in vitro study.  Ann Thorac Surg. 1999;  68 1384-1390
• 3 Lardo A C, Webber S A, Friehs I, del Nido P J, Cape E G. Fluid dynamic comparison of intraatrial and extracardiac total cavopulmonary connections.  J Thorac Cardiovasc Surg. 1999;  117 697-704
• 4 Amodeo A, Galletti L, Marianeschi S, Picardo S, Giannico S, Paolo D P, Marcelletti C. Extracardiac Fontan operation for complex cardiac anomalies: Seven years' experience.  J Thorac Cardiovasc Surg. 1997;  114 1020-1031
• 5 Conte S, Gewillig M, Eyskens B, Dumoulin M, Daenen W. Management of late complications after classic Fontan procedure by conversion to total cavopulmonary connection.  Cardiovasc Surg. 1999;  7 651-655
• 6 Uemura H, Yagihara T, Hattori R, Kawahira Y, Tsukano S, Watanabe K. Redirection of hepatic venous drainage after total cavopulmonary shunt in left isomerism.  Ann Thorac Surg. 1999;  68 1731-1735
• 7 Marcelletti C F, Hanley F L, Mavroidis C, McElhinney D B, Abella R F, Maraneschi S M, Seddio F. et al . Revision of previous Fontan connections to total extracardiac cavopulmonary anastomosis: A multicenter experience.  J Thorac Cardiovasc Surg. 2000;  119 340-346
• 8 Sarıoğlu T, Paker T, Türkoğlu H, Akçevin A, Sarıoğlu A, Yalçınbaş Y, Akpınar B, Aytaç A. The modified Fontan operation in hearts associated with atrioventricular valvar atresia or common atrioventricular valve - neoseptation of the atriums using a right atrial flap.  Cardiology in the Young. 1994;  4 353-357
• 9 Yalcinbas Y K, Erek E, Salihoglu E, Sarioglu A, Sarioglu T. Early results of extracardiac Fontan procedure with autologous pericardial tube conduit.  Thorac Cardiovasc Surg. 2005;  53 37-40
• 10 Henglein D, Cauchemez B, Bloch G. Simultaneous surgical treatment of atrial septal defect and atrial flutter using a simple modification of the atrial incision.  Cardiol Young. 1999;  9 197-199
• 11 Kreutzer J, Keane J F, Lock J E, Walsh E P, Jonas R A, Castaneda A R, Mayer J E. Conversion of modified Fontan procedure to lateral atrial tunnel cavopulmonary anastomosis.  J Thorac Cardiovasc Surg. 1996;  111 1169-1176
• 12 Kao J M, Alejos J C, Grant P W. et al . Conversion of atriopulmonary to cavopulmonary anastomosis in management of late arrhythmias and atrial thrombosis.  Ann Thorac Surg. 1994;  58 1510-1514
• 13 Deal B J, Mavroidis C, Backer C L, Johnsrude C L, Rocchini A P. Impact of arrhythmia circuit cryoablation during Fontan conversion for refractory atrial tachycardia.  Am J Cardiol. 1999;  83 563-568
• 14 Michielon G, Parisi F, Di Carlo D, Squitieri C, Carotti A, Buratta M, Di Donato R M. Orthotopic heart transplantation for failing single ventricle physiology.  Eur J Cardiothorac Surg. 2003;  24 502-510
• 15 Duncan B W, Desai S. Pulmonary arteriovanous malformations after cavopulmonary anastomosis.  Ann Thorac Surg. 2003;  76 1759-1766
• 16 Agnoletti G, Borghi A, Annecchino F P, Crupi G. Regression of pulmonary fistulas in congenital heart disease after redirection of hepatic venous flow to the lungs.  Ann Thorac Surg. 2001;  72 909-911

1 This article was presented at the 52nd European Society for Cardiovascular Surgery Congress, Istanbul, 7 - 10 November 2003