Converting the Fontan Circulation: Challenges and Evolution
DOI:
https://doi.org/10.59958/hsf.6983Keywords:
Fontan circulation, Fontan conversion, total cavopulmonary connection, single ventricle physiologyAbstract
The Fontan operation, the final palliative step after a series of complex operations in patients with univentricular hearts, has undergone multiple modifications throughout the last decades, with the goal of finding the method which combines the optimal hemodynamic effects of the Fontan circulation with minimal long term side effects. An understanding of the operative evolution and subsequent side effects, as well as the management thereof seems imperative. Since its inception by Francis Fontan the, now obsolete, initial atriopulmonary connection has passed through several milestones into having now reached the era of total cavopulmonary connection. However, recently published results bring to light the new option of biventricular conversion which comes to challenge the management of Fontan patients as we know it. Currently, there is no consensus as to whether to continue with palliation in this challenging group of patients or proceed with heart transplantation. In this collective review, we provide a historic overview of the Fontan evolution as well as future insights, discussing the perspectives of options for patients with failing Fontan, including the latest addition of biventricular correction.
References
Fontan F, Baudet E. Surgical repair of tricuspid atresia. Thorax. 1971; 26: 240–248.
de Leval MR. The Fontan circulation: What have we learned? What to expect? Pediatric Cardiology. 1998; 19: 316–320.
de Leval MR, Kilner P, Gewillig M, Bull C. Total cavopulmonary connection: a logical alternative to atriopulmonary connection for complex Fontan operations. Experimental studies and early clinical experience. The Journal of Thoracic and Cardiovascular Surgery. 1988; 96: 682–695.
Mavroudis C, Backer CL, Deal BJ, Johnsrude CL. Fontan conversion to cavopulmonary connection and arrhythmia circuit cryoblation. The Journal of Thoracic and Cardiovascular Surgery. 1998; 115: 547–556.
Mavroudis C, Stulak JM, Ad N, Siegel A, Giamberti A, Harris L, et al. Prophylactic atrial arrhythmia surgical procedures with congenital heart operations: review and recommendations. The Annals of Thoracic Surgery. 2015; 99: 352–359.
Mavroudis C, Backer CL, Deal BJ, Johnsrude C, Strasburger J. Total cavopulmonary conversion and maze procedure for patients with failure of the Fontan operation. The Journal of Thoracic and Cardiovascular Surgery. 2001; 122: 863–871.
Alexander PMA, Swager A, Lee KJ, Shipp A, Konstantinov IE, Wilkinson JL, et al. Paediatric heart transplantation in Australia comes of age: 21 years of experience in a national centre. Internal Medicine Journal. 2014; 44: 1223–1231.
Backer CL, Russell HM, Pahl E, Mongé MC, Gambetta K, Kindel SJ, et al. Heart transplantation for the failing Fontan. The Annals of Thoracic Surgery. 2013; 96: 1413–1419.
Doulamis IP, Marathe SP, Piekarski B, Beroukhim RS, Marx GR, Del Nido PJ, et al. Biventricular conversion after Fontan completion: A preliminary experience. The Journal of Thoracic and Cardiovascular Surgery. 2022; 163: 1211–1223.
Kumar SR, Detterich J. Considerations for Biventricular Conversion of Fontan Circulation. Seminars in Thoracic and Cardiovascular Surgery. Pediatric Cardiac Surgery Annual. 2022; 25: 11–18.
Deal BJ, Jacobs ML. Management of the failing Fontan circulation. Heart (British Cardiac Society). 2012; 98: 1098–1104.
Atz AM, Zak V, Mahony L, Uzark K, Shrader P, Gallagher D, et al. Survival data and predictors of functional outcome an average of 15 years after the Fontan procedure: the pediatric heart network Fontan cohort. Congenital Heart Disease. 2015; 10: E30–E42.
d'Udekem Y, Iyengar AJ, Galati JC, Forsdick V, Weintraub RG, Wheaton GR, et al. Redefining expectations of long-term survival after the Fontan procedure: twenty-five years of follow-up from the entire population of Australia and New Zealand. Circulation. 2014; 130: S32–S38.
Diller GP, Giardini A, Dimopoulos K, Gargiulo G, Müller J, Derrick G, et al. Predictors of morbidity and mortality in contemporary Fontan patients: results from a multicenter study including cardiopulmonary exercise testing in 321 patients. European Heart Journal. 2010; 31: 3073–3083.
Mavroudis C, Deal BJ. Fontan Conversion: Literature Review and Lessons Learned Over 20 Years. 2016. Available at: https://journals.sagepub.com/doi/abs/10.1177/2150135115623960 (Accessed: 19 September 2023).
Balaji S, Johnson TB, Sade RM, Case CL, Gillette PC. Management of atrial flutter after the Fontan procedure. Journal of the American College of Cardiology. 1994; 23: 1209–1215.
Case CL, Gillette PC, Zeigler V, Sade RM. Problems with permanent atrial pacing in the Fontan patient. Pacing and Clinical Electrophysiology: PACE. 1989; 12: 92–96.
Deal BJ, Mavroudis C, Backer CL, Johnsrude CL, Rocchini AP. Impact of arrhythmia circuit cryoablation during Fontan conversion for refractory atrial tachycardia. The American Journal of Cardiology. 1999; 83: 563–568.
Backer CL, Mavroudis C. 149 Fontan Conversions. Methodist DeBakey Cardiovascular Journal. 2019; 15: 105–110.
Erek E, Yalcinbas YK, Sarioglu A, Sarioglu T. Current management of late failure after classic Fontan modifications: Fontan conversion. The Thoracic and Cardiovascular Surgeon. 2006; 54: 21–25.
Mavroudis C, Deal BJ, Backer CL. Surgical Therapy of Cardiac Arrhythmias. Pediatric Cardiac Surgery (pp. 769–812). John Wiley & Sons, Ltd., West Sussex, PO19 8SQ, UK. 2013.
Mavroudis C, Backer CL, Deal BJ. Late reoperations for Fontan patients: state of the art invited review. European Journal of Cardio-thoracic Surgery: Official Journal of the European Association for Cardio-thoracic Surgery. 2008; 34: 1034–1040.
Mavroudis C, Deal BJ, Backer CL, Stewart RD, Franklin WH, Tsao S, et al. J. Maxwell Chamberlain Memorial Paper for congenital heart surgery. 111 Fontan conversions with arrhythmia surgery: surgical lessons and outcomes. The Annals of Thoracic Surgery. 2007; 84: 1457–1457–65; discussion 1465–1466.
Poh CL, Cochrane A, Galati JC, Bullock A, Celermajer DS, Gentles T, et al. Ten-year outcomes of Fontan conversion in Australia and New Zealand demonstrate the superiority of a strategy of early conversion. European Journal of Cardio-thoracic Surgery: Official Journal of the European Association for Cardio-thoracic Surgery. 2016; 49: 530–5; discussion 535.
Terada T, Sakurai H, Nonaka T, Sakurai T, Sugiura J, Taneichi T, et al. Surgical outcome of Fontan conversion and arrhythmia surgery: Need a pacemaker? Asian Cardiovascular & Thoracic Annals. 2014; 22: 682–686.
Backer CL, Costello JM, Deal BJ. Fontan conversion: guidelines from Down Under. European Journal of Cardio-thoracic Surgery: Official Journal of the European Association for Cardio-thoracic Surgery. 2016; 49: 536–537.
Betts TR, Roberts PR, Allen SA, Salmon AP, Keeton BR, Haw MP, et al. Electrophysiological Mapping and Ablation of Intra-Atrial Reentry Tachycardia After Fontan Surgery With the Use of a Noncontact Mapping System. 2000. Available at: https://www.ahajournals.org/doi/full/10.1161/01.CIR.102.4.419 (Accessed: 20 September 2023).
Correa R, Walsh EP, Alexander ME, Mah DY, Cecchin F, Abrams DJ, et al. Transbaffle mapping and ablation for atrial tachycardias after mustard, senning, or Fontan operations. Journal of the American Heart Association. 2013; 2: e000325.
Padalino MA, Ponzoni M, Castaldi B, Leoni L, Chemello L, Toscano G, et al. Surgical management of failing Fontan circulation: results from 30 cases with 285 patient-years follow-up. European Journal of Cardio-thoracic Surgery: Official Journal of the European Association for Cardio-thoracic Surgery. 2022; 61: 338–345.
Rajab TK, Jaggers J. Transplantation in Fontan failure: The final stage. JTCVS Open. 2020; 3: 154–159.
Kramer P, Ovroutski S. Fontan conversion for Fontan failure: lessons from a vanishing craft. European Journal of Cardio-thoracic Surgery: Official Journal of the European Association for Cardio-thoracic Surgery. 2022; 61: 346–347.
Konstantinov IE, Schulz A, Buratto E. Heart transplantation after Fontan operation. JTCVS Techniques. 2022; 13: 182–191.
Michielon G, van Melle JP, Wolff D, Di Carlo D, Jacobs JP, Mattila IP, et al. Favourable mid-term outcome after heart transplantation for late Fontan failure. European Journal of Cardio-thoracic Surgery: Official Journal of the European Association for Cardio-thoracic Surgery. 2015; 47: 665–671.
McCormick AD, Schumacher KR. Transplantation of the failing Fontan. Translational Pediatrics. 2019; 8: 290–301.
Deal BJ, Costello JM, Webster G, Tsao S, Backer CL, Mavroudis C. Intermediate-Term Outcome of 140 Consecutive Fontan Conversions with Arrhythmia Operations. The Annals of Thoracic Surgery. 2016; 101: 717–724.
Said SM, Burkhart HM, Schaff HV, Cetta F, Driscoll DJ, Li Z, et al. Fontan conversion: identifying the high-risk patient. The Annals of Thoracic Surgery. 2014; 97: 2115–2115–21; discussion 2121–2.
Riggs KW, Broderick JT, Chin C, Iii RB, Zafar F, Morales DL. Cardiac Transplantation in Fontan Patients Has Equally Excellent Results as Biventricular Congenital Heart Disease Patients. The Journal of Heart and Lung Transplantation. 2019; 38: S115.
Carlo WF, Villa CR, Lal AK, Morales DL. Ventricular assist device use in single ventricle congenital heart disease. Pediatric Transplantation. 2017; 21: 10.1111/petr.13031.
Frazier OH, Gregoric ID, Messner GN. Total circulatory support with an LVAD in an adolescent with a previous Fontan procedure. Texas Heart Institute Journal. 2005; 32: 402–404.
Hoganson DM, Boston US, Gazit AZ, Canter CE, Eghtesady P. Successful bridge through transplantation with berlin heart ventricular assist device in a child with failing fontan. The Annals of Thoracic Surgery. 2015; 99: 707–709.
Niebler RA, Ghanayem NS, Shah TK, De La Rosa Bobke A, Zangwill S, Brosig C, et al. Use of a HeartWare ventricular assist device in a patient with failed Fontan circulation. The Annals of Thoracic Surgery. 2014; 97: e115–e116.
Cedars A, Kutty S, Danford D, Schumacher K, ACTION Learning Network Investigators, Auerbach SR, et al. Systemic ventricular assist device support in Fontan patients: A report by ACTION. The Journal of Heart and Lung Transplantation: the Official Publication of the International Society for Heart Transplantation. 2021; 40: 368–376.
Jayakumar KA, Addonizio LJ, Kichuk-Chrisant MR, Galantowicz ME, Lamour JM, Quaegebeur JM, et al. Cardiac transplantation after the Fontan or Glenn procedure. Journal of the American College of Cardiology. 2004; 44: 2065–2072.
Delius RE, Rademecker MA, de Leval MR, Elliott MJ, Stark J. Is a high-risk biventricular repair always preferable to conversion to a single ventricle repair? The Journal of Thoracic and Cardiovascular Surgery. 1996; 112: 1561–1561–8; discussion 1568–9.
Oh NA, Doulamis IP, Guariento A, Piekarski B, Marx GR, Del Nido PJ, et al. Staged ventricular recruitment and biventricular conversion following single-ventricle palliation in unbalanced atrioventricular canal defects. JTCVS Open. 2022; 13: 278–291.
