Fontan circulation has improved life expectancy for infants born with complex heart disease over the last 50 years but has also resulted in significant morbidity.
Fontan circulation
life expectancy
morbidity
surgery
univentricular heart malformation
Journal
Acta paediatrica (Oslo, Norway : 1992)
ISSN: 1651-2227
Titre abrégé: Acta Paediatr
Pays: Norway
ID NLM: 9205968
Informations de publication
Date de publication:
Jan 2022
Jan 2022
Historique:
revised:
22
06
2021
received:
22
04
2021
accepted:
06
07
2021
pubmed:
9
7
2021
medline:
28
1
2022
entrez:
8
7
2021
Statut:
ppublish
Résumé
The prognosis for infants born with complex heart disease improved dramatically with the introduction of the Fontan circulation 50 years ago. With today's carefully designed and staged operations to a Fontan circulation, life expectancy has increased and most children will survive into adult life. The Fontan circulation entails an unphysiological circulation with high risk for multiple organ system dysfunction. Neurodevelopmental disabilities with adverse psychosocial effects are prevalent. The Fontan circulation may eventually fail and necessitate heart transplantation. CONCLUSION: Fifty years development of the Fontan circulation to today's staged surgical procedures has improved survival but also revealed the burden of a high morbidity for a growing number of patients.
Types de publication
Journal Article
Review
Langues
eng
Sous-ensembles de citation
IM
Pagination
11-16Informations de copyright
© 2021 The Authors. Acta Paediatrica published by John Wiley & Sons Ltd on behalf of Foundation Acta Paediatrica.
Références
Fontan F, Baudet E. Surgical repair of tricuspid atresia. Thorax. 1971;26:240-248.
Poh CL, Zannino D, Weintraub RG, et al. Three decades later: The fate of the population of patients who underwent the Atriopulmonary Fontan procedure. Int J Cardiol. 2017;231:99-104.
Bjork VO, Olin CL, Bjarke BB, Thoren CA. Right atrial-right ventricular anastomosis for correction of tricuspid atresia. J Thorac Cardiovasc Surg. 1979;77:452-458.
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;96:682-695.
Restrepo M, Crouch AC, Haggerty CM, et al. Hemodynamic impact of superior vena cava placement in the y-graft Fontan connection. Ann Thorac Surg. 2016;101:183-189.
Bakker MK, Bergman JEH, Krikov S, et al. Prenatal diagnosis and prevalence of critical congenital heart defects: an international retrospective cohort study. BMJ Open. 2019;9:e028139.
Corno AF. "Functionally" univentricular hearts: impact of pre-natal diagnosis. Front Pediatr. 2015;3:15.
Idorn L, Olsen M, Jensen AS, et al. Univentricular hearts in Denmark 1977 to 2009: incidence and survival. Int J Cardiol. 2013;167:1311-1316.
Liu MY, Zielonka B, Snarr BS, Zhang X, Gaynor JW, Rychik J. Longitudinal assessment of outcome from prenatal diagnosis through Fontan operation for over 500 fetuses with single ventricle-type congenital heart disease: the Philadelphia Fetus-to-Fontan cohort study. J Am Heart Assoc. 2018;7:e009145.
McGovern E, Kelleher E, Potts JE, et al. Predictors of poor outcome among children with heterotaxy syndrome: a retrospective review. Open Heart. 2016;3:e000328.
de Leval MR, McKay R, Jones M, Stark J, Macartney FJ. Modified Blalock-Taussig shunt. Use of subclavian artery orifice as flow regulator in prosthetic systemic-pulmonary artery shunts. J Thorac Cardiovasc Surg. 1981;81:112-119.
Taussig H, Blalock A. Surgery of congenital heart disease. Br Med J. 1947;2:462.
Ohye RG, Schranz D, D'Udekem Y. Current therapy for hypoplastic left heart syndrome and related single ventricle lesions. Circulation. 2016;134:1265-1279.
Schranz D. High risk HLHS: hybrid approach yes, but how does it work? Transl Pediatr. 2018;7:222-225.
Sano S, Ishino K, Kawada M, et al. Right ventricle-pulmonary artery shunt in first-stage palliation of hypoplastic left heart syndrome. J Thorac Cardiovasc Surg. 2003;126:504-509; discussion 509-510.
Feinstein JA, Benson DW, Dubin AM, et al. Hypoplastic left heart syndrome: current considerations and expectations. J Am Coll Cardiol. 2012;59:S1-42.
Norwood WI, Kirklin JK, Sanders SP. Hypoplastic left heart syndrome: experience with palliative surgery. Am J Cardiol. 1980;45:87-91.
Edwards L, Morris KP, Siddiqui A, Harrington D, Barron D, Brawn W. Norwood procedure for hypoplastic left heart syndrome: BT shunt or RV-PA conduit? Arch Dis Child Fetal Neonatal Ed. 2007;92:F210-214.
Gewillig M, Brown SC. The Fontan circulation after 45 years: update in physiology. Heart. 2016;102:1081-1086.
Gewillig M, Brown SC, Eyskens B, et al. The Fontan circulation: who controls cardiac output? Interact Cardiovasc Thorac Surg. 2010;10:428-433.
Gewillig M, Brown SC, van de Bruaene A, Rychik J. Providing a framework of principles for conceptualising the Fontan circulation. Acta Paediatr. 2020;109:651-658.
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;140(6):CIR0000000000000696.
Mohanakumar S, Telinius N, Kelly B, et al. Morphology and function of the lymphatic vasculature in patients with a Fontan circulation. Circ Cardiovasc Imaging. 2019;12:e008074.
Udholm S, Aldweib N, Hjortdal VE, Veldtman GR. Prognostic power of cardiopulmonary exercise testing in Fontan patients: a systematic review. Open Heart. 2018;5:e000812.
Goldberg DJ, Avitabile CM, McBride MG, Paridon SM. Exercise capacity in the Fontan circulation. Cardiol Young. 2013;23:824-830.
Hedlund ER, Lundell B, Soderstrom L, Sjoberg G. Can endurance training improve physical capacity and quality of life in young Fontan patients? Cardiol Young. 2018;28:438-446.
Hedlund ER, Lundell B, Villard L, Sjoberg G. Reduced physical exercise and health-related quality of life after Fontan palliation. Acta Paediatr. 2016;105:1322-1328.
Holbein CE, Veldtman GR, Moons P, et al. Perceived health mediates effects of physical activity on quality of life in patients with a Fontan circulation. Am J Cardiol. 2019;124:144-150.
Scheffers LE, Berg LEV, Ismailova G, Dulfer K, Takkenberg JJ, Helbing WA. Physical exercise training in patients with a Fontan circulation: a systematic review. Eur J Prev Cardiol. 2020:2047487320942869. online ahead of print.
Gaynor JW, Ittenbach RF, Gerdes M, et al. Neurodevelopmental outcomes in preschool survivors of the Fontan procedure. J Thorac Cardiovasc Surg. 2014;147:1276-1282. discussion 1282-1283 e1275.
Sarajuuri A, Jokinen E, Mildh L, et al. Neurodevelopmental burden at age 5 years in patients with univentricular heart. Pediatrics. 2012;130:e1636-1646.
Siciliano RE, Prussien KV, Lee CA, et al. Cognitive function in pediatric hypoplastic left heart syndrome: systematic review and meta-analysis. J Pediatr Psychol. 2019;44:937-947.
DeMaso DR, Calderon J, Taylor GA, et al. Psychiatric disorders in adolescents with single ventricle congenital heart disease. Pediatrics. 2017;139(3):e20162241.
Wittekind S, Mays W, Gerdes Y, et al. A novel mechanism for improved exercise performance in pediatric Fontan patients after cardiac rehabilitation. Pediatr Cardiol. 2018;39:1023-1030.
Laohachai K, Winlaw D, Selvadurai H, et al. Inspiratory muscle training is associated with improved inspiratory muscle strength, resting cardiac output, and the ventilatory efficiency of exercise in patients with a Fontan circulation. J Am Heart Assoc. 2017;6(8):e005750.
Alsaied T, Bokma JP, Engel ME, et al. Factors associated with long-term mortality after Fontan procedures: a systematic review. Heart. 2017;103:104-110.
Jenkins PC, Chinnock RE, Jenkins KJ, et al. Decreased exercise performance with age in children with hypoplastic left heart syndrome. J Pediatr. 2008;152:507-512.
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;27:552-559.
Gewillig M, Cools B, Van De Bruaene A. Pulmonary vascular reserve in Fontan patients: looking upstream for the true heart of the matter. J Am Coll Cardiol. 2020;76:2764-2767.
Snarr BS, Paridon SM, Rychik J, Goldberg DJ. Pulmonary vasodilator therapy in the failing Fontan circulation: rationale and efficacy. Cardiol Young. 2015;25:1489-1492.
Budts W, Ravekes WJ, Danford DA, Kutty S. Diastolic heart failure in patients with the Fontan circulation: a review. JAMA Cardiol. 2020;5:590-597.
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:E230-236.
Goldstein BH, Connor CE, Gooding L, Rocchini AP. Relation of systemic venous return, pulmonary vascular resistance, and diastolic dysfunction to exercise capacity in patients with single ventricle receiving Fontan palliation. Am J Cardiol. 2010;105:1169-1175.
Rijnberg FM, Blom NA, Sojak V, et al. A 45-year experience with the Fontan procedure: tachyarrhythmia, an important sign for adverse outcome. Interact Cardiovasc Thorac Surg. 2019;29:461-468.
Rychik J. Protein-losing enteropathy after Fontan operation. Congenit Heart Dis. 2007;2:288-300.
Harteveld LM, Blom NA, Hazekamp MG, Ten Harkel ADJ. Treatment and outcome of plastic bronchitis in single ventricle patients: a systematic review. Interact Cardiovasc Thorac Surg. 2020;30:846-853.
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;94:996-1002.
Gordon-Walker TT, Bove K, Veldtman G. Fontan-associated liver disease: a review. J Cardiol. 2019;74:223-232.
Pundi K, Pundi KN, Kamath PS, et al. Liver disease in patients after the Fontan operation. Am J Cardiol. 2016;117:456-460.
Zafar F, Lubert AM, Katz DA, et al. Long-term kidney function after the Fontan operation: JACC review topic of the week. J Am Coll Cardiol. 2020;76:334-341.
Attard C, Huang J, Monagle P, Ignjatovic V. Pathophysiology of thrombosis and anticoagulation post Fontan surgery. Thromb Res. 2018;172:204-213.
McCormick AD, Schumacher KR. Transplantation of the failing Fontan. Transl Pediatr. 2019;8:290-301.
Mery CM, De Leon LE, Trujillo-Diaz D, et al. Contemporary outcomes of the Fontan operation: a large single-institution cohort. Ann Thorac Surg. 2019;108:1439-1446.
Kverneland LS, Kramer P, Ovroutski S. Five decades of the Fontan operation: A systematic review of international reports on outcomes after univentricular palliation. Congenit Heart Dis. 2018;13:181-193.
Schwartz I, McCracken CE, Petit CJ, Sachdeva R. Late outcomes after the Fontan procedure in patients with single ventricle: a meta-analysis. Heart. 2018;104:1508-1514.
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:S32-38.
Miller JR, Lancaster TS, Callahan C, Abarbanell AM, Eghtesady P. An overview of mechanical circulatory support in single-ventricle patients. Transl Pediatr. 2018;7:151-161.