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Technical modifications for transplant in the failing Fontan

Published online by Cambridge University Press:  19 November 2020

Juan-Miguel Gil-Jaurena Open the ORCID record for Juan-Miguel Gil-Jaurena [Opens in a new window] ,
Carlos Pardo ,
Ana Pita ,
Ramón Pérez-Caballero ,
José Zamorano ,
Manuela Camino ,
Nuria Gil-Villanueva ,
Eduardo Zataráin  and
Raquel Prieto
Juan-Miguel Gil-Jaurena*
Affiliation:
Pediatric Cardiac Surgery, Hospital Gregorio Marañón, Madrid, Spain Instituto de Investigación Sanitaria Gregorio Marañón, Madrid, Spain
Carlos Pardo
Affiliation:
Pediatric Cardiac Surgery, Hospital Gregorio Marañón, Madrid, Spain Instituto de Investigación Sanitaria Gregorio Marañón, Madrid, Spain
Ana Pita
Affiliation:
Pediatric Cardiac Surgery, Hospital Gregorio Marañón, Madrid, Spain Instituto de Investigación Sanitaria Gregorio Marañón, Madrid, Spain
Ramón Pérez-Caballero
Affiliation:
Pediatric Cardiac Surgery, Hospital Gregorio Marañón, Madrid, Spain Instituto de Investigación Sanitaria Gregorio Marañón, Madrid, Spain
José Zamorano
Affiliation:
Instituto de Investigación Sanitaria Gregorio Marañón, Madrid, Spain Perfusionist, Hospital Gregorio Marañón, Madrid, Spain
Manuela Camino
Affiliation:
Instituto de Investigación Sanitaria Gregorio Marañón, Madrid, Spain Pediatric Cardiology, Transplant Unit, Hospital Gregorio Marañón, Madrid, Spain
Nuria Gil-Villanueva
Affiliation:
Instituto de Investigación Sanitaria Gregorio Marañón, Madrid, Spain Pediatric Cardiology, Transplant Unit, Hospital Gregorio Marañón, Madrid, Spain
Eduardo Zataráin
Affiliation:
Instituto de Investigación Sanitaria Gregorio Marañón, Madrid, Spain Adult Cardiology, Hospital Gregorio Marañón and CIBER-CV Instituto de Salud Carlos III, Madrid, Spain
Raquel Prieto
Affiliation:
Instituto de Investigación Sanitaria Gregorio Marañón, Madrid, Spain Adult Cardiology, Hospital Gregorio Marañón and CIBER-CV Instituto de Salud Carlos III, Madrid, Spain
*
Author for correspondence: Juan-Miguel Gil-Jaurena, MD, Pediatric Cardiac Surgery, Hospital Gregorio Marañón, C/O’Donnell, No. 50, 28009 Madrid, Spain. Tel/Fax: +34 91 529 0041. E-mail: [email protected]
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Abstract

Introduction:

Heart transplant after Fontan completion poses a unique surgical challenge. Twenty patients are presented, stressing the technical hints performed in the five anastomoses to match the graft in the recipient.

Methods:

Data are collected from 20 Fontan patients between 2013 and 2019. Age (13 years), weight (37 kg.), and time interval between Fontan and transplant (7 years) are presented as median. Extracardiac conduit (size 18/20) was implanted in 15 patients, whereas atrio-pulmonary connection was performed in 4 and lateral tunnel in 1. Six patients developed protein-losing enteropathy. Seventeen stents had been previously deployed.

Results:

The five anastomoses underwent some changes. Left atrium once, aorta 9 times, superior vena cava 7 times, pulmonary branches 15 times, and inferior vena cava 12 times. Follow-up was complete for a median of 42 months (range 6–84). Two patients died. ECMO was needed in six cases for pulmonary hypertension. Four patients had collateral vessels occluded in the cath lab, and stents were placed in superior vena cava (1) and aorta (1) post-transplant. Protein-losing enteropathy was resolved in five patients. Interestingly, one patient was on a systemic assist device before transplant (Levitronix) and right assistance (ECMO) afterwards.

Conclusions:

Transplant in Fontan patients is actually challenging. Hints in every of the five proposed anastomoses must be anticipated, including stents removal. Extra tissue from the donor (innominate vein, aortic arch, and pericardium) is strongly advisable. ECMO for right ventricular dysfunction was needed in nearly one-third of the cases. Overall results can match other transplant cohorts.

Keywords

TransplantFontanChildren
Type
Original Article
Information
Cardiology in the Young , Volume 31 , Issue 3 , March 2021 , pp. 400 - 405
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Copyright
© The Author(s), 2020. Published by Cambridge University Press

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References

Fontan, F, Kirklin, J, Fernández, G, et al. Outcome after a perfect Fontan operation. Circulation 1990; 81: 1520–1536.CrossRefGoogle ScholarPubMed
Gentles, TL, Mayer, JE, Gauvreau, K, et al. Fontan operation in five hundred consecutive patients: factors influencing early and late outcome. J Thorac Cardiovasc Surg 1997; 114: 376–391.CrossRefGoogle ScholarPubMed
De Leval, MR. The Fontan circulation: what have we learned? What to expect? Pediatr Cardiol 1998; 19: 316–320.CrossRefGoogle ScholarPubMed
Freedom, RM, Hamilton, R, Yoo, SJ, et al. The Fontan procedure: analysis of cohorts and late complications. Cardiol Young 2000; 10: 307–331.CrossRefGoogle ScholarPubMed
Stewart, RD, Pasquali, SK, Jacobs, JP, et al. Contemporary Fontan operation: association between early outcome and type of cavopulmonary connection. Ann Thorac Surg 2012; 93: 1254–1260.CrossRefGoogle ScholarPubMed
Iyengar, AJ, Winlaw, DS, Galati, JC, et al. Trends in Fontan surgery and risk factors for early adverse outcomes after Fontan surgery: the Australia and New Zealand Fontan Registry experience. J Thorac Cardiovasc Surg 2014; 148: 566–575.CrossRefGoogle ScholarPubMed
Michielon, G, Parisi, F, di carlo, D, et al. Orthotopic heart transplantation for failing single ventricle physiology. Eur J Cardiothorac Surg 2003; 24: 502–510.CrossRefGoogle ScholarPubMed
Mitchell, MB, Campbell, DN, Bouceck, MM. Heart transplantation for the failing Fontan circulation. Pediatr Card Surg Ann Semin Thorac Cardiovasc Surg 2004; 7: 56–64.CrossRefGoogle ScholarPubMed
Jayakumar, KA, Addonizio, LJ, Kichuk-Chrisant, RM, et al. Cardiac transplantation after the Fontan or Glenn procedure. J Am Coll Cardiol 2004; 44: 2065–2072.CrossRefGoogle ScholarPubMed
Bernstein, D, Naftel, D, Chin, C, et al. Pediatric Heart Transplant Study. Outcome of listing for cardiac transplantation for failed Fontan: a multi-institutional study. Circulation 2006; 114: 273–280.CrossRefGoogle ScholarPubMed
Kanter, KR. Heart transplantation in children after a Fontan procedure: better than people think. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 2016; 19: 44–49.CrossRefGoogle ScholarPubMed
Backer, CL, Russell, HM, Pahl, E, et al. Heart transplantation in the failing Fontan. Ann Thorac Surg 2013; 96: 1413–1419.CrossRefGoogle ScholarPubMed
Michielon, G, vanMelle, JP, Wolff, D, et al. Favourable mid-term outcome after heart transplantation for late Fontan failure. Eur J Cardiothorac Surg 2015; 47: 665–671.CrossRefGoogle ScholarPubMed
Mauchley, DC, Mitchell, MB. Transplantation in the Fontan patient. Semin Thorac Cardiovasc Surg Pediatr Card Surg Ann 2015; 18: 7–16.CrossRefGoogle Scholar
Doty, DB, Renlund, DG, Caputo, GR, Burton, NA, Jones, KW. Cardiac transplantation in situs inversus. J Thorac Cardiovasc Surg 1990; 99: 493–499.CrossRefGoogle ScholarPubMed
Chartrand, C. Pediatric cardiac transplantation despite atrial and venous return anomalies. Ann Thorac Surg 1991; 52: 716–721.CrossRefGoogle ScholarPubMed
Menkis, AH, McKencie, FN, Novick, RJ, et al. The pediatric heart transplant group: expanding applicability of transplantation after multiple prior palliative procedures. Ann Thorac Surg 1991; 52: 722–726.CrossRefGoogle ScholarPubMed
Vouhé, PR, Tamisier, DR, le Bidois, J, et al. Pediatric cardiac transplantation for congenital heart defects: Surgical considerations and results. Ann Thorac Surg 1993; 56: 1239–1247.CrossRefGoogle ScholarPubMed
Hosseinpour, AR, Cullen, S, Tsang, V. Transplantation for adults with congenital heart disease. Eur J Cardiothorac Surg 2006; 30: 508–514.CrossRefGoogle ScholarPubMed
González-López, MT, Pérez-Caballero, R, Amorós, C, Zamorano, J, Pita, A, Gil-Jaurena, JM. Orthotopic transplantation in an adult patient with heterotaxy syndrome: surgical implications. J Card Surg 2015; 12: 910–912.CrossRefGoogle Scholar
Gil-Jaurena, JM, Pérez-Caballero, R, Pita, A, et al. Trasplante cardiaco en cardiopatías congénitas: peculiaridades técnicas. Cir Cardiov 2019; 26 (S1): 35–41.Google Scholar
Rychik, J, Atz, AM, Celermajer, DS, et al. Evaluation and management of the child and adult with Fontan: a scientific statement from the American Heart Association. Circulation 2019; 140: e234–e284.CrossRefGoogle Scholar
Mavroudis, C, Backer, CL, Deal, BJ, Johnsrude, C, Strasburger, J. Total cavopulmonary conversion and maze procedure for patients with failure of the Fontan operation. J Thorac Cardiovasc Surg 2001; 122: 863–871.CrossRefGoogle ScholarPubMed
Home, D, Conway, W, Rebeyka, IM, Buchholz, H. Mechanical circulatory support in univentricular hearts: current management. Semin Thorac Cardiovasc Surg Pediatr Card Surg Ann 2015; 18: 17–24.Google Scholar
Adachi, I, Burki, S, Fraser, CD Jr. Current status of pediatric ventricular assist device support. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 2017; 20: 2–8.CrossRefGoogle ScholarPubMed
Mitchell, MB, Campbell, DN, Ivy, D, et al. Evidence of pulmonary vascular disease after heart transplantation for Fontan circulation failure. J Thorac Cardiovasc Surg 2004; 128: 693–702.CrossRefGoogle ScholarPubMed
Hosseinpour, AR, González-Calle, A, Adsuar, A, et al. Surgical technique for heart transplantation: a strategy for congenital heart disease. Eur J Cardiothorac Surg 2013; 44: 598–604.CrossRefGoogle ScholarPubMed
Iyengar, AJ, Sharma, VJ, Weintraub, RG, et al. Surgical strategies to facilitate heart transplantation in children after failed univentricular palliations: the role of advanced intraoperative surgical preparation. Eur J Cardiothorac Surg 2014; 46: 480–485.CrossRefGoogle ScholarPubMed
Kanter, K, Mahle, WT, Vincent, RN, Berg, AM. Aortic complications after pediatric cardiac transplantation in patients with a previous Norwood reconstruction. Semin Thorac Cardiovasc Surg Pediatr Card Surg Ann 2011; 14: 24–28.CrossRefGoogle Scholar
Gil-Jaurena, JM, Zunzunegui, JL, Pérez-Caballero, R, et al. Surgical management of vascular stents in pediatric cardiac surgery: clues for a staged partnership. Pediatr Cardiol 2015; 36: 1685–1691.CrossRefGoogle ScholarPubMed
Krishnan, US, Lamour, LM, Hsu, DT, Kichuk, RM, Donnelly, CM, Addonizio, DJ. Management of aortopulmonary collaterals in children following cardiac transplantation for complex congenital heart disease. J Heart lung Transplant 2004; 22: 564–569.CrossRefGoogle Scholar
Murtuza, B, Hermuzi, A, Crossland, DS, et al. Impact of mode of failure and end-organ dysfunction on the survival of adult Fontan patients undergoing cardiac transplantation. Eur J Cardiothorac Surg 2017; 51: 135–141.CrossRefGoogle ScholarPubMed
Hernández, GA, Lemor, A, Clark, D, et al. Heart transplantation and in-hospital outcomes in adult congenital heart disease patients with Fontan: a decade nationwide analysis from 2004 to 2014. J Card Surg 2020; 35:603–608.CrossRefGoogle ScholarPubMed
Michielon, G, Carotti, A, Pongiglione, C, Cogo, A, Marino, B. Orthotopic heart transplantation in patients with univentricular physiology. Curr Cardiol Rev 2011; 7: 85–91.CrossRefGoogle ScholarPubMed
McCormick, AD, Schumacher, KR. Transplantation of the failing Fontan. Transl Pediatr 2019; 8: 290–301.CrossRefGoogle ScholarPubMed