Official reprint from UpToDate®
www.uptodate.com ©2017 UpToDate®

Management of patients post-Fontan procedure

Jonathan Johnson, MD
Heidi M Connolly, MD, FASE
Section Editor
Gruschen Veldtman, MBChB, FRCP, Dip Obst
Deputy Editor
Susan B Yeon, MD, JD, FACC


The Fontan operation is a palliative surgical procedure performed in patients with a functional or anatomic single ventricle (also known as univentricular heart) [1]. The Fontan operation originally was described for patients with tricuspid atresia in 1971 [2]. In the past 40 years, the types of malformations for which the Fontan operation is utilized has expanded considerably, and it has become the most common operation performed for patients with any type of single ventricle [3]. Following the Fontan procedure, patients face substantial morbidity and mortality risk and require lifelong follow-up with a cardiologist experienced in the care of patients with complex congenital heart disease (pediatric cardiologist or adult congenital heart disease specialist).

This topic will discuss management of patients who have undergone a Fontan procedure. Lesions for which a Fontan may be performed are discussed separately.


The Fontan procedure is performed in patients with functional or anatomic single ventricle, defined as presence of only one well-developed ventricle with the rudimentary ventricle (if present) less than 30 percent of its normal volume [1]. Lesions for which a Fontan may be performed include:

Hypoplastic left heart syndrome (see "Hypoplastic left heart syndrome")

Tricuspid atresia (see "Tricuspid valve (TV) atresia")


Subscribers log in here

To continue reading this article, you must log in with your personal, hospital, or group practice subscription. For more information or to purchase a personal subscription, click below on the option that best describes you:
Literature review current through: Apr 2017. | This topic last updated: May 02, 2017.
The content on the UpToDate website is not intended nor recommended as a substitute for medical advice, diagnosis, or treatment. Always seek the advice of your own physician or other qualified health care professional regarding any medical questions or conditions. The use of this website is governed by the UpToDate Terms of Use ©2017 UpToDate, Inc.
  1. Warnes CA, Williams RG, Bashore TM, et al. ACC/AHA 2008 guidelines for the management of adults with congenital heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Develop Guidelines on the Management of Adults With Congenital Heart Disease). Developed in Collaboration With the American Society of Echocardiography, Heart Rhythm Society, International Society for Adult Congenital Heart Disease, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons. J Am Coll Cardiol 2008; 52:e143.
  2. Fontan F, Baudet E. Surgical repair of tricuspid atresia. Thorax 1971; 26:240.
  3. Driscoll DJ. Long-term results of the Fontan operation. Pediatr Cardiol 2007; 28:438.
  4. Shafer KM, Garcia JA, Babb TG, et al. The importance of the muscle and ventilatory blood pumps during exercise in patients without a subpulmonary ventricle (Fontan operation). J Am Coll Cardiol 2012; 60:2115.
  5. Beghetti M. Fontan and the pulmonary circulation: a potential role for new pulmonary hypertension therapies. Heart 2010; 96:911.
  6. Goldberg DJ, Dodds K, Rychik J. Rare problems associated with the Fontan circulation. Cardiol Young 2010; 20 Suppl 3:113.
  7. Hosein RB, Clarke AJ, McGuirk SP, et al. Factors influencing early and late outcome following the Fontan procedure in the current era. The 'Two Commandments'? Eur J Cardiothorac Surg 2007; 31:344.
  8. Feinstein JA, Benson DW, Dubin AM, et al. Hypoplastic left heart syndrome: current considerations and expectations. J Am Coll Cardiol 2012; 59:S1.
  9. Veldtman GR, Nishimoto A, Siu S, et al. The Fontan procedure in adults. Heart 2001; 86:330.
  10. Kawashima Y, Kitamura S, Matsuda H, et al. Total cavopulmonary shunt operation in complex cardiac anomalies. A new operation. J Thorac Cardiovasc Surg 1984; 87:74.
  11. Gewillig M. The Fontan circulation. Heart 2005; 91:839.
  12. 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; 96:682.
  13. Pridjian AK, Mendelsohn AM, Lupinetti FM, et al. Usefulness of the bidirectional Glenn procedure as staged reconstruction for the functional single ventricle. Am J Cardiol 1993; 71:959.
  14. 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; 100:228.
  15. Bridges ND, Lock JE, Castaneda AR. Baffle fenestration with subsequent transcatheter closure. Modification of the Fontan operation for patients at increased risk. Circulation 1990; 82:1681.
  16. Thompson LD, Petrossian E, McElhinney DB, et al. Is it necessary to routinely fenestrate an extracardiac fontan? J Am Coll Cardiol 1999; 34:539.
  17. Hsu DT, Quaegebeur JM, Ing FF, et al. Outcome after the single-stage, nonfenestrated Fontan procedure. Circulation 1997; 96:II.
  18. Lemler MS, Scott WA, Leonard SR, et al. Fenestration improves clinical outcome of the fontan procedure: a prospective, randomized study. Circulation 2002; 105:207.
  19. 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; 52:85.
  20. 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; 84:1457.
  21. Mavroudis C, Backer CL, Deal BJ, Johnsrude CL. Fontan conversion to cavopulmonary connection and arrhythmia circuit cryoblation. J Thorac Cardiovasc Surg 1998; 115:547.
  22. Marcelletti CF, Hanley FL, Mavroudis C, et al. Revision of previous Fontan connections to total extracardiac cavopulmonary anastomosis: A multicenter experience. J Thorac Cardiovasc Surg 2000; 119:340.
  23. Mair DD, Puga FJ, Danielson GK. The Fontan procedure for tricuspid atresia: early and late results of a 25-year experience with 216 patients. J Am Coll Cardiol 2001; 37:933.
  24. Driscoll DJ, Offord KP, Feldt RH, et al. Five- to fifteen-year follow-up after Fontan operation. Circulation 1992; 85:469.
  25. Ono M, Boethig D, Goerler H, et al. Clinical outcome of patients 20 years after Fontan operation--effect of fenestration on late morbidity. Eur J Cardiothorac Surg 2006; 30:923.
  26. Khairy P, Fernandes SM, Mayer JE Jr, et al. Long-term survival, modes of death, and predictors of mortality in patients with Fontan surgery. Circulation 2008; 117:85.
  27. Salazar JD, Zafar F, Siddiqui K, et al. Fenestration during Fontan palliation: now the exception instead of the rule. J Thorac Cardiovasc Surg 2010; 140:129.
  28. Hirsch JC, Ohye RG, Devaney EJ, et al. The lateral tunnel Fontan procedure for hypoplastic left heart syndrome: results of 100 consecutive patients. Pediatr Cardiol 2007; 28:426.
  29. Morris SA, Ethen MK, Penny DJ, et al. Prenatal diagnosis, birth location, surgical center, and neonatal mortality in infants with hypoplastic left heart syndrome. Circulation 2014; 129:285.
  30. Ghanayem NS, Allen KR, Tabbutt S, et al. Interstage mortality after the Norwood procedure: Results of the multicenter Single Ventricle Reconstruction trial. J Thorac Cardiovasc Surg 2012; 144:896.
  31. Ghelani SJ, Harrild DM, Gauvreau K, et al. Comparison Between Echocardiography and Cardiac Magnetic Resonance Imaging in Predicting Transplant-Free Survival After the Fontan Operation. Am J Cardiol 2015; 116:1132.
  32. Lasa JJ, Glatz AC, Daga A, Shah M. Prevalence of arrhythmias late after the Fontan operation. Am J Cardiol 2014; 113:1184.
  33. d'Udekem Y, Iyengar AJ, Cochrane AD, et al. The Fontan procedure: contemporary techniques have improved long-term outcomes. Circulation 2007; 116:I157.
  34. Durongpisitkul K, Porter CJ, Cetta F, et al. Predictors of early- and late-onset supraventricular tachyarrhythmias after Fontan operation. Circulation 1998; 98:1099.
  35. Correa R, Walsh EP, Alexander ME, et al. Transbaffle mapping and ablation for atrial tachycardias after mustard, senning, or Fontan operations. J Am Heart Assoc 2013; 2:e000325.
  36. Tsao S, Deal BJ, Backer CL, et al. Device management of arrhythmias after Fontan conversion. J Thorac Cardiovasc Surg 2009; 138:937.
  37. Driscoll DJ, Danielson GK, Puga FJ, et al. Exercise tolerance and cardiorespiratory response to exercise after the Fontan operation for tricuspid atresia or functional single ventricle. J Am Coll Cardiol 1986; 7:1087.
  38. Zellers TM, Driscoll DJ, Mottram CD, et al. Exercise tolerance and cardiorespiratory response to exercise before and after the Fontan operation. Mayo Clin Proc 1989; 64:1489.
  39. Paridon SM, Mitchell PD, Colan SD, et al. A cross-sectional study of exercise performance during the first 2 decades of life after the Fontan operation. J Am Coll Cardiol 2008; 52:99.
  40. Harrison DA, Liu P, Walters JE, et al. Cardiopulmonary function in adult patients late after Fontan repair. J Am Coll Cardiol 1995; 26:1016.
  41. Durongpisitkul K, Driscoll DJ, Mahoney DW, et al. Cardiorespiratory response to exercise after modified Fontan operation: determinants of performance. J Am Coll Cardiol 1997; 29:785.
  42. Rhodes J, Garofano RP, Bowman FO Jr, et al. Effect of right ventricular anatomy on the cardiopulmonary response to exercise. Implications for the Fontan procedure. Circulation 1990; 81:1811.
  43. Strömvall Larsson E, Eriksson BO. Haemodynamic adaptation during exercise in fontan patients at a long-term follow-up. Scand Cardiovasc J 2003; 37:107.
  44. Goldberg DJ, Avitabile CM, McBride MG, Paridon SM. Exercise capacity in the Fontan circulation. Cardiol Young 2013; 23:824.
  45. Shachar GB, Fuhrman BP, Wang Y, et al. Rest and exercise hemodynamics after the Fontan procedure. Circulation 1982; 65:1043.
  46. Balfour IC, Drimmer AM, Nouri S, et al. Pediatric cardiac rehabilitation. Am J Dis Child 1991; 145:627.
  47. McCrindle BW, Williams RV, Mital S, et al. Physical activity levels in children and adolescents are reduced after the Fontan procedure, independent of exercise capacity, and are associated with lower perceived general health. Arch Dis Child 2007; 92:509.
  48. Jacobsen RM, Ginde S, Mussatto K, et al. Can a Home-based Cardiac Physical Activity Program Improve the Physical Function Quality of Life in Children with Fontan Circulation? Congenit Heart Dis 2016; 11:175.
  49. Goldberg B, Fripp RR, Lister G, et al. Effect of physical training on exercise performance of children following surgical repair of congenital heart disease. Pediatrics 1981; 68:691.
  50. Marino BS, Lipkin PH, Newburger JW, et al. Neurodevelopmental outcomes in children with congenital heart disease: evaluation and management: a scientific statement from the American Heart Association. Circulation 2012; 126:1143.
  51. DeMaso DR, Calderon J, Taylor GA, et al. Psychiatric Disorders in Adolescents With Single Ventricle Congenital Heart Disease. Pediatrics 2017; 139.
  52. Kouatli AA, Garcia JA, Zellers TM, et al. Enalapril does not enhance exercise capacity in patients after Fontan procedure. Circulation 1997; 96:1507.
  53. Goldberg DJ, French B, McBride MG, et al. Impact of oral sildenafil on exercise performance in children and young adults after the fontan operation: a randomized, double-blind, placebo-controlled, crossover trial. Circulation 2011; 123:1185.
  54. Hebert A, Jensen AS, Idorn L, et al. The effect of bosentan on exercise capacity in Fontan patients; rationale and design for the TEMPO study. BMC Cardiovasc Disord 2013; 13:36.
  55. Sojak V, Mazic U, Cesen M, et al. Cardiac resynchronization therapy for the failing Fontan patient. Ann Thorac Surg 2008; 85:2136.
  56. Niebler RA, Ghanayem NS, Shah TK, et al. Use of a HeartWare ventricular assist device in a patient with failed Fontan circulation. Ann Thorac Surg 2014; 97:e115.
  57. Rungan S, Finucane K, Gentles T, et al. Heart transplantation in pediatric and congenital heart disease: a single-center experience. World J Pediatr Congenit Heart Surg 2014; 5:200.
  58. Newcomb AE, Negri JC, Brizard CP, d'Udekem Y. Successful left ventricular assist device bridge to transplantation after failure of a fontan revision. J Heart Lung Transplant 2006; 25:365.
  59. Durham LA 3rd, Dearani JA, Burkhart HM, et al. Application of Computer Modeling in Systemic VAD Support of Failing Fontan Physiology. World J Pediatr Congenit Heart Surg 2011; 2:243.
  60. Almond CS, Thiagarajan RR, Piercey GE, et al. Waiting list mortality among children listed for heart transplantation in the United States. Circulation 2009; 119:717.
  61. Bernstein D, Naftel D, Chin C, et al. Outcome of listing for cardiac transplantation for failed Fontan: a multi-institutional study. Circulation 2006; 114:273.
  62. Michielon G, Parisi F, Di Carlo D, et al. Orthotopic heart transplantation for failing single ventricle physiology. Eur J Cardiothorac Surg 2003; 24:502.
  63. Kanter KR, Mahle WT, Vincent RN, et al. Heart transplantation in children with a Fontan procedure. Ann Thorac Surg 2011; 91:823.
  64. Jayakumar KA, Addonizio LJ, Kichuk-Chrisant MR, et al. Cardiac transplantation after the Fontan or Glenn procedure. J Am Coll Cardiol 2004; 44:2065.
  65. 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; 119:1269.
  66. Simpson KE, Pruitt E, Kirklin JK, et al. Fontan Patient Survival After Pediatric Heart Transplantation Has Improved in the Current Era. Ann Thorac Surg 2017; 103:1315.
  67. Lund LH, Edwards LB, Dipchand AI, et al. The Registry of the International Society for Heart and Lung Transplantation: Thirty-third Adult Heart Transplantation Report-2016; Focus Theme: Primary Diagnostic Indications for Transplant. J Heart Lung Transplant 2016; 35:1158.
  68. Mavroudis C, Stewart RD, Backer CL, et al. Atrioventricular valve procedures with repeat fontan operations: influence of valve pathology, ventricular function, and arrhythmias on outcome. Ann Thorac Surg 2005; 80:29.
  69. Mori M, Aguirre AJ, Elder RW, et al. Beyond a broken heart: circulatory dysfunction in the failing Fontan. Pediatr Cardiol 2014; 35:569.
  70. Sammour F, Haw M, Paisey J, et al. Renal function of patients with a failing Fontan circuit undergoing total cavopulmonary revision surgery. Pediatr Cardiol 2009; 30:282.
  71. Magee AG, McCrindle BW, Mawson J, et al. Systemic venous collateral development after the bidirectional cavopulmonary anastomosis. Prevalence and predictors. J Am Coll Cardiol 1998; 32:502.
  72. Weber HS. Incidence and predictors for the development of significant supradiaphragmatic decompressing venous collateral channels following creation of Fontan physiology. Cardiol Young 2001; 11:289.
  73. Poterucha JT, Johnson JN, Taggart NW, et al. Embolization of Veno-venous Collaterals after the Fontan Operation Is Associated with Decreased Survival. Congenit Heart Dis 2015; 10:E230.
  74. Tsang W, Johansson B, Salehian O, et al. Intracardiac thrombus in adults with the Fontan circulation. Cardiol Young 2007; 17:646.
  75. Monagle P, Cochrane A, Roberts R, et al. A multicenter, randomized trial comparing heparin/warfarin and acetylsalicylic acid as primary thromboprophylaxis for 2 years after the Fontan procedure in children. J Am Coll Cardiol 2011; 58:645.
  76. McCrindle BW, Manlhiot C, Cochrane A, et al. Factors associated with thrombotic complications after the Fontan procedure: a secondary analysis of a multicenter, randomized trial of primary thromboprophylaxis for 2 years after the Fontan procedure. J Am Coll Cardiol 2013; 61:346.
  77. Kudumula V, Mathur S, Bu'Lock F. Successful thrombolysis of massive intracardiac thrombus in atriopulmonary Fontan circulation. Cardiol Young 2010; 20:443.
  78. Rychik J. Protein-losing enteropathy after Fontan operation. Congenit Heart Dis 2007; 2:288.
  79. Mertens L, Hagler DJ, Sauer U, et al. Protein-losing enteropathy after the Fontan operation: an international multicenter study. PLE study group. J Thorac Cardiovasc Surg 1998; 115:1063.
  80. Feldt RH, Driscoll DJ, Offord KP, et al. Protein-losing enteropathy after the Fontan operation. J Thorac Cardiovasc Surg 1996; 112:672.
  81. Rychik J, Gui-Yang S. Relation of mesenteric vascular resistance after Fontan operation and protein-losing enteropathy. Am J Cardiol 2002; 90:672.
  82. Bode L, Salvestrini C, Park PW, et al. Heparan sulfate and syndecan-1 are essential in maintaining murine and human intestinal epithelial barrier function. J Clin Invest 2008; 118:229.
  83. Johnson JN, Driscoll DJ, O'Leary PW. Protein-losing enteropathy and the Fontan operation. Nutr Clin Pract 2012; 27:375.
  84. Dori Y, Keller MS, Rychik J, Itkin M. Successful treatment of plastic bronchitis by selective lymphatic embolization in a Fontan patient. Pediatrics 2014; 134:e590.
  85. Florent C, L'Hirondel C, Desmazures C, et al. Intestinal clearance of alpha 1-antitrypsin. A sensitive method for the detection of protein-losing enteropathy. Gastroenterology 1981; 81:777.
  86. Fujii T, Shimizu T, Takahashi K, et al. Fecal alpha1-antitrypsin concentrations as a measure of enteric protein loss after modified fontan operations. J Pediatr Gastroenterol Nutr 2003; 37:577.
  87. Vyas H, Driscoll DJ, Cetta F, et al. Gastrointestinal bleeding and protein-losing enteropathy after the fontan operation. Am J Cardiol 2006; 98:666.
  88. Bhagirath KM, Tam JW. Resolution of protein-losing enteropathy with low-molecular weight heparin in an adult patient with Fontan palliation. Ann Thorac Surg 2007; 84:2110.
  89. Donnelly JP, Rosenthal A, Castle VP, Holmes RD. Reversal of protein-losing enteropathy with heparin therapy in three patients with univentricular hearts and Fontan palliation. J Pediatr 1997; 130:474.
  90. Ryerson L, Goldberg C, Rosenthal A, Armstrong A. Usefulness of heparin therapy in protein-losing enteropathy associated with single ventricle palliation. Am J Cardiol 2008; 101:248.
  91. John AS, Phillips SD, Driscoll DJ, et al. The use of octreotide to successfully treat protein-losing enteropathy following the Fontan operation. Congenit Heart Dis 2011; 6:653.
  92. John AS, Driscoll DJ, Warnes CA, et al. The use of oral budesonide in adolescents and adults with protein-losing enteropathy after the Fontan operation. Ann Thorac Surg 2011; 92:1451.
  93. Asrani SK, Warnes CA, Kamath PS. Hepatocellular carcinoma after the Fontan procedure. N Engl J Med 2013; 368:1756.
  94. Pundi K, Pundi KN, Kamath PS, et al. Liver Disease in Patients After the Fontan Operation. Am J Cardiol 2016; 117:456.
  95. 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; 33:1001.
  96. Kiesewetter CH, Sheron N, Vettukattill JJ, et al. Hepatic changes in the failing Fontan circulation. Heart 2007; 93:579.
  97. Simpson KE, Esmaeeli A, Khanna G, et al. Liver cirrhosis in Fontan patients does not affect 1-year post-heart transplant mortality or markers of liver function. J Heart Lung Transplant 2014; 33:170.
  98. Wallihan DB, Podberesky DJ, Marino BS, et al. Relationship of MR elastography determined liver stiffness with cardiac function after Fontan palliation. J Magn Reson Imaging 2014; 40:1328.
  99. Song P, Mellema DC, Sheedy SP, et al. Performance of 2-Dimensional Ultrasound Shear Wave Elastography in Liver Fibrosis Detection Using Magnetic Resonance Elastography as the Reference Standard: A Pilot Study. J Ultrasound Med 2016; 35:401.
  100. Assenza GE, Graham DA, Landzberg MJ, et al. MELD-XI score and cardiac mortality or transplantation in patients after Fontan surgery. Heart 2013; 99:491.
  101. Elder RW, McCabe NM, Hebson C, et al. Features of portal hypertension are associated with major adverse events in Fontan patients: the VAST study. Int J Cardiol 2013; 168:3764.
  102. Opotowsky AR, Landzberg MJ, Earing MG, et al. Abnormal spirometry after the Fontan procedure is common and associated with impaired aerobic capacity. Am J Physiol Heart Circ Physiol 2014; 307:H110.
  103. Fredriksen PM, Therrien J, Veldtman G, et al. Lung function and aerobic capacity in adult patients following modified Fontan procedure. Heart 2001; 85:295.
  104. Kadhim M, Pizarro C, Holmes L Jr, et al. Prevalence of scoliosis in patients with Fontan circulation. Arch Dis Child 2013; 98:170.
  105. Matthews IL, Fredriksen PM, Bjørnstad PG, et al. Reduced pulmonary function in children with the Fontan circulation affects their exercise capacity. Cardiol Young 2006; 16:261.
  106. Brogan TV, Finn LS, Pyskaty DJ Jr, et al. Plastic bronchitis in children: a case series and review of the medical literature. Pediatr Pulmonol 2002; 34:482.
  107. Caruthers RL, Kempa M, Loo A, et al. Demographic characteristics and estimated prevalence of Fontan-associated plastic bronchitis. Pediatr Cardiol 2013; 34:256.
  108. Schumacher KR, Singh TP, Kuebler J, et al. Risk factors and outcome of Fontan-associated plastic bronchitis: a case-control study. J Am Heart Assoc 2014; 3:e000865.
  109. Costello JM, Steinhorn D, McColley S, et al. Treatment of plastic bronchitis in a Fontan patient with tissue plasminogen activator: a case report and review of the literature. Pediatrics 2002; 109:e67.
  110. Gossett JG, Almond CS, Kirk R, et al. Outcomes of cardiac transplantation in single-ventricle patients with plastic bronchitis: a multicenter study. J Am Coll Cardiol 2013; 61:985.
  111. Valente AM, Bhatt AB, Cook S, et al. The CALF (Congenital Heart Disease in Adults Lower Extremity Systemic Venous Health in Fontan Patients) study. J Am Coll Cardiol 2010; 56:144.
  112. Bhatt AB, Landzberg MJ, Gerhard-Herman M, et al. Pathophysiology of chronic venous insufficiency in adults with a Fontan circulation (a pre-defined substudy of the CALF investigation). Int J Cardiol 2013; 165:41.
  113. Rabbitts JA, Groenewald CB, Mauermann WJ, et al. Outcomes of general anesthesia for noncardiac surgery in a series of patients with Fontan palliation. Paediatr Anaesth 2013; 23:180.
  114. Baumgartner H, Bonhoeffer P, De Groot NM, et al. ESC Guidelines for the management of grown-up congenital heart disease (new version 2010). Eur Heart J 2010; 31:2915.
  115. Graham TP Jr, Driscoll DJ, Gersony WM, et al. Task Force 2: congenital heart disease. J Am Coll Cardiol 2005; 45:1326.
  116. Drenthen W, Boersma E, Balci A, et al. Predictors of pregnancy complications in women with congenital heart disease. Eur Heart J 2010; 31:2124.
  117. Canobbio MM, Mair DD, van der Velde M, Koos BJ. Pregnancy outcomes after the Fontan repair. J Am Coll Cardiol 1996; 28:763.
  118. Hoare JV, Radford D. Pregnancy after fontan repair of complex congenital heart disease. Aust N Z J Obstet Gynaecol 2001; 41:464.
  119. Drenthen W, Pieper PG, Roos-Hesselink JW, et al. Pregnancy and delivery in women after Fontan palliation. Heart 2006; 92:1290.