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Current Cardiology Reviews

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ISSN (Print): 1573-403X
ISSN (Online): 1875-6557

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General Review Article

Current Treatment Options for the Failing Fontan Circulation

Author(s): Bart W. Driesen, Michiel Voskuil and Heynric B. Grotenhuis*

Volume 18, Issue 4, 2022

Published on: 17 March, 2022

Article ID: e060122200067 Pages: 15

DOI: 10.2174/1573403X18666220106114518

Price: $65

Abstract

The Fontan operation was introduced in 1968. For congenital malformations, where biventricular repair is unsuitable, the Fontan procedure has provided a long-term palliation strategy with improved outcomes compared to the initially developed procedures. Despite these improvements, several complications merely due to a failing Fontan circulation, including myocardial dysfunction, arrhythmias, increased pulmonary vascular resistance, protein-losing enteropathy, hepatic dysfunction, plastic bronchitis, and thrombo-embolism, may occur, thereby limiting the life-expectancy in this patient cohort. This review provides an overview of the most common complications of Fontan circulation and the currently available treatment options.

Keywords: Pediatrics, heart defects, congenital, fontan, complications, treatment.

Graphical Abstract

[1]
Fontan F, Baudet E. Surgical repair of tricuspid atresia. Thorax 1971; 26(3): 240-8.
[http://dx.doi.org/10.1136/thx.26.3.240] [PMID: 5089489]
[2]
Bull C, de Leval MR, Stark J, Taylor JF, Macartney FJ. Use of a subpulmonary ventricular chamber in the Fontan circulation. J Thorac Cardiovasc Surg 1983; 85(1): 21-31.
[http://dx.doi.org/10.1016/S0022-5223(19)38895-6] [PMID: 6848884]
[3]
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. J Thorac Cardiovasc Surg 1988; 96(5): 682-95.
[http://dx.doi.org/10.1016/S0022-5223(19)35174-8] [PMID: 3184963]
[4]
Marcelletti C, Corno A, Giannico S, Marino B. Inferior vena cava-pulmonary artery extracardiac conduit. A new form of right heart bypass. J Thorac Cardiovasc Surg 1990; 100(2): 228-32.
[http://dx.doi.org/10.1016/S0022-5223(19)35562-X] [PMID: 2143549]
[5]
d’Udekem Y, Iyengar AJ, Galati JC, 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(11 Suppl. 1): S32-8.
[http://dx.doi.org/10.1161/CIRCULATIONAHA.113.007764] [PMID: 25200053]
[6]
Poh CL, d’Udekem Y. Life after surviving Fontan surgery: A meta-analysis of the incidence and predictors of late death. Heart Lung Circ 2018; 27(5): 552-9.
[http://dx.doi.org/10.1016/j.hlc.2017.11.007] [PMID: 29402692]
[7]
Dennis M, Zannino D, du Plessis K, et al. Clinical outcomes in adolescents and adults after the Fontan procedure. J Am Coll Cardiol 2018; 71(9): 1009-17.
[http://dx.doi.org/10.1016/j.jacc.2017.12.054] [PMID: 29495980]
[8]
Gewillig M, Brown SC. The Fontan circulation after 45 years: Update in physiology. Heart 2016; 102(14): 1081-6.
[http://dx.doi.org/10.1136/heartjnl-2015-307467] [PMID: 27220691]
[9]
van der Ven JPG, van den Bosch E, Bogers AJCC, Helbing WA. State of the art of the Fontan strategy for treatment of univentricular heart disease. F1000 Res 2018; 7: F1000.
[http://dx.doi.org/10.12688/f1000research.13792.1] [PMID: 30002816]
[10]
Al-Shawk M, Banjoko A, Axiaq A, Amin K, Harky A. Perioperative and long-term management of Fontan patients. Cardiol Young 2021; 31(5): 775-85.
[http://dx.doi.org/10.1017/S1047951120004618] [PMID: 33431102]
[11]
Egbe A, Khan AR, Al-Otaibi M, Said SM, Connolly HM. Outcomes of hospitalization in adults with Fontan palliation: The Mayo clinic experience. Am Heart J 2018; 198: 115-22.
[http://dx.doi.org/10.1016/j.ahj.2017.12.012] [PMID: 29653632]
[12]
Kantor PF, Redington AN. Pathophysiology and management of heart failure in repaired congenital heart disease. Heart Fail Clin 2010; 6(4): 497-506, ix.
[http://dx.doi.org/10.1016/j.hfc.2010.06.002] [PMID: 20869649]
[13]
Opina AD, Franklin WJ. Management of heart failure in adult congenital heart disease. Prog Cardiovasc Dis 2018; 61(3-4): 308-13.
[http://dx.doi.org/10.1016/j.pcad.2018.08.003] [PMID: 30107184]
[14]
Anderson PA, Sleeper LA, Mahony L, et al. Contemporary outcomes after the Fontan procedure: A pediatric heart network multicenter study. J Am Coll Cardiol 2008; 52(2): 85-98.
[http://dx.doi.org/10.1016/j.jacc.2008.01.074] [PMID: 18598886]
[15]
Rychik J, Atz AM, Celermajer DS, et al. Evaluation and management of the child and adult with Fontan circulation: A scientific statement from the American heart association. Circulation 2019; CIR0000000000000696.
[http://dx.doi.org/10.1161/CIR.0000000000000696] [PMID: 31256636]
[16]
Alsaied T, Bokma JP, Engel ME, et al. Predicting long-term mortality after Fontan procedures: A risk score based on 6707 patients from 28 studies. Congenit Heart Dis 2017; 12(4): 393-8.
[http://dx.doi.org/10.1111/chd.12468] [PMID: 28480627]
[17]
Honjo O, Mertens L, Van Arsdell GS. Atrioventricular valve repair in patients with single-ventricle physiology: Mechanisms, techniques of repair, and clinical outcomes. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 2011; 14(1): 75-84.
[http://dx.doi.org/10.1053/j.pcsu.2011.02.001] [PMID: 21444052]
[18]
Kotani Y, Chetan D, Atlin CR, et al. Longevity and durability of atrioventricular valve repair in single-ventricle patients. Ann Thorac Surg 2012; 94(6): 2061-9.
[http://dx.doi.org/10.1016/j.athoracsur.2012.04.048] [PMID: 22877640]
[19]
Rathod RH, Prakash A, Powell AJ, Geva T. Myocardial fibrosis identified by cardiac magnetic resonance late gadolinium enhancement is associated with adverse ventricular mechanics and ventricular tachycardia late after Fontan operation. J Am Coll Cardiol 2010; 55(16): 1721-8.
[http://dx.doi.org/10.1016/j.jacc.2009.12.036] [PMID: 20394877]
[20]
Kato A, Riesenkampff E, Yim D, Yoo SJ, Seed M, Grosse-Wortmann L. Pediatric Fontan patients are at risk for myocardial fibrotic remodeling and dysfunction. Int J Cardiol 2017; 240: 172-7.
[http://dx.doi.org/10.1016/j.ijcard.2017.04.073] [PMID: 28461021]
[21]
Penny DJ, Rigby ML, Redington AN. Abnormal patterns of intraventricular flow and diastolic filling after the Fontan operation: Evidence for incoordinate ventricular wall motion. Br Heart J 1991; 66(5): 375-8.
[http://dx.doi.org/10.1136/hrt.66.5.375] [PMID: 1747299]
[22]
Brida M, et al. Pharmacological therapy in adult congenital heart disease: growing need, yet limited evidence. Eur Heart J 2018; 40(13): 1049-56.
[http://dx.doi.org/10.1093/eurheartj/ehy480] [PMID: 30137263]
[23]
Hebson CL, McCabe NM, Elder RW, et al. Hemodynamic phenotype of the failing Fontan in an adult population. Am J Cardiol 2013; 112(12): 1943-7.
[http://dx.doi.org/10.1016/j.amjcard.2013.08.023] [PMID: 24075283]
[24]
Mahle WT, Wang A, Quyyumi AA, McConnell ME, Book WM. Impact of spironolactone on endothelial function in patients with single ventricle heart. Congenit Heart Dis 2009; 4(1): 12-6.
[http://dx.doi.org/10.1111/j.1747-0803.2008.00240.x] [PMID: 19207397]
[25]
Ishibashi N, Park IS, Waragai T, et al. Effect of carvedilol on heart failure in patients with a functionally univentricular heart. Circ J 2011; 75(6): 1394-9.
[http://dx.doi.org/10.1253/circj.CJ-10-0845] [PMID: 21436593]
[26]
Kouatli AA, Garcia JA, Zellers TM, Weinstein EM, Mahony L. Enalapril does not enhance exercise capacity in patients after Fontan procedure. Circulation 1997; 96(5): 1507-12.
[http://dx.doi.org/10.1161/01.CIR.96.5.1507] [PMID: 9315539]
[27]
Yim DL, Jones BO, Alexander PM, d’Udekem Y, Cheung MM. Effect of anti-heart failure therapy on diastolic function in children with single-ventricle circulations. Cardiol Young 2015; 25(7): 1293-9.
[http://dx.doi.org/10.1017/S1047951114002376] [PMID: 25739738]
[28]
Beghetti M. Fontan and the pulmonary circulation: A potential role for new pulmonary hypertension therapies. Heart 2010; 96(12): 911-6.
[http://dx.doi.org/10.1136/hrt.2010.193912] [PMID: 20538665]
[29]
Appadurai V, Thoreau J, Malpas T, Nicolae M. Sacubitril/valsartan in adult congenital heart disease patients with chronic heart failure - a single centre case series and call for an international registry. Heart Lung Circ 2020; 29(1): 137-41.
[http://dx.doi.org/10.1016/j.hlc.2018.12.003] [PMID: 30686641]
[30]
Appadurai V, Nicolae M, Aboulhosn J, Lluri G. The protocol and rationale for the efficacy aNd ToleRability of sacUbitril-valSarTan in adult congenital heart disease patients with heart failure (ENTRUST ACHD HF) registry. Int J Cardiol Congenital Heart Disease 2021; 3: 100096.
[http://dx.doi.org/10.1016/j.ijcchd.2021.100096]
[31]
Hoffman TM, Wernovsky G, Atz AM, et al. Efficacy and safety of milrinone in preventing low cardiac output syndrome in infants and children after corrective surgery for congenital heart disease. Circulation 2003; 107(7): 996-1002.
[http://dx.doi.org/10.1161/01.CIR.0000051365.81920.28] [PMID: 12600913]
[32]
Ghanayem NS, Berger S, Tweddell JS. Medical management of the failing Fontan. Pediatr Cardiol 2007; 28(6): 465-71.
[http://dx.doi.org/10.1007/s00246-007-9007-0] [PMID: 17763892]
[33]
Barber BJ, Batra AS, Burch GH, et al. Acute hemodynamic effects of pacing in patients with Fontan physiology: A prospective study. J Am Coll Cardiol 2005; 46(10): 1937-42.
[http://dx.doi.org/10.1016/j.jacc.2005.07.045] [PMID: 16286183]
[34]
Estner HL, Kolb C, Schmitt C, et al. Long-term transvenous AV-sequential pacing in a failing atriopulmonary Fontan patient. Int J Cardiol 2008; 127(2): e93-5.
[http://dx.doi.org/10.1016/j.ijcard.2007.04.085] [PMID: 17764764]
[35]
Lopez JA. Transvenous right atrial and left ventricular pacing after the Fontan operation: Long-term hemodynamic and electrophysiologic benefit of early atrioventricular resynchronization. Tex Heart Inst J 2007; 34(1): 98-101.
[PMID: 17420803]
[36]
Enomoto Y, Aoki M, Nakamura Y, Hagino I, Fujiwara T, Nakajima H. Successful Fontan completion after cardiac resynchronization therapy. Circulation 2012; 125(19): e655-8.
[http://dx.doi.org/10.1161/CIRCULATIONAHA.111.070979] [PMID: 22586294]
[37]
Dodge-Khatami A, Rahn M, Prêtre R, Bauersfeld U. Dual chamber epicardial pacing for the failing atriopulmonary Fontan patient. Ann Thorac Surg 2005; 80(4): 1440-4.
[http://dx.doi.org/10.1016/j.athoracsur.2005.03.128] [PMID: 16181884]
[38]
Fishberger SB, Wernovsky G, Gentles TL, et al. Long-term outcome in patients with pacemakers following the Fontan operation. Am J Cardiol 1996; 77(10): 887-9.
[http://dx.doi.org/10.1016/S0002-9149(97)89191-6] [PMID: 8623749]
[39]
Umamaheshwar KL, Singh AS, Sivakumar K. Endocardial transvenous pacing in patients with surgically palliated univentricular hearts: A review on different techniques, problems and management. Indian Pacing Electrophysiol J 2019; 19(1): 15-22.
[http://dx.doi.org/10.1016/j.ipej.2018.11.013] [PMID: 30508590]
[40]
Egbe AC, Huntley GD, Connolly HM, et al. Outcomes of cardiac pacing in adult patients after a Fontan operation. Am Heart J 2017; 194: 92-8.
[http://dx.doi.org/10.1016/j.ahj.2017.08.019] [PMID: 29223440]
[41]
Udink Ten Cate FEA, Trieschmann U, Germund I, et al. Stenting the Fontan pathway in paediatric patients with obstructed extracardiac conduits. Heart 2017; 103(14): 1111-6.
[http://dx.doi.org/10.1136/heartjnl-2016-310511] [PMID: 28237969]
[42]
Rijnberg FM, Hazekamp MG, Wentzel JJ, et al. Energetics of blood flow in cardiovascular disease: Concept and clinical implications of adverse energetics in patients with a Fontan circulation. Circulation 2018; 137(22): 2393-407.
[http://dx.doi.org/10.1161/CIRCULATIONAHA.117.033359] [PMID: 29844073]
[43]
Hagler DJ, Miranda WR, Haggerty BJ, et al. Fate of the Fontan connection: Mechanisms of stenosis and management. Congenit Heart Dis 2019; 14(4): 571-81.
[http://dx.doi.org/10.1111/chd.12757] [PMID: 30801968]
[44]
Mets JM, Bergersen L, Mayer JE Jr, Marshall AC, McElhinney DB. Outcomes of stent implantation for obstruction of intracardiac lateral tunnel Fontan pathways. Circ Cardiovasc Interv 2013; 6(1): 92-100.
[http://dx.doi.org/10.1161/CIRCINTERVENTIONS.112.000099] [PMID: 23403383]
[45]
Griselli M, Sinha R, Jang S, Perri G, Adachi I. Mechanical circulatory support for single ventricle failure. Front Cardiovasc Med 2018; 5: 115.
[http://dx.doi.org/10.3389/fcvm.2018.00115] [PMID: 30211172]
[46]
Kanani M, Hsia TY. Options for the failing ventricle in pediatric heart disease. Curr Cardiol Rep 2013; 15(10): 404.
[http://dx.doi.org/10.1007/s11886-013-0404-0] [PMID: 24000116]
[47]
Chen JM, Richmond ME, Charette K, et al. A decade of pediatric mechanical circulatory support before and after cardiac transplantation. J Thorac Cardiovasc Surg 2012; 143(2): 344-51.
[http://dx.doi.org/10.1016/j.jtcvs.2011.10.072] [PMID: 22143101]
[48]
Cohen G, Permut L. Decision making for mechanical cardiac assist in pediatric cardiac surgery. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 2005; 41-50.
[http://dx.doi.org/10.1053/j.pcsu.2005.02.004] [PMID: 15818357]
[49]
Cedars A, Vanderpluym C, Koehl D, Cantor R, Kutty S, Kirklin JK. An interagency registry for mechanically assisted circulatory support (INTERMACS) analysis of hospitalization, functional status, and mortality after mechanical circulatory support in adults with congenital heart disease. J Heart Lung Transplant 2018; 37(5): 619-30.
[http://dx.doi.org/10.1016/j.healun.2017.11.010] [PMID: 29198868]
[50]
Weinstein S, Bello R, Pizarro C, et al. The use of the Berlin heart EXCOR in patients with functional single ventricle. J Thorac Cardiovasc Surg 2014; 147(2): 697-704.
[http://dx.doi.org/10.1016/j.jtcvs.2013.10.030] [PMID: 24290716]
[51]
Arnaoutakis GJ, Blitzer D, Fuller S, et al. Mechanical circulatory support as bridge to transplantation for the failing single ventricle. Ann Thorac Surg 2017; 103(1): 193-7.
[http://dx.doi.org/10.1016/j.athoracsur.2016.05.015] [PMID: 27424467]
[52]
Buratto E, Shi WY, Ye XT, Konstantinov IE. Ventricular assist devices for the failing univentricular circulation. Expert Rev Med Devices 2017; 14(6): 449-59.
[http://dx.doi.org/10.1080/17434440.2017.1332523] [PMID: 28532240]
[53]
Rossano JW, Goldberg DJ, Fuller S, Ravishankar C, Montenegro LM, Gaynor JW. Successful use of the total artificial heart in the failing Fontan circulation. Ann Thorac Surg 2014; 97(4): 1438-40.
[http://dx.doi.org/10.1016/j.athoracsur.2013.06.120] [PMID: 24694426]
[54]
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(6): 1407-8.
[http://dx.doi.org/10.1016/j.jtcvs.2006.02.015] [PMID: 16733184]
[55]
Valeske K, Yerebakan C, Mueller M, Akintuerk H. Urgent implantation of the Berlin heart excor biventricular assist device as a total artificial heart in a patient with single ventricle circulation. J Thorac Cardiovasc Surg 2014; 147(5): 1712-4.
[http://dx.doi.org/10.1016/j.jtcvs.2014.01.012] [PMID: 24521952]
[56]
de Groot NMS, Bogers AJJC. Development of tachyarrhythmias late after the Fontan procedure: The role of ablative therapy. Card Electrophysiol Clin 2017; 9(2): 273-84.
[http://dx.doi.org/10.1016/j.ccep.2017.02.009] [PMID: 28457241]
[57]
Alexander ME. Pushing back the cliff (but there probably still is a cliff): Arrhythmia, late outcome, and uncertainty in Fontan patients. J Thorac Cardiovasc Surg 2016; 152(5): 1364-5.
[http://dx.doi.org/10.1016/j.jtcvs.2016.08.030] [PMID: 27650006]
[58]
Fishberger SB, Wernovsky G, Gentles TL, et al. Factors that influence the development of atrial flutter after the Fontan operation. J Thorac Cardiovasc Surg 1997; 113(1): 80-6.
[http://dx.doi.org/10.1016/S0022-5223(97)70402-1] [PMID: 9011705]
[59]
Weipert J, Noebauer C, Schreiber C, et al. Occurrence and management of atrial arrhythmia after long-term Fontan circulation. J Thorac Cardiovasc Surg 2004; 127(2): 457-64.
[http://dx.doi.org/10.1016/j.jtcvs.2003.08.054] [PMID: 14762355]
[60]
Giannakoulas G, Dimopoulos K, Yuksel S, et al. Atrial tachyarrhythmias late after Fontan operation are related to increase in mortality and hospitalization. Int J Cardiol 2012; 157(2): 221-6.
[http://dx.doi.org/10.1016/j.ijcard.2010.12.049] [PMID: 21196055]
[61]
Song MK, Bae EJ, Kwon BS, et al. Intra-atrial reentrant tachycardia in adult patients after Fontan operation. Int J Cardiol 2015; 187: 157-63.
[http://dx.doi.org/10.1016/j.ijcard.2015.03.157] [PMID: 25828344]
[62]
Pundi KN, Pundi KN, Johnson JN, et al. Sudden cardiac death and late arrhythmias after the Fontan operation. Congenit Heart Dis 2017; 12(1): 17-23.
[http://dx.doi.org/10.1111/chd.12401] [PMID: 27545004]
[63]
Quinton E, Nightingale P, Hudsmith L, et al. Prevalence of atrial tachyarrhythmia in adults after Fontan operation. Heart 2015; 101(20): 1672-7.
[http://dx.doi.org/10.1136/heartjnl-2015-307514] [PMID: 26289423]
[64]
Carins TA, Shi WY, Iyengar AJ, et al. Long-term outcomes after first-onset arrhythmia in Fontan physiology. J Thorac Cardiovasc Surg 2016; 152(5): 1355-1363.e1.
[http://dx.doi.org/10.1016/j.jtcvs.2016.07.073] [PMID: 27751239]
[65]
Egbe AC, Connolly HM, Khan AR, et al. Outcomes in adult Fontan patients with atrial tachyarrhythmias. Am Heart J 2017; 186: 12-20.
[http://dx.doi.org/10.1016/j.ahj.2016.12.015] [PMID: 28454826]
[66]
Fujita S, Takahashi K, Takeuchi D, et al. Management of late atrial tachyarrhythmia long after Fontan operation. J Cardiol 2009; 53(3): 410-6.
[http://dx.doi.org/10.1016/j.jjcc.2009.01.009] [PMID: 19477384]
[67]
Egbe AC, Connolly HM, Niaz T, McLeod CJ. Outcome of direct current cardioversion for atrial arrhythmia in adult Fontan patients. Int J Cardiol 2016; 208: 115-9.
[http://dx.doi.org/10.1016/j.ijcard.2016.01.209] [PMID: 26849685]
[68]
Cohen MI, Wernovsky G, Vetter VL, et al. Sinus node function after a systematically staged Fontan procedure. Circulation 1998; 98(19 Suppl.): II352-8.
[PMID: 9852926]
[69]
Dilawar M, Bradley SM, Saul JP, Stroud MR, Balaji S. Sinus node dysfunction after intraatrial lateral tunnel and extracardiac conduit fontan procedures. Pediatr Cardiol 2003; 24(3): 284-8.
[http://dx.doi.org/10.1007/s00246-002-0238-9] [PMID: 12522657]
[70]
Bossers SS, Duppen N, Kapusta L, et al. Comprehensive rhythm evaluation in a large contemporary Fontan population. Eur J Cardiothorac Surg 2015; 48(6): 833-40.
[http://dx.doi.org/10.1093/ejcts/ezu548] [PMID: 25602059]
[71]
Tsao S, Deal BJ, Backer CL, Ward K, Franklin WH, Mavroudis C. Device management of arrhythmias after Fontan conversion. J Thorac Cardiovasc Surg 2009; 138(4): 937-40.
[http://dx.doi.org/10.1016/j.jtcvs.2008.11.066] [PMID: 19660377]
[72]
Kramer CC, Maldonado JR, Olson MD, Gingerich JC, Ochoa LA, Law IH. Safety and efficacy of atrial antitachycardia pacing in congenital heart disease. Heart Rhythm 2018; 15(4): 543-7.
[http://dx.doi.org/10.1016/j.hrthm.2017.12.016] [PMID: 29246827]
[73]
Yap SC, Harris L, Downar E, Nanthakumar K, Silversides CK, Chauhan VS. Evolving electroanatomic substrate and intra-atrial reentrant tachycardia late after Fontan surgery. J Cardiovasc Electrophysiol 2012; 23(4): 339-45.
[http://dx.doi.org/10.1111/j.1540-8167.2011.02202.x] [PMID: 22035149]
[74]
Yap SC, Harris L, Silversides CK, Downar E, Chauhan VS. Outcome of intra-atrial re-entrant tachycardia catheter ablation in adults with congenital heart disease: Negative impact of age and complex atrial surgery. J Am Coll Cardiol 2010; 56(19): 1589-96.
[http://dx.doi.org/10.1016/j.jacc.2010.04.061] [PMID: 21029876]
[75]
Moore JP, Shannon KM, Fish FA, et al. Catheter ablation of supraventricular tachyarrhythmia after extracardiac Fontan surgery. Heart Rhythm 2016; 13(9): 1891-7.
[http://dx.doi.org/10.1016/j.hrthm.2016.05.019] [PMID: 27236028]
[76]
Sridhar A, Giamberti A, Foresti S, et al. Fontan conversion with concomitant arrhythmia surgery for the failing atriopulmonary connections: Mid-term results from a single centre. Cardiol Young 2011; 21(6): 665-9.
[http://dx.doi.org/10.1017/S1047951111000643] [PMID: 21615976]
[77]
Mavroudis C, Deal BJ. Fontan conversion: Literature review and lessons learned over 20 years. World J Pediatr Congenit Heart Surg 2016; 7(2): 192-8.
[http://dx.doi.org/10.1177/2150135115623960] [PMID: 26957403]
[78]
Mavroudis C, Deal BJ, Backer CL, et al. J. Maxwell Chamberlain memorial paper for congenital heart surgery. 111 Fontan conversions with arrhythmia surgery: Surgical lessons and outcomes. Ann Thorac Surg 2007; 84(5): 1457-65.
[http://dx.doi.org/10.1016/j.athoracsur.2007.06.079] [PMID: 17954046]
[79]
Brida M, Grbcic S, Holbein C, Veldtman GR. Bridging the psychological issues of living with the Fontan circulation. Int J Cardiol 2018; 260: 72-3.
[http://dx.doi.org/10.1016/j.ijcard.2018.02.105] [PMID: 29622457]
[80]
Henaine R, Vergnat M, Bacha EA, et al. Effects of lack of pulsatility on pulmonary endothelial function in the Fontan circulation. J Thorac Cardiovasc Surg 2013; 146(3): 522-9.
[http://dx.doi.org/10.1016/j.jtcvs.2012.11.031] [PMID: 23219498]
[81]
Snarr BS, Paridon SM, Rychik J, Goldberg DJ. Pulmonary vasodilator therapy in the failing Fontan circulation: Rationale and efficacy. Cardiol Young 2015; 25(8): 1489-92.
[http://dx.doi.org/10.1017/S1047951115002309] [PMID: 26675595]
[82]
Gewillig M, Brown SC, Heying R, et al. Volume load paradox while preparing for the Fontan: Not too much for the ventricle, not too little for the lungs. Interact Cardiovasc Thorac Surg 2010; 10(2): 262-5.
[http://dx.doi.org/10.1510/icvts.2009.218586] [PMID: 19945986]
[83]
Presson RG Jr, Baumgartner WA Jr, Peterson AJ, Glenny RW, Wagner WW Jr. Pulmonary capillaries are recruited during pulsatile flow. J Appl Physiol (1985) 2002; 92(3): 1183-90.
[http://dx.doi.org/10.1152/japplphysiol.00845.2001] [PMID: 11842057]
[84]
Wang W, Hu X, Liao W, et al. The efficacy and safety of pulmonary vasodilators in patients with Fontan circulation: A meta-analysis of randomized controlled trials. Pulm Circ 2019; 9(1): 2045894018790450.
[http://dx.doi.org/10.1177/2045894018790450] [PMID: 29972332]
[85]
Mori H, Park IS, Yamagishi H, et al. Sildenafil reduces pulmonary vascular resistance in single ventricular physiology. Int J Cardiol 2016; 221: 122-7.
[http://dx.doi.org/10.1016/j.ijcard.2016.06.322] [PMID: 27400308]
[86]
Ovaert C, Thijs D, Dewolf D, et al. The effect of bosentan in patients with a failing Fontan circulation. Cardiol Young 2009; 19(4): 331-9.
[http://dx.doi.org/10.1017/S1047951109990023] [PMID: 19519964]
[87]
Rhodes J, Ubeda-Tikkanen A, Clair M, et al. Effect of inhaled iloprost on the exercise function of Fontan patients: A demonstration of concept. Int J Cardiol 2013; 168(3): 2435-40.
[http://dx.doi.org/10.1016/j.ijcard.2013.03.014] [PMID: 23545150]
[88]
Sabri MR, Zolfi-Gol A, Ahmadi A, Haghjooy-Javanmard S. Effect of Tadalafil on myocardial and endothelial function and exercise performance after modified Fontan operation. Pediatr Cardiol 2016; 37(1): 55-61.
[http://dx.doi.org/10.1007/s00246-015-1238-x] [PMID: 26215768]
[89]
Uhm JY, Jhang WK, Park JJ, Seo DM, Yun SC, Yun TJ. Postoperative use of oral sildenafil in pediatric patients with congenital heart disease. Pediatr Cardiol 2010; 31(4): 515-20.
[http://dx.doi.org/10.1007/s00246-009-9632-x] [PMID: 20054528]
[90]
Van De Bruaene A, La Gerche A, Claessen G, et al. Sildenafil improves exercise hemodynamics in Fontan patients. Circ Cardiovasc Imaging 2014; 7(2): 265-73.
[http://dx.doi.org/10.1161/CIRCIMAGING.113.001243] [PMID: 24478333]
[91]
Castaldi B, Bordin G, Padalino M, Cuppini E, Vida V, Milanesi O. Hemodynamic impact of pulmonary vasodilators on single ventricle physiology. Cardiovasc Ther 2018; 36(1)
[http://dx.doi.org/10.1111/1755-5922.12314] [PMID: 29193758]
[92]
Goldberg DJ, et al. Results from the pediatric heart network’s Fontan udenafil exercise longitudinal trial. Circulation 2019; 140(25): E993-4.
[93]
Bridges ND, Lock JE, Castaneda AR. Baffle fenestration with subsequent transcatheter closure. Modification of the Fontan operation for patients at increased risk. Circulation 1990; 82(5): 1681-9.
[http://dx.doi.org/10.1161/01.CIR.82.5.1681] [PMID: 2225370]
[94]
Driscoll DJ. Long-term results of the Fontan operation. Pediatr Cardiol 2007; 28(6): 438-42.
[http://dx.doi.org/10.1007/s00246-007-9003-4] [PMID: 17768650]
[95]
Vyas H, Driscoll DJ, Cabalka AK, Cetta F, Hagler DJ. Results of transcatheter Fontan fenestration to treat protein losing enteropathy. Catheter Cardiovasc Interv 2007; 69(4): 584-9.
[http://dx.doi.org/10.1002/ccd.21045] [PMID: 17323361]
[96]
Kanter KR, Vincent RN. Management of aortopulmonary collateral arteries in Fontan patients: Occlusion improves clinical outcome. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 2002; 5: 48-54.
[http://dx.doi.org/10.1053/pcsu.2002.31501] [PMID: 11994864]
[97]
Stern HJ. The argument for aggressive coiling of aortopulmonary collaterals in single ventricle patients. Catheter Cardiovasc Interv 2009; 74(6): 897-900.
[http://dx.doi.org/10.1002/ccd.22156] [PMID: 19626684]
[98]
Bradley SM. Management of aortopulmonary collateral arteries in Fontan patients: Routine occlusion is not warranted. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 2002; 5: 55-67.
[http://dx.doi.org/10.1053/pcsu.2002.31483] [PMID: 11994865]
[99]
Rychik J. Protein-losing enteropathy after Fontan operation. Congenit Heart Dis 2007; 2(5): 288-300.
[http://dx.doi.org/10.1111/j.1747-0803.2007.00116.x] [PMID: 18377444]
[100]
Johnson JN, Driscoll DJ, O’Leary PW. Protein-losing enteropathy and the Fontan operation. Nutr Clin Pract 2012; 27(3): 375-84.
[http://dx.doi.org/10.1177/0884533612444532] [PMID: 22516942]
[101]
John AS, Phillips SD, Driscoll DJ, Warnes CA, Cetta F. The use of octreotide to successfully treat protein-losing enteropathy following the Fontan operation. Congenit Heart Dis 2011; 6(6): 653-6.
[http://dx.doi.org/10.1111/j.1747-0803.2011.00518.x] [PMID: 21545466]
[102]
Brizard CP, Lane GK, Alex G, Cheung MM. Original surgical procedure for the treatment of protein-losing enteropathy in Fontan patients: Report of two midterm successes. Circulation 2016; 134(8): 625-7.
[http://dx.doi.org/10.1161/CIRCULATIONAHA.116.023424] [PMID: 27550969]
[103]
Schumacher KR, Stringer KA, Donohue JE, et al. Fontan-associated protein-losing enteropathy and plastic bronchitis. J Pediatr 2015; 166(4): 970-7.
[http://dx.doi.org/10.1016/j.jpeds.2014.12.068] [PMID: 25661406]
[104]
Tomasulo CE, Chen JM, Smith CL, Maeda K, Rome JJ, Dori Y. Lymphatic disorders and management in patients with congenital heart disease. Ann Thorac Surg 2020; S0003-4975(20)32169-X.
[http://dx.doi.org/10.1016/j.athoracsur.2020.10.058] [PMID: 33373590]
[105]
Trojnarska O, Ciepłucha A. Challenges of management and therapy in patients with a functionally single ventricle after Fontan operation. Cardiol J 2011; 18(2): 119-27.
[PMID: 21432816]
[106]
Rychik J, Spray TL. Strategies to treat protein-losing enteropathy. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 2002; 5: 3-11.
[http://dx.doi.org/10.1053/pcsu.2002.31498] [PMID: 11994860]
[107]
Guariso G, Cerutti A, Moreolo GS, Milanesi O. Protein-losing enteropathy after Fontan operation: Treatment with elementary diet in one case. Pediatr Cardiol 2000; 21(3): 292.
[http://dx.doi.org/10.1007/s002460010066] [PMID: 10818200]
[108]
Gursu HA, Erdogan I, Varan B, et al. Oral budesonide as a therapy for protein-losing enteropathy in children after the Fontan operation. J Card Surg 2014; 29(5): 712-6.
[http://dx.doi.org/10.1111/jocs.12355] [PMID: 24889258]
[109]
Turner Z, Lanford L, Webber S. Oral budesonide as a therapy for protein-losing enteropathy in patients having undergone Fontan palliation. Congenit Heart Dis 2012; 7(1): 24-30.
[http://dx.doi.org/10.1111/j.1747-0803.2011.00593.x] [PMID: 22129195]
[110]
Thacker D, Patel A, Dodds K, Goldberg DJ, Semeao E, Rychik J. Use of oral budesonide in the management of protein-losing enteropathy after the Fontan operation. Ann Thorac Surg 2010; 89(3): 837-42.
[http://dx.doi.org/10.1016/j.athoracsur.2009.09.063] [PMID: 20172140]
[111]
Schumacher KR, Cools M, Goldstein BH, et al. Oral budesonide treatment for protein-losing enteropathy in Fontan-palliated patients. Pediatr Cardiol 2011; 32(7): 966-71.
[http://dx.doi.org/10.1007/s00246-011-0029-2] [PMID: 21660539]
[112]
John AS, Driscoll DJ, Warnes CA, Phillips SD, Cetta F. The use of oral budesonide in adolescents and adults with protein-losing enteropathy after the Fontan operation. Ann Thorac Surg 2011; 92(4): 1451-6.
[http://dx.doi.org/10.1016/j.athoracsur.2011.03.103] [PMID: 21784410]
[113]
Zaupper LB, Nielsen BW, Herlin T. Protein-losing enteropathy after the total cavopulmonary connection: Impact of intravenous immunoglobulin. Congenit Heart Dis 2011; 6(6): 624-9.
[http://dx.doi.org/10.1111/j.1747-0803.2011.00568.x] [PMID: 22010984]
[114]
Hoashi T, Ichikawa H, Ueno T, Kogaki S, Sawa Y. Steroid pulse therapy for protein-losing enteropathy after the Fontan operation. Congenit Heart Dis 2009; 4(4): 284-7.
[http://dx.doi.org/10.1111/j.1747-0803.2009.00274.x] [PMID: 19664034]
[115]
Windram JD, Clift PF, Speakman J, Thorne SA. An unusual treatment for protein losing enteropathy. Congenit Heart Dis 2011; 6(3): 253-6.
[http://dx.doi.org/10.1111/j.1747-0803.2011.00484.x] [PMID: 21418528]
[116]
Itkin M, Piccoli DA, Nadolski G, et al. Protein-losing enteropathy in patients with congenital heart disease. J Am Coll Cardiol 2017; 69(24): 2929-37.
[http://dx.doi.org/10.1016/j.jacc.2017.04.023] [PMID: 28619193]
[117]
Hallbergson A, Mascio CE, Rome JJ. Transcatheter Fontan takedown. Catheter Cardiovasc Interv 2015; 86(5): 849-54.
[http://dx.doi.org/10.1002/ccd.25963] [PMID: 25945427]
[118]
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(5): 1063-73.
[http://dx.doi.org/10.1016/S0022-5223(98)70406-4] [PMID: 9605076]
[119]
Greenway SC, Crossland DS, Hudson M, et al. Fontan-associated liver disease: Implications for heart transplantation. J Heart Lung Transplant 2016; 35(1): 26-33.
[http://dx.doi.org/10.1016/j.healun.2015.10.015] [PMID: 26586487]
[120]
Asrani SK, Warnes CA, Kamath PS. Hepatocellular carcinoma after the Fontan procedure. N Engl J Med 2013; 368(18): 1756-7.
[http://dx.doi.org/10.1056/NEJMc1214222] [PMID: 23635071]
[121]
Baek JS, Bae EJ, Ko JS, et al. Late hepatic complications after Fontan operation; Non-invasive markers of hepatic fibrosis and risk factors. Heart 2010; 96(21): 1750-5.
[http://dx.doi.org/10.1136/hrt.2010.201772] [PMID: 20956491]
[122]
Ofei SY, Gariepy C, Hanje J, Sisk T, Daniels CJ, Zaidi AN. Liver fibrosis in adults with Fontan palliation: Do common screening studies predict disease severity? Int J Cardiol 2015; 181: 174-5.
[http://dx.doi.org/10.1016/j.ijcard.2014.12.031] [PMID: 25528305]
[123]
Camposilvan S, Milanesi O, Stellin G, Pettenazzo A, Zancan L, D’Antiga L. Liver and cardiac function in the long term after Fontan operation. Ann Thorac Surg 2008; 86(1): 177-82.
[http://dx.doi.org/10.1016/j.athoracsur.2008.03.077] [PMID: 18573420]
[124]
Haller C. Taking care all along the way. J Thorac Cardiovasc Surg 2016; 151(3): 685-6.
[http://dx.doi.org/10.1016/j.jtcvs.2015.10.009] [PMID: 26519241]
[125]
Yoo SJ, Prsa M, Schantz D, et al. MR assessment of abdominal circulation in Fontan physiology. Int J Cardiovasc Imaging 2014; 30(6): 1065-72.
[http://dx.doi.org/10.1007/s10554-014-0424-x] [PMID: 24752955]
[126]
Friedrich-Rust M, Koch C, Rentzsch A, et al. Noninvasive assessment of liver fibrosis in patients with Fontan circulation using transient elastography and biochemical fibrosis markers. J Thorac Cardiovasc Surg 2008; 135(3): 560-7.
[http://dx.doi.org/10.1016/j.jtcvs.2007.09.039] [PMID: 18329470]
[127]
Johnson JA, Cetta F, Graham RP, et al. Identifying predictors of hepatic disease in patients after the Fontan operation: A postmortem analysis. J Thorac Cardiovasc Surg 2013; 146(1): 140-5.
[http://dx.doi.org/10.1016/j.jtcvs.2012.09.005] [PMID: 23072704]
[128]
Kiesewetter CH, Sheron N, Vettukattill JJ, et al. Hepatic changes in the failing Fontan circulation. Heart 2007; 93(5): 579-84.
[http://dx.doi.org/10.1136/hrt.2006.094516] [PMID: 17005713]
[129]
Goldberg DJ, Surrey LF, Glatz AC, et al. Hepatic fibrosis is universal following Fontan operation, and severity is associated with time from surgery: A liver biopsy and hemodynamic study. J Am Heart Assoc 2017; 6(5): e004809.
[http://dx.doi.org/10.1161/JAHA.116.004809] [PMID: 28446492]
[130]
Rychik J, Veldtman G, Rand E, et al. The precarious state of the liver after a Fontan operation: Summary of a multidisciplinary symposium. Pediatr Cardiol 2012; 33(7): 1001-12.
[http://dx.doi.org/10.1007/s00246-012-0315-7] [PMID: 22534759]
[131]
Lindsay I, Johnson J, Everitt MD, Hoffman J, Yetman AT. Impact of liver disease after the fontan operation. Am J Cardiol 2015; 115(2): 249-52.
[http://dx.doi.org/10.1016/j.amjcard.2014.10.032] [PMID: 25534765]
[132]
Evans WN, Acherman RJ, Ciccolo ML, et al. A composite noninvasive index correlates with liver fibrosis scores in post-Fontan patients: Preliminary findings. Congenit Heart Dis 2018; 13(1): 38-45.
[http://dx.doi.org/10.1111/chd.12558] [PMID: 29194972]
[133]
Fidai A, Dallaire F, Alvarez N, et al. Non-invasive investigations for the diagnosis of Fontan-associated liver disease in pediatric and adult Fontan patients. Front Cardiovasc Med 2017; 4: 15.
[http://dx.doi.org/10.3389/fcvm.2017.00015] [PMID: 28396861]
[134]
Hebson C, Book W, Elder RW, et al. “Frontiers in Fontan failure: A summary of conference proceedings”. Congenit Heart Dis 2017; 12(1): 6-16.
[http://dx.doi.org/10.1111/chd.12407] [PMID: 27597140]
[135]
Surrey LF, Russo P, Rychik J, et al. Defining the role of liver biopsy in the assessment of liver fibrosis in patients with Fontan circulation-reply. Hum Pathol 2017; 69: 141.
[http://dx.doi.org/10.1016/j.humpath.2017.04.029] [PMID: 28603067]
[136]
Hebert A, Mikkelsen UR, Thilen U, et al. Bosentan improves exercise capacity in adolescents and adults after Fontan operation: The TEMPO (treatment with endothelin receptor antagonist in fontan patients, a randomized, placebo-controlled, double-blind study measuring peak oxygen consumption) study. Circulation 2014; 130(23): 2021-30.
[http://dx.doi.org/10.1161/CIRCULATIONAHA.113.008441] [PMID: 25446057]
[137]
Shah H, Kuehl K, Sherker AH. Liver disease after the Fontan procedure: What the hepatologist needs to know. J Clin Gastroenterol 2010; 44(6): 428-31.
[http://dx.doi.org/10.1097/MCG.0b013e3181d476fc] [PMID: 20216078]
[138]
Téllez L, Rodríguez de Santiago E, Albillos A. Fontan-associated liver disease. Rev Esp Cardiol (Engl Ed) 2018; 71(3): 192-202.
[http://dx.doi.org/10.1016/j.rec.2017.10.052] [PMID: 29433942]
[139]
D’Souza BA, Fuller S, Gleason LP, et al. Single-center outcomes of combined heart and liver transplantation in the failing Fontan. Clin Transplant 2017; 31(3): ctr.12892.
[http://dx.doi.org/10.1111/ctr.12892] [PMID: 27988989]
[140]
Elder RW, Parekh S, Book WM. More on hepatocellular carcinoma after the Fontan procedure. N Engl J Med 2013; 369(5): 490.
[http://dx.doi.org/10.1056/NEJMc1306854] [PMID: 23902510]
[141]
Horai T, Bhama JK, Fontes PA, Toyoda Y. Combined heart and liver transplantation in a patient with situs ambiguous. Ann Thorac Surg 2011; 91(2): 600-1.
[http://dx.doi.org/10.1016/j.athoracsur.2010.07.078] [PMID: 21256326]
[142]
Vallabhajosyula P, Komlo C, Wallen TJ, Olthoff K, Pochettino A. Combined heart-liver transplant in a situs-ambiguous patient with failed Fontan physiology. J Thorac Cardiovasc Surg 2013; 145(4): e39-41.
[http://dx.doi.org/10.1016/j.jtcvs.2012.12.066] [PMID: 23380512]
[143]
Mauchley DC, Mitchell MB. Transplantation in the Fontan patient. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 2015; 18(1): 7-16.
[http://dx.doi.org/10.1053/j.pcsu.2015.01.001] [PMID: 25939837]
[144]
Heath L, Ling S, Racz J, et al. Prospective, longitudinal study of plastic bronchitis cast pathology and responsiveness to tissue plasminogen activator. Pediatr Cardiol 2011; 32(8): 1182-9.
[http://dx.doi.org/10.1007/s00246-011-0058-x] [PMID: 21786171]
[145]
Racz J, Mane G, Ford M, et al. Immunophenotyping and protein profiling of Fontan-associated plastic bronchitis airway casts. Ann Am Thorac Soc 2013; 10(2): 98-107.
[http://dx.doi.org/10.1513/AnnalsATS.201209-080OC] [PMID: 23607837]
[146]
Preciado D, Verghese S, Choi S. Aggressive bronchoscopic management of plastic bronchitis. Int J Pediatr Otorhinolaryngol 2010; 74(7): 820-2.
[http://dx.doi.org/10.1016/j.ijporl.2010.02.005] [PMID: 20546943]
[147]
Rychik J, Goldberg D, Rand E, et al. End-organ consequences of the Fontan operation: Liver fibrosis, protein-losing enteropathy and plastic bronchitis. Cardiol Young 2013; 23(6): 831-40.
[http://dx.doi.org/10.1017/S1047951113001650] [PMID: 24401255]
[148]
Dori Y, Itkin M. Etiology and new treatment options for patients with plastic bronchitis. J Thorac Cardiovasc Surg 2016; 152(2): e49-50.
[http://dx.doi.org/10.1016/j.jtcvs.2016.05.008] [PMID: 27423853]
[149]
Grutter G, Di Carlo D, Gandolfo F, et al. Plastic bronchitis after extracardiac Fontan operation. Ann Thorac Surg 2012; 94(3): 860-4.
[http://dx.doi.org/10.1016/j.athoracsur.2012.04.043] [PMID: 22698771]
[150]
Itkin MG, McCormack FX, Dori Y. Diagnosis and treatment of lymphatic plastic bronchitis in adults using advanced lymphatic imaging and percutaneous embolization. Ann Am Thorac Soc 2016; 13(10): 1689-96.
[http://dx.doi.org/10.1513/AnnalsATS.201604-292OC] [PMID: 27599255]
[151]
Tanase D, Ewert P, Eicken A. Plastic bronchitis: Symptomatic improvement after pulmonary arterial stenting in four patients with Fontan circulation. Cardiol Young 2015; 25(1): 151-3.
[http://dx.doi.org/10.1017/S1047951113002163] [PMID: 24345740]
[152]
Caruthers RL, Kempa M, Loo A, et al. Demographic characteristics and estimated prevalence of Fontan-associated plastic bronchitis. Pediatr Cardiol 2013; 34(2): 256-61.
[http://dx.doi.org/10.1007/s00246-012-0430-5] [PMID: 22797520]
[153]
Schumacher KR, Singh TP, Kuebler J, Aprile K, O’Brien M, Blume ED. Risk factors and outcome of Fontan-associated plastic bronchitis: A case-control study. J Am Heart Assoc 2014; 3(2): e000865.
[http://dx.doi.org/10.1161/JAHA.114.000865] [PMID: 24755155]
[154]
Chaudhari M, Stumper O. Plastic bronchitis after Fontan operation: Treatment with stent fenestration of the Fontan circuit. Heart 2004; 90(7): 801.
[http://dx.doi.org/10.1136/hrt.2003.029041] [PMID: 15201255]
[155]
Fredenburg TB, Johnson TR, Cohen MD. The Fontan procedure: Anatomy, complications, and manifestations of failure. Radiographics 2011; 31(2): 453-63.
[http://dx.doi.org/10.1148/rg.312105027] [PMID: 21415190]
[156]
Goldberg DJ, Dodds K, Rychik J. Rare problems associated with the Fontan circulation. Cardiol Young 2010; 20(Suppl. 3): 113-9.
[http://dx.doi.org/10.1017/S1047951110001162] [PMID: 21087567]
[157]
Tzifa A, Robards M, Simpson JM. Plastic bronchitis; a serious complication of the Fontan operation. Int J Cardiol 2005; 101(3): 513-4.
[http://dx.doi.org/10.1016/j.ijcard.2004.03.085] [PMID: 15907428]
[158]
Madsen P, Shah SA, Rubin BK. Plastic bronchitis: new insights and a classification scheme. Paediatr Respir Rev 2005; 6(4): 292-300.
[http://dx.doi.org/10.1016/j.prrv.2005.09.001] [PMID: 16298313]
[159]
Lis G, Cichocka-Jarosz E, Jedynak-Wasowicz U, Glowacka E. Add-on treatment with nebulized hypertonic saline in a child with plastic bronchitis after the Glenn procedure. J Bras Pneumol 2014; 40(1): 82-5.
[http://dx.doi.org/10.1590/S1806-37132014000100013] [PMID: 24626275]
[160]
Brooks K, Caruthers RL, Schumacher KR, Stringer KA. Pharmacotherapy challenges of Fontan-associated plastic bronchitis: A rare pediatric disease. Pharmacotherapy 2013; 33(9): 922-34.
[http://dx.doi.org/10.1002/phar.1290] [PMID: 23686915]
[161]
Silva RC, Simons JP, Chi DH, Yellon RF, Alper CM. Endoscopic treatment of plastic bronchitis. Arch Otolaryngol Head Neck Surg 2011; 137(4): 401-3.
[http://dx.doi.org/10.1001/archoto.2011.43] [PMID: 21502481]
[162]
Eberlein MH, Drummond MB, Haponik EF. Plastic bronchitis: A management challenge. Am J Med Sci 2008; 335(2): 163-9.
[http://dx.doi.org/10.1097/MAJ.0b013e318068b60e] [PMID: 18277130]
[163]
Do P, Randhawa I, Chin T, Parsapour K, Nussbaum E. Successful management of plastic bronchitis in a child post Fontan: Case report and literature review. Lung 2012; 190(4): 463-8.
[http://dx.doi.org/10.1007/s00408-012-9384-x] [PMID: 22430124]
[164]
Onoue Y, Adachi Y, Ichida F, Miyawaki T. Effective use of corticosteroid in a child with life-threatening plastic bronchitis after Fontan operation. Pediatr Int 2003; 45(1): 107-9.
[http://dx.doi.org/10.1046/j.1442-200X.2003.01659.x] [PMID: 12654082]
[165]
Costello JM, Steinhorn D, McColley S, Gerber ME, Kumar SP. Treatment of plastic bronchitis in a Fontan patient with tissue plasminogen activator: A case report and review of the literature. Pediatrics 2002; 109(4): e67.
[http://dx.doi.org/10.1542/peds.109.4.e67] [PMID: 11927740]
[166]
Wakeham MK, Van Bergen AH, Torero LE, Akhter J. Long-term treatment of plastic bronchitis with aerosolized tissue plasminogen activator in a Fontan patient. Pediatr Crit Care Med 2005; 6(1): 76-8.
[http://dx.doi.org/10.1097/01.PCC.0000149320.06424.1D] [PMID: 15636664]
[167]
Lubcke NL, Nussbaum VM, Schroth M. Use of aerosolized tissue plasminogen activator in the treatment of plastic bronchitis. Ann Pharmacother 2013; 47(3): e13.
[http://dx.doi.org/10.1345/aph.1R690] [PMID: 23424228]
[168]
Avitabile CM, Goldberg DJ, Dodds K, Dori Y, Ravishankar C, Rychik J. A multifaceted approach to the management of plastic bronchitis after cavopulmonary palliation. Ann Thorac Surg 2014; 98(2): 634-40.
[http://dx.doi.org/10.1016/j.athoracsur.2014.04.015] [PMID: 24841545]
[169]
Quasney MW, Orman K, Thompson J, et al. Plastic bronchitis occurring late after the Fontan procedure: Treatment with aerosolized urokinase. Crit Care Med 2000; 28(6): 2107-11.
[http://dx.doi.org/10.1097/00003246-200006000-00074] [PMID: 10890673]
[170]
Haseyama K, Satomi G, Yasukochi S, Matsui H, Harada Y, Uchita S. Pulmonary vasodilation therapy with sildenafil citrate in a patient with plastic bronchitis after the Fontan procedure for hypoplastic left heart syndrome. J Thorac Cardiovasc Surg 2006; 132(5): 1232-3.
[http://dx.doi.org/10.1016/j.jtcvs.2006.05.067] [PMID: 17059953]
[171]
Apostolopoulou SC, Papagiannis J, Rammos S. Bosentan induces clinical, exercise and hemodynamic improvement in a pre-transplant patient with plastic bronchitis after Fontan operation. J Heart Lung Transplant 2005; 24(8): 1174-6.
[http://dx.doi.org/10.1016/j.healun.2004.11.018] [PMID: 16102469]
[172]
Wilson J, Russell J, Williams W, Benson L. Fenestration of the Fontan circuit as treatment for plastic bronchitis. Pediatr Cardiol 2005; 26(5): 717-9.
[http://dx.doi.org/10.1007/s00246-005-0913-8] [PMID: 16132280]
[173]
Seear M, Hui H, Magee F, Bohn D, Cutz E. Bronchial casts in children: A proposed classification based on nine cases and a review of the literature. Am J Respir Crit Care Med 1997; 155(1): 364-70.
[http://dx.doi.org/10.1164/ajrccm.155.1.9001337] [PMID: 9001337]
[174]
Hug MI, Ersch J, Moenkhoff M, Burger R, Fanconi S, Bauersfeld U. Chylous bronchial casts after fontan operation. Circulation 2001; 103(7): 1031-3.
[http://dx.doi.org/10.1161/01.CIR.103.7.1031] [PMID: 11181481]
[175]
Parikh K, Witte MH, Samson R, et al. Successful treatment of plastic bronchitis with low fat diet and subsequent thoracic duct ligation in child with Fontan physiology. Lymphology 2012; 45(2): 47-52.
[PMID: 23057148]
[176]
Nayar S, Parmar R, Kulkarni S, Cherian KM. Treatment of plastic bronchitis. Ann Thorac Surg 2007; 83(5): 1884-6.
[http://dx.doi.org/10.1016/j.athoracsur.2006.12.027] [PMID: 17462426]
[177]
Shah SS, Drinkwater DC, Christian KG. Plastic bronchitis: Is thoracic duct ligation a real surgical option? Ann Thorac Surg 2006; 81(6): 2281-3.
[http://dx.doi.org/10.1016/j.athoracsur.2005.07.004] [PMID: 16731170]
[178]
Ugaki S, Lord DJ, Sherwood MC, Winlaw DS. Lymphangiography is a diagnostic and therapeutic intervention for patients with plastic bronchitis after the Fontan operation. J Thorac Cardiovasc Surg 2016; 152(2): e47-9.
[http://dx.doi.org/10.1016/j.jtcvs.2016.04.051] [PMID: 27179837]
[179]
Egbe AC, Connolly HM, Niaz T, et al. Prevalence and outcome of thrombotic and embolic complications in adults after Fontan operation. Am Heart J 2017; 183: 10-7.
[http://dx.doi.org/10.1016/j.ahj.2016.09.014] [PMID: 27979032]
[180]
Alsaied T, Alsidawi S, Allen CC, Faircloth J, Palumbo JS, Veldtman GR. Strategies for thromboprophylaxis in Fontan circulation: A meta-analysis. Heart 2015; 101(21): 1731-7.
[http://dx.doi.org/10.1136/heartjnl-2015-307930] [PMID: 26319122]
[181]
Jacobs ML, Pourmoghadam KK. Thromboembolism and the role of anticoagulation in the Fontan patient. Pediatr Cardiol 2007; 28(6): 457-64.
[http://dx.doi.org/10.1007/s00246-007-9006-1] [PMID: 17762953]
[182]
Firdouse M, Agarwal A, Chan AK, Mondal T. Thrombosis and thromboembolic complications in fontan patients: A literature review. Clin Appl Thromb Hemost 2014; 20(5): 484-92.
[http://dx.doi.org/10.1177/1076029613520464] [PMID: 24463598]
[183]
Karsenty C, Zhao A, Marijon E, Ladouceur M. Risk of thromboembolic complications in adult congenital heart disease: A literature review. Arch Cardiovasc Dis 2018; 111(10): 613-20.
[http://dx.doi.org/10.1016/j.acvd.2018.04.003] [PMID: 29859704]
[184]
Takeuchi D, Inai K, Shinohara T, Nakanishi T, Park IS. Blood coagulation abnormalities and the usefulness of D-dimer level for detecting intracardiac thrombosis in adult Fontan patients. Int J Cardiol 2016; 224: 139-44.
[http://dx.doi.org/10.1016/j.ijcard.2016.09.017] [PMID: 27648983]
[185]
Tomkiewicz-Pajak L, Hoffman P, Trojnarska O, Lipczyńska M, Podolec P, Undas A. Abnormalities in blood coagulation, fibrinolysis, and platelet activation in adult patients after the Fontan procedure. J Thorac Cardiovasc Surg 2014; 147(4): 1284-90.
[http://dx.doi.org/10.1016/j.jtcvs.2013.06.011] [PMID: 23891464]
[186]
Tomkiewicz-Pajak L, Wojcik T, Chłopicki S, et al. Aspirin resistance in adult patients after Fontan surgery. Int J Cardiol 2015; 181: 19-26.
[http://dx.doi.org/10.1016/j.ijcard.2014.11.219] [PMID: 25479533]
[187]
Egbe AC, Connolly HM, McLeod CJ, et al. Thrombotic and embolic complications associated with atrial arrhythmia after Fontan operation: Role of prophylactic therapy. J Am Coll Cardiol 2016; 68(12): 1312-9.
[http://dx.doi.org/10.1016/j.jacc.2016.06.056] [PMID: 27634123]
[188]
Stout KK, Daniels CJ, Aboulhosn JA, et al. AHA/ACC guideline for the management of adults with congenital heart disease: Executive summary: A report of the American college of cardiology/American heart association task force on clinical practice guidelines. J Am Coll Cardiol 2019; 73(12): 1494-563.
[http://dx.doi.org/10.1016/j.jacc.2018.08.1028] [PMID: 30121240]
[189]
Canter CE, Shaddy RE, Bernstein D, et al. Indications for heart transplantation in pediatric heart disease: A scientific statement from the American heart association council on cardiovascular disease in the young; the councils on clinical cardiology, cardiovascular nursing, and cardiovascular surgery and anesthesia; and the quality of care and outcomes research interdisciplinary working group. Circulation 2007; 115(5): 658-76.
[http://dx.doi.org/10.1161/CIRCULATIONAHA.106.180449] [PMID: 17261651]
[190]
Mitchell MB, Campbell DN, Boucek MM. Heart transplantation for the failing Fontan circulation. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 2004; 7: 56-64.
[http://dx.doi.org/10.1053/j.pcsu.2004.02.013] [PMID: 15283353]
[191]
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(5): 693-702.
[http://dx.doi.org/10.1016/j.jtcvs.2004.07.013] [PMID: 15514596]
[192]
Griffiths ER, Kaza AK, Wyler von Ballmoos MC, et al. Evaluating failing Fontans for heart transplantation: Predictors of death. Ann Thorac Surg 2009; 88(2): 558-63.
[http://dx.doi.org/10.1016/j.athoracsur.2009.03.085] [PMID: 19632412]
[193]
Tabarsi N, Guan M, Simmonds J, et al. Meta-analysis of the effectiveness of heart transplantation in patients with a failing Fontan. Am J Cardiol 2017; 119(8): 1269-74.
[http://dx.doi.org/10.1016/j.amjcard.2017.01.001] [PMID: 28233535]
[194]
Van De Bruaene A, Meier L, Droogne W, et al. Management of acute heart failure in adult patients with congenital heart disease. Heart Fail Rev 2018; 23(1): 1-14.
[http://dx.doi.org/10.1007/s10741-017-9664-x] [PMID: 29277859]
[195]
Leeser DB, Jeevanandam V, Furukawa S, et al. Simultaneous heart and kidney transplantation in patients with end-stage heart and renal failure. Am J Transplant 2001; 1(1): 89-92.
[http://dx.doi.org/10.1034/j.1600-6143.2001.010116.x] [PMID: 12095045]
[196]
Vermes E, Kirsch M, Houel R, et al. Immunologic events and long term survival after combined heart and kidney transplantation: A twelve-year single-center experience. J Heart Lung Transplant 2001; 20(2): 247.
[http://dx.doi.org/10.1016/S1053-2498(00)00561-1] [PMID: 11250488]
[197]
Porrett PM, Desai SS, Timmins KJ, Twomey CR, Sonnad SS, Olthoff KM. Combined orthotopic heart and liver transplantation: the need for exception status listing. Liver Transpl 2004; 10(12): 1539-44.
[http://dx.doi.org/10.1002/lt.20279] [PMID: 15558654]
[198]
Rijnberg FM, van Assen HC, Hazekamp MG, Roest AAW. Tornado-like flow in the Fontan circulation: Insights from quantification and visualization of viscous energy loss rate using 4D flow MRI. Eur Heart J 2019; 40(26): 2170.
[http://dx.doi.org/10.1093/eurheartj/ehz160] [PMID: 30916327]
[199]
Ginde S, Goot BH, Frommelt PC. Imaging adult patients with Fontan circulation. Curr Opin Cardiol 2017; 32(5): 521-8.
[http://dx.doi.org/10.1097/HCO.0000000000000422] [PMID: 28548989]
[200]
Mehra MR, Goldstein DJ, Uriel N, et al. Two-year outcomes with a magnetically levitated cardiac pump in heart failure. N Engl J Med 2018; 378(15): 1386-95.
[http://dx.doi.org/10.1056/NEJMoa1800866] [PMID: 29526139]
[201]
Adachi I, Burki S, Horne D, et al. The miniaturized pediatric continuous-flow device: Preclinical assessment in the chronic sheep model. J Thorac Cardiovasc Surg 2017; 154(1): 291-300.
[http://dx.doi.org/10.1016/j.jtcvs.2016.12.070] [PMID: 28495065]
[202]
Miller JR, Lancaster TS, Callahan C, Abarbanell AM, Eghtesady P. An overview of mechanical circulatory support in single-ventricle patients. Transl Pediatr 2018; 7(2): 151-61.
[http://dx.doi.org/10.21037/tp.2018.03.03] [PMID: 29770296]
[203]
Rodefeld MD, Coats B, Fisher T, et al. Cavopulmonary assist for the univentricular Fontan circulation: von Kármán viscous impeller pump. J Thorac Cardiovasc Surg 2010; 140(3): 529-36.
[http://dx.doi.org/10.1016/j.jtcvs.2010.04.037] [PMID: 20561640]
[204]
Rodefeld MD, Frankel SH, Giridharan GA. Cavopulmonary assist: (em)powering the univentricular fontan circulation. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 2011; 14(1): 45-54.
[http://dx.doi.org/10.1053/j.pcsu.2011.01.015] [PMID: 21444049]
[205]
Swartz MF, DiVincenti L, Smith K, et al. A modified LVAD technique to augment caval and pulmonary arterial blood flow in the “failing Fontan” circulation. J Card Surg 2017; 32(2): 126-32.
[http://dx.doi.org/10.1111/jocs.12931] [PMID: 28070902]
[206]
Jaquiss RD, Aziz H. Is four stage management the future of univentricular hearts? Destination therapy in the young. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 2016; 19(1): 50-4.
[http://dx.doi.org/10.1053/j.pcsu.2015.12.004] [PMID: 27060043]
[207]
Kogon B, McConnell M, Book W. Fontan conversion with hepatic vein exclusion: A means for hepatic preservation in single ventricle heart disease. Cardiol Young 2016; 26(3): 582-5.
[http://dx.doi.org/10.1017/S104795111500133X] [PMID: 26139025]

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