Hostname: page-component-586b7cd67f-rdxmf Total loading time: 0 Render date: 2024-11-24T08:05:05.065Z Has data issue: false hasContentIssue false

Surgical revisions and mechanical support of the failing Fontan

Published online by Cambridge University Press:  09 January 2014

Stephanie Fuller*
Affiliation:
Perelman School of Medicine, The Cardiac Center, Division of Cardiothoracic Surgery, The Children's Hospital of Philadelphia, University of Pennsylvania, Philadelphia, Pennsylvania, United States of America
*
Correspondence to: S. Fuller, Perelman School of Medicine, The Cardiac Center, Division of Cardiothoracic Surgery, The Children's Hospital of Philadelphia, University of Pennsylvania, Philadelphia, PA 19104, United States of America. Tel: 215-590-2708; Fax: 215-590-2715; E-mail: fullers@email.chop.edu

Abstract

Despite its many anatomic modifications, Fontan failure remains prevalent in adult patients living with single-ventricle physiology. Although the Fontan operation has created a generation of survivors of complex single-ventricle disease, recognition of the limitation of this circulation is increasing. Most commonly, survivors suffer from ventricular dysfunction, protein-losing enteropathy, plastic bronchitis and arrhythmias. Although several medical therapies exist to palliate these long-term complications, surgical treatments are becoming increasingly useful, including the introduction of mechanical devices and cardiac transplantation. Currently, there are a variety of options available for mechanical support of the failing Fontan. Further developments in mechanical support technology will be required to tailor-make specific devices for this unique population. Owing to the fact that availability of donor hearts for transplantation is limited, mechanical support will play an increasingly important role in the management of complications of the Fontan circulation.

Type
Original Article
Copyright
Copyright © Cambridge University Press 2013 

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

References

1.Goldberg, DJ, Shaddy, RE, Ravishankar, C, Rychik, J. The failing fontan: etiology, diagnosis and management. Exp Rev Cardiovasc Ther 2011; 9: 785793.Google Scholar
2.Mertens, L, Hagler, DJ, Sauer, U, Somerville, J, Gewillig, M. Protein-losing enteropathy after the Fontan operation: an international multicenter study. PLE study group. J Thorac Cardiovasc Surg 1998; 115: 10631073.Google Scholar
3.Feldt, RH, D, DJ, Offord, KP, et al. Protein-losing enteropathy after the Fontan operation. J Thorac Cardiovasc Surg 1996; 112: 672680.Google Scholar
4.Petko, M, Myung, RJ, Wernovsky, G, et al. Surgical reinterventions following the Fontan procedure. Eur J Cardiothorac Surg 2003; 24: 255259.CrossRefGoogle ScholarPubMed
5.Cohen, MI, Wernovsky, G, Vetter, VL, et al. Sinus node function after a systematically staged Fontan procedure. Circulation 1998; 98: II352II358; discussion II358–II359.Google Scholar
6.Mavroudis, C, Deal, BJ, Backer, CL, et al. 111 Fontan conversions iwth arrhythmia surgery: surgical lesions and outcome. Ann Thorac Surg 2007; 84: 14571466.Google Scholar
7.Kogon, BE. Surgery for the failing Fontan. Prog Pediatr Cardiol 2012; 34: 2730.Google Scholar
8.Nathan, M, Baird, C, Fynn-Thompson, F, et al. Successful implantation of a Berlin heart biventricular assist device in a failing single ventricle. J Thorac Cardiovasc Surg 2006; 131: 14071408.CrossRefGoogle Scholar
9.Davies, RR, Sorabella, RA, Yang, JY, Mosca, RS, Chen, JM, Quaegebeur, JA. Outcomes after transplantation for “failed” Fontan: a single-institution experience. J Thorac Cardiovasc Surg 2012; 143: 11831192.Google Scholar
10.Kanter, KR, Mahle, WT, Vincent, RN, Berg, AM, Kogon, BE, Kirshbom, PM. Heart transplantation in children with a Fontan procedure. Ann Thorac Surg 2011; 91: 823830.Google Scholar
11.Davies, RR, Russo, MJ, Yang, JY, Quaegebeur, JA, Mosca, RS, Chen, JM. Listing and transplanting adults with congenital heart disease. Circulation 2011; 123: 759767.Google Scholar