Single Ventricle Fontan: The Basics for General Cardiologists
References
- Jacobs JP, Franklin RCG, Béland MJ, et al. Nomenclature for Pediatric and Congenital Cardiac Care: Unification of Clinical and Administrative Nomenclature - The 2021 International Paediatric and Congenital Cardiac Code (IPCCC) and the Eleventh Revision of the International Classification of Diseases (ICD-11). Cardiol Young. 2021 Jul;31(7):1057-1188. doi: 10.1017/S104795112100281X
- Jacobs ML, Anderson RH. Nomenclature of the functionally univentricular heart. Cardiol Young. 2006 Feb;16 Suppl 1:3-8. doi: 10.1017/S104795110500226X
- Hoffman JI, Kaplan S. The incidence of congenital heart disease. J Am Coll Cardiol. 2002 Jun 19;39(12):1890-1900. doi: 10.1016/s0735-1097(02)01886-7
- Blue GM, Kirk EP, Sholler GF, Harvey RP, Winlaw DS. Congenital heart disease: current knowledge about causes and inheritance. Med J Aust. 2012 Aug 6;197(3):155-159. doi: 10.5694/mja12.10811
- Gewillig M, Brown SC. The Fontan circulation after 45 years: update in physiology. Heart. 2016 Jul 15;102(14):1081-1086. doi: 10.1136/heartjnl-2015-307467
- 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 Sep 9;130(11 Suppl 1):S32-S38. doi: 10.1161/CIRCULATIONAHA.113.007764
- Fusco F, Shimada E, Scognamiglio G, et al. Restrictive ventricular septal defect resulting in systemic outflow obstruction in adults with Fontan circulation: challenging diagnosis of a serious and potentially fatal complication. J Cardiovasc Med (Hagerstown). 2020 Mar;21(3):276-279. doi: 10.2459/JCM.0000000000000903
- Fontan F, Baudet E. Surgical repair of tricuspid atresia. Thorax. 1971 May;26(3):240-248. doi: 10.1136/thx.26.3.240
- Freedom RM, Nykanen D, Benson LN. The physiology of the bidirectional cavopulmonary connection. Ann Thorac Surg. 1998 Aug;66(2):664-667. doi: 10.1016/s0003-4975(98)00618-3
- Dennis M, Zannino D, du Plessis K, et al. Clinical Outcomes in Adolescents and Adults After the Fontan Procedure. J Am Coll Cardiol. 2018 Mar 6;71(9):1009-1017. doi: 10.1016/j.jacc.2017.12.054
- Montanaro C, Arvanitaki A, Kerrigan W, et al. The Fontan Adult Brompton clinical score and mortality risk. Eur Heart J. 2025 May 21;46(20):1933-1945. doi: 10.1093/eurheartj/ehaf137
- Gurvitz M, Krieger EV, Fuller S, et al. 2025 ACC/AHA/HRS/ISACHD/SCAI Guideline for the Management of Adults With Congenital Heart Disease: A Report of the American College of Cardiology/American Heart Association Joint Committee on Clinical Practice Guidelines. J Am Coll Cardiol. 2026 Feb 24;87(7):822-976. doi: 10.1016/j.jacc.2025.09.006
- Kamp AN, Nair K, Fish FA, Khairy P. Catheter Ablation of Atrial Arrhythmias in Patients Post-Fontan. Can J Cardiol. 2022 Jul;38(7):1036-1047. doi: 10.1016/j.cjca.2022.02.023
- Moore BM, Anderson R, Nisbet AM, et al. Ablation of Atrial Arrhythmias After the Atriopulmonary Fontan Procedure: Mechanisms of Arrhythmia and Outcomes. JACC Clin Electrophysiol. 2018 Oct;4(10):1338-1346. doi: 10.1016/j.jacep.2018.08.012
- Houck CA, de Groot NMS, Kardys I, Niehot CD, Bogers AJJC, Mouws EMJP. Outcomes of Atrial Arrhythmia Surgery in Patients With Congenital Heart Disease: A Systematic Review. J Am Heart Assoc. 2020 Oct 20;9(19):
e016921 . doi: 10.1161/JAHA.120.016921 - Poh CL, Celermajer DS, Grigg LE, et al. Pacemakers are associated with a higher risk of late death and transplantation in the Fontan population. Int J Cardiol. 2019 May 1;282:33-37. doi: 10.1016/j.ijcard.2019.01.088
- Egbe AC, Huntley GD, Connolly HM, et al. Outcomes of cardiac pacing in adult patients after a Fontan operation. Am Heart J. 2017 Dec;194:92-98. doi: 10.1016/j.ahj.2017.08.019
- Al-Jazairi AS, Al Alshaykh HA, Di Salvo G, De Vol EB, Alhalees ZY. Assessment of Late Thromboembolic Complications Post-Fontan Procedure in Relation to Different Antithrombotic Regimens: 30-Years’ Follow-up Experience. Ann Pharmacother. 2019 Aug;53(8):786-793. doi: 10.1177/1060028019829860
- Kaulitz R, Ziemer G, Rauch R, et al. Prophylaxis of thromboembolic complications after the Fontan operation (total cavopulmonary anastomosis). J Thorac Cardiovasc Surg. 2005 Mar;129(3):569-575. doi: 10.1016/j.jtcvs.2004.08.045
- Seipelt RG, Franke A, Vazquez-Jimenez JF, et al. Thromboembolic complications after Fontan procedures: comparison of different therapeutic approaches. Ann Thorac Surg. 2002 Aug;74(2):556-562. doi: 10.1016/s0003-4975(02)03677-9
- Yang H, Veldtman GR, Bouma BJ, et al. Non-vitamin K antagonist oral anticoagulants in adults with a Fontan circulation: are they safe. Open Heart. 2019 Jun 3;6(1):
e000985 . doi: 10.1136/openhrt-2018-000985 - Kawamatsu N, Ishizu T, Machino-Ohtsuka T, et al. Direct oral anticoagulant use and outcomes in adult patients with Fontan circulation: A multicenter retrospective cohort study. Int J Cardiol. 2021 Mar 15;327:74-79. doi: 10.1016/j.ijcard.2020.11.024
- Rubin BK. Plastic Bronchitis. Clin Chest Med. 2016 Sep;37(3):405-408. doi: 10.1016/j.ccm.2016.04.003
- 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 May;115(5):1063-1073. doi: 10.1016/s0022-5223(98)70406-4
- Ostrow AM, Freeze H, Rychik J. Protein-losing enteropathy after Fontan operation: investigations into possible pathophysiologic mechanisms. Ann Thorac Surg. 2006 Aug;82(2):695-700. doi: 10.1016/j.athoracsur.2006.02.048
- Menon S, Chennapragada M, Ugaki S, Sholler GF, Ayer J, Winlaw DS. The lymphatic circulation in adaptations to the Fontan circulation. Pediatr Cardiol. 2017 Jun;38(5):886-892. doi: 10.1007/s00246-017-1576-y.
- Itkin M, Piccoli DA, Nadolski G, et al. Protein-losing enteropathy in patients with congenital heart disease. J Am Coll Cardiol. 2017 Jun 20;69(24):2929-2937. doi: 10.1016/j.jacc.2017.04.023.
- Barracano R, Merola A, Fusco F, Scognamiglio G, Sarubbi B. Protein-losing enteropathy in Fontan circulation: Pathophysiology, outcome and treatment options of a complex condition. Int J Cardiol Congenit Heart Dis. 2022 Jan 5;7:100322. doi: 10.1016/j.ijcchd.2022.100322
- Kewcharoen J, Mekraksakit P, Limpruttidham N, et al. Budesonide for Protein Losing Enteropathy in Patients with Fontan Circulation: A Systematic Review and Meta-Analysis. World J Pediatr Congenit Heart Surg. 2020 Jan;11(1):85-91. doi: 10.1177/2150135119872196
- 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 Dec;84(6):2110-2112. doi: 10.1016/j.athoracsur.2007.06.064
- 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 Nov-Dec;6(6):653-656. doi: 10.1111/j.1747-0803.2011.00518.x
- Weingarten AJ, Menachem JN, Smith CA, Frischhertz BP, Book WM. Usefulness of midodrine in protein-losing enteropathy. J Heart Lung Transplant. 2019 Jul;38(7):784-787. doi: 10.1016/j.healun.2019.04.002
- Maleux G, Storme E, Cools B, et al. Percutaneous embolization of lymphatic fistulae as treatment for protein-losing enteropathy and plastic bronchitis in patients with failing Fontan circulation. Catheter Cardiovasc Interv. 2019 Dec 1;94(7):996-1002. doi: 10.1002/ccd.28501
- Zaltsberg GS, Lam CZ, Ling SC, et al. Magnetic Resonance Liver Lymphangiography for Investigation and Transhepatic Lymphatic Embolization for the Treatment of Protein-Losing Enteropathy. J Vasc Interv Radiol. 2021 Feb;32(2):327-329.e2. doi: 10.1016/j.jvir.2020.10.012.
- Fusco F, Li W.
Echocardiography and the Adult Fontan Patient . In: Clift P, Dimopoulos K, Angelini A, editors. Univentricular Congenital Heart Defects: Practical Manual for Patient Management: Practical Manual for Patient Management. Berlin, Germany: Springer Nature; 2024. 937 p. - Sandler KL, Markham LW, Mah ML, et al. Optimizing CT angiography in patients with Fontan physiology: single-center experience of dual-site power injection. Clin Radiol. 2014 Dec;69(12):e562-e567. doi: 10.1016/j.crad.2014.09.011
- Agasthi P, Jain CC, Egbe AC, et al. Clinical Outcomes of Percutaneous Fontan Stenting in Adults. Can J Cardiol. 2023 Oct;39(10):1358-1365. doi: 10.1016/j.cjca.2023.04.023
- Hagler DJ, Miranda WR, Haggerty BJ, et al. Fate of the Fontan connection: Mechanisms of stenosis and management. Congenit Heart Dis. 2019 Jul;14(4):571-581. doi: 10.1111/chd.12757
- Téllez L, Rodríguez de Santiago E, Albillos A. Fontan-Associated Liver Disease: Pathophysiology, Staging, and Management. Semin Liver Dis. 2021 Nov;41(4):538-550. doi: 10.1055/s-0041-1732355
- Rosenthal BE, Hoteit MA, Lluri G, et al. Characteristics and Survival Outcomes of Hepatocellular Carcinoma After the Fontan Operation. JACC Adv. 2025 Apr;4(4):101646. doi: 10.1016/j.jacadv.2025.101646
- Munsterman ID, Duijnhouwer AL, Kendall TJ, et al; Nijmegen Fontan Initiative. The clinical spectrum of Fontan-associated liver disease: results from a prospective multimodality screening cohort. Eur Heart J. 2019 Apr 1;40(13):1057-1068. doi: 10.1093/eurheartj/ehy620
- Lewis MJ, Reardon LC, Aboulhosn J, et al. Clinical Outcomes of Adult Fontan-Associated Liver Disease and Combined Heart-Liver Transplantation. J Am Coll Cardiol. 2023 Jun 6;81(22):2149-2160. doi: 10.1016/j.jacc.2023.03.421
- Bartoli CR, Hennessy-Strahs S, Dowling RD, Gaynor JW, Glatz AC. Abnormalities in the Von Willebrand-Angiopoietin Axis Contribute to Dysregulated Angiogenesis and Angiodysplasia in Children With a Glenn Circulation. JACC Basic Transl Sci. 2021 Mar 22;6(3):222-235. doi: 10.1016/j.jacbts.2020.12.014
- Greenleaf CE, Lim ZN, Li W, LaPar DJ, Salazar JD, Corno AF. Impact on clinical outcomes from transcatheter closure of the Fontan fenestration: A systematic review and meta-analysis. Front Pediatr. 2022 Oct 4;10:915045. doi: 10.3389/fped.2022.915045
- Nederend M, Egorova AD, van der Kley F, et al. Percutaneous closure of veno-venous collaterals in adult patients with univentricular physiology after Fontan palliation: Single centre experience and systematic review. Int J Cardiol Congenit Heart Dis. 2023 Oct 11;14:100479. doi: 10.1016/j.ijcchd.2023.100479
- Dori Y, Glatz AC, Hanna BD, et al. Acute effects of embolizing systemic-to-pulmonary arterial collaterals on blood flow in patients with superior cavopulmonary connections: a pilot study. Circ Cardiovasc Interv. 2013 Feb;6(1):101-106. doi: 10.1161/CIRCINTERVENTIONS.112.972265
- Krishnan US, Lamour JM, Hsu DT, Kichuk MR, Donnelly CM, Addonizio LJ. Management of aortopulmonary collaterals in children following cardiac transplantation for complex congenital heart disease. J Heart Lung Transplant. 2004 May;23(5):564-569. doi: 10.1016/S1053-2498(03)00305-X
- Fusco F, Scognamiglio G, Merola A, et al. Safety and Efficacy of Sacubitril/Valsartan in Patients With a Failing Systemic Right Ventricle: A Prospective Single-Center Study. Circ Heart Fail. 2023 Feb;16(2):
e009848 . doi: 10.1161/CIRCHEARTFAILURE.122.009848 - Neijenhuis RML, Nederend M, van Groningen AE, et al. Sacubitril/valsartan is associated with improvements in quality of life in adult congenital heart disease patients with systemic right ventricular failure. Open Heart. 2025 Jan 30;12(1):
e003009 . doi: 10.1136/openhrt-2024-003009 - Neijenhuis RML, MacDonald ST, Zemrak F, et al. Effect of Sodium-Glucose Cotransporter 2 Inhibitors in Adults With Congenital Heart Disease. J Am Coll Cardiol. 2024 Apr 16;83(15):1403-1414. doi: 10.1016/j.jacc.2024.02.017
- Fusco F, Scognamiglio G, Abbate M, et al. Dapagliflozin in Patients With a Failing Systemic Right Ventricle: Results From the DAPA-SERVE Trial. JACC Heart Fail. 2024 Apr;12(4):789-791. doi: 10.1016/j.jchf.2024.01.006
- Bright C, Rizvi A, Ezekwueme F, et al. Impact of guideline directed medical therapy on myocardial function in adults with congenital heart disease. Int J Cardiol. 2024 Nov 1;414:132413. doi: 10.1016/j.ijcard.2024.132413
- Neijenhuis RML, Regeer MV, Walker NL, et al. Echocardiographic effects of sodium-glucose cotransporter 2 inhibitors in single ventricle circulatory failure. Int J Cardiol Congenit Heart Dis. 2025 Jun 21;21:100603. doi: 10.1016/j.ijcchd.2025.100603
- Konduri A, West C, Lowery R, et al. Experience with SGLT2 Inhibitors in Patients with Single Ventricle Congenital Heart Disease and Fontan Circulatory Failure. Pediatr Cardiol. 2025 Jan;46(1):81-88. doi: 10.1007/s00246-023-03332-5
- Goldberg DJ, Zak V, Goldstein BH, et al. Results of the FUEL Trial. Circulation. 2020 Feb 25;141(8):641-651. doi: 10.1161/CIRCULATIONAHA.119.044352. Epub 2019 Nov 17. Erratum in: Circulation. 2020 Jul 14;142(2):
e31 . doi: 10.1161/CIR.0000000000000882 - 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 Mar 22;123(11):1185-1193. doi: 10.1161/CIRCULATIONAHA.110.981746
- Dipchand AI, Honjo O, Alonso-Gonzalez R, McDonald M, Roche SL. Heart Transplant Indications, Considerations, and Outcomes in Fontan Patients: Age-Related Nuances, Transplant Listing, and Disease-Specific Indications. Can J Cardiol. 2022 Jul;38(7):1072-1085. doi: 10.1016/j.cjca.2022.02.019
- Schumacher KR, Rosenthal DN, Batazzi A, et al. The Impact of Fontan Circulatory Failure on Heart Transplant Survival: A 20-Center Retrospective Cohort Study. Circulation. 2025 Aug 26;152(8):508-518. doi: 10.1161/CIRCULATIONAHA.124.072961
- Cedars A, Kutty S, Danford D, Schumacher K; ACTION Learning Network Investigators. Systemic ventricular assist device support in Fontan patients: A report by ACTION. J Heart Lung Transplant. 2021 May;40(5):368-376. doi: 10.1016/j.healun.2021.01.011. Epub 2021 Jan 19. Erratum in: J Heart Lung Transplant. 2021 Dec;40(12):1685. doi: 10.1016/j.healun.2021.09.012
- Bedzra EKS, Adachi I, Peng DM, et al. Society of Thoracic Surgeons Fontan VAD Group. Systemic ventricular assist device support of the Fontan circulation yields promising outcomes: An analysis of The Society of Thoracic Surgeons Pedimacs and Intermacs Databases. J Thorac Cardiovasc Surg. 2022 Aug;164(2):353-364. doi: 10.1016/j.jtcvs.2021.11.054
- Sughimoto K, Hirata Y, Hirahara N, et al. Mid-term result of atrioventricular valve replacement in patients with a single ventricle. Interact Cardiovasc Thorac Surg. 2018 Dec 1;27(6):895-900. doi: 10.1093/icvts/ivy155
- Liu VJ, Yong MS, d’Udekem Y, et al. Outcomes of atrioventricular valve operation in patients with Fontan circulation. Ann Thorac Surg. 2015 May;99(5):1632-1638. doi: 10.1016/j.athoracsur.2015.01.035
- Opotowsky AR, Carazo M, Singh MN, et al. Creatinine versus cystatin C to estimate glomerular filtration rate in adults with congenital heart disease: Results of the Boston Adult Congenital Heart Disease Biobank. Am Heart J. 2019 Aug;214:142-155. doi: 10.1016/j.ahj.2019.04.018
- Egbe AC, Driscoll DJ, Khan AR, et al. Cardiopulmonary exercise test in adults with prior Fontan operation: The prognostic value of serial testing. Int J Cardiol. 2017 May 15;235:6-10. doi: 10.1016/j.ijcard.2017.02.140
- Alsaied T, Li R, Christopher AB, et al; FORCE Investigators. High-Performing Fontan Patients: A Fontan Outcome Registry by Cardiac Magnetic Resonance Imaging Study. JACC Adv. 2024 Sep 9;3(10):101254. doi: 10.1016/j.jacadv.2024.101254
- Arvanitaki A, Frigiola A, Iannaccone G, Montanaro C. Benefit of Exercise in Patients with a Fontan Circulation. Int J Cardiol. 2023 Dec 1;392:131288. doi: 10.1016/j.ijcard.2023.131288
- Herrmann JE, Selamet Tierney ES. Exercise Capacity and Training Programs in Paediatric Fontan Patients: A Systematic Review. CJC Pediatr Congenit Heart Dis. 2022 May 6;1(3):108-118. doi: 10.1016/j.cjcpc.2022.04.005
- Fusco F, Scognamiglio G, Merola A, et al. Coronavirus disease 2019 in patients with Fontan circulation. Int J Cardiol Congenit Heart Dis. 2021 May;3:100126. doi: 10.1016/j.ijcchd.2021.100126
- Heidecker B, Libby P, Vassiliou VS, et al. Vaccination as a new form of cardiovascular prevention: a European Society of Cardiology clinical consensus statement. Eur Heart J. 2025 Sep 22;46(36):3518-3531. doi: 10.1093/eurheartj/ehaf384
- Fusco F, Scognamiglio G, Merola A, et al. COVID-19 vaccination in adults with congenital heart disease: Real-world data from an Italian tertiary centre. Int J Cardiol Congenit Heart Dis. 2021 Dec;6:100266. doi: 10.1016/j.ijcchd.2021.100266
- Fusco F, Scognamiglio G, Roma AS, et al. Mid-term follow-up after COVID-19 vaccination in adults with CHD: a prospective study. Cardiol Young. 2023 Dec;33(12):2574-2580. doi: 10.1017/S1047951123000689
- Montanaro C, Dahill K, Frigiola A, Boyle S, Gatzoulis MA. The Fontan-infective endocarditis paradox: should patients with a Fontan circulation be counselled for antibiotic prophylaxis? Eur J Prev Cardiol. 2024 May 11;31(7):e47-e48. doi: 10.1093/eurjpc/zwad260
- Moons P, Van Bulck L, Daelman B, Luyckx K. Mental health in adult congenital heart disease. Int J Cardiol Congenit Heart Dis. 2023 Apr 1;12:100455. doi: 10.1016/j.ijcchd.2023.100455
- Fusco F, Scognamiglio G, Sarubbi B. Living a Full Life With Half of a Heart: A Congenital Heart Disease Patient’s Perspective. JACC Case Rep. 2023 Oct 19;26:102076. doi: 10.1016/j.jaccas.2023.102076
- De Backer J, Haugaa KH, Hasselberg NE, et al; ESC Scientific Document Group. 2025 ESC Guidelines for the management of cardiovascular disease and pregnancy. Eur Heart J. 2025 Nov 14;46(43):4462-4568. doi: 10.1093/eurheartj/ehaf193. Erratum in: Eur Heart J. 2026 Feb 18;47(7):812. doi: 10.1093/eurheartj/ehaf1011
- Baró-Mariné F, Pijuan-Domènech A, Goya MDM, et al. Progestogen only contraception in women with congenital heart disease. J Obstet Gynaecol. 2024 Dec;44(1):2320296. doi: 10.1080/01443615.2024.2320296
- van Dissel AC, Moons P, Gallego P, et al. Daily issues in the life of adults with congenital heart disease: new insights from EURO-ACHD Congress 2025. Eur Heart J Suppl. 2026 Jan 30;28(Suppl 1):i17-i24. doi: 10.1093/eurheartjsupp/suaf087
DOI: https://doi.org/10.14797/mdcvj.1816 | Journal eISSN: 1947-6108
Language: English
Page range: 54 - 66
Submitted on: Mar 3, 2026
Accepted on: Jun 3, 2026
Published on: Jun 30, 2026
Published by: Houston Methodist DeBakey Heart & Vascular Center
In partnership with: Paradigm Publishing Services
Keywords:
© 2026 Berardo Sarubbi, Flavia Fusco, Michela Palma, Rosaria Barracano, Giovanni Domenico Ciriello, Nunzia Borrelli, Giancarlo Scognamiglio, published by Houston Methodist DeBakey Heart & Vascular Center
This work is licensed under the Creative Commons Attribution-NonCommercial 4.0 License.