Khairy P, Poirier N, Mercier L-A (2007) Univentricular heart. Circulation 115:800–812
Baumgartner H, de Backer J, Babu-Narayan SV, Budts W, Chessa M, Diller GP, Iung B, Kluin J, Lang IM, Meijboom F, Moons P, Mulder BJM, Oechslin E, Roos-Hesselink JW, Schwerzmann M, Sondergaard L, Zeppenfeld K, Windecker S, Aboyans V, Baigent C, Collet JP, Dean V, Delgado V, Fitzsimons D, Gale CP, Grobbee DE, Halvorsen S, Hindricks G, Iung B, Jüni P, Katus HA, Landmesser U, Leclercq C, Lettino M, Lewis BS, Merkely B, Mueller C, Petersen SE, Petronio AS, Richter DJ, Roffi M, Shlyakhto E, Simpson IA, Sousa-Uva M, Touyz RM (2021) 2020 ESC guidelines for the management of adult congenital heart disease. Eur Heart J 42:563–645
d’Udekem Y, Iyengar AJ, Cochrane AD, Grigg LE, Ramsay JM, Wheaton GR, Penny DJ, Brizard CP (2007) The Fontan procedure: contemporary techniques have improved long-term outcomes. Circulation 116:I157–I164
Dennis M, Zannino D, du Plessis K, Bullock A, Disney PJS, Radford DJ, Hornung T, Grigg L, Cordina R, d’Udekem Y, Celermajer DS (2018) Clinical outcomes in adolescents and adults after the Fontan procedure. J Am Coll Cardiol 71:1009–1017
Mahle WT, Todd K, Fyfe DA (2003) Endothelial function following the Fontan operation. Am J Cardiol 91:1286–1288
Baroutidou A, Arvanitaki A, Pagkopoulou E, Anyfanti P, Ziakas A, Kamperidis V, Giannakoulas G, Dimitroulas T (2025) Nailfold videocapillaroscopy as a non-invasive tool for the assessment of peripheral microangiopathy in cardiovascular diseases. J Hypertens 43:48–65
Baroutidou A, Dimitroulas T, Arvanitaki A, Grantza T, Otountzidis N, Farmakis IT, Karagiannidis AG, Kamperidis V, Ziakas A, Sarafidis P, Giannakoulas G (2024) Endothelial dysfunction in adults with congenital heart disease: a systematic review and meta-analysis. Eur J Clin Invest 55(5):e14376
Goldstein BH, Golbus JR, Sandelin AM, Warnke N, Gooding L, King KK, Donohue JE, Gurney JG, Goldberg CS, Rocchini AP, Charpie JR (2011) Usefulness of peripheral vascular function to predict functional health status in patients with fontan circulation. Am J Cardiol 108:428–434. https://doi.org/10.1016/j.amjcard.2011.03.064
Inai K, Saita Y, Takeda S, Nakazawa M, Kimura H (2004) Skeletal muscle hemodynamics and endothelial function in patients after Fontan operation. Am J Cardiol 93:792–797
Lambert E, D’Udekem Y, Cheung M, Sari CI, Inman J, Ahimastos A, Eikelis N, Pathak A, King I, Grigg L, Schlaich M, Lambert G (2013) Sympathetic and vascular dysfunction in adult patients with Fontan circulation. Int J Cardiol 167:1333–1338. https://doi.org/10.1016/j.ijcard.2012.04.015
Gewillig M, Brown SC (2016) The Fontan circulation after 45 years: update in physiology. Heart 102:1081–1086
Gewillig M, Cools B, Van De BA (2020) Pulmonary vascular reserve in Fontan patients: looking upstream for the true heart of the matter. J Am Coll Cardiol 76:2764–2767
Rychik J, Atz AM, Celermajer DS, Deal BJ, Gatzoulis MA, Gewillig MH, Hsia TY, Hsu DT, Kovacs AH, McCrindle BW, Newburger JW, Pike NA, Rodefeld M, Rosenthal DN, Schumacher KR, Marino BS, Stout K, Veldtman G, Younoszai AK, D’Udekem Y (2019) Evaluation and management of the child and adult with Fontan circulation: a scientific statement from the American heart association. Circulation 140(6):e234–e284
Gewillig M, Brown SC, van de Bruaene A, Rychik J (2020) Providing a framework of principles for conceptualising the Fontan circulation. Acta Paediatr Int J Paediatr 109:651–658
Becker K, Uebing A, Hansen JH (2021) Pulmonary vascular disease in Fontan circulation-is there a rationale for pulmonary vasodilator therapies? Cardiovasc Diagn Ther 11:1111–1121
PubMed PubMed Central Google Scholar
Zongtao Y, Huishan W, Zengwei W, Hongyu Z, Minhua F, Xinmin L, Nanbin Z, Hongguang H (2010) Experimental study of nonpulsatile flow perfusion and structural remodeling of pulmonary microcirculation vessels. Thorac Cardiovasc Surg 58:468–472
Khambadkone S, Li J, de Leval MR, Cullen S, Deanfield JE, Redington AN (2003) Basal pulmonary vascular resistance and nitric oxide responsiveness late after Fontan-type operation. Circulation 107:3204–3208
Vallance P, Chan N (2001) Endothelial function and nitric oxide: clinical relevance. Heart 85:342–350
CAS PubMed PubMed Central Google Scholar
Ridderbos FJS, Wolff D, Timmer A, van Melle JP, Ebels T, Dickinson MG, Timens W, Berger RMF (2015) Adverse pulmonary vascular remodeling in the Fontan circulation. J Hear LUNG Transplant 34:404–413
Kurotobi S, Sano T, Kogaki S, Matsushita T, Miwatani T, Takeuchi M, Matsuda H, Okada S (2001) Bidirectional cavopulmonary shunt with right ventricular outflow patency: the impact of pulsatility on pulmonary endothelial function. J Thorac Cardiovasc Surg 121:1161–1168
Henaine R, Vergnat M, Bacha EA, Baudet B, Lambert V, Belli E, Serraf A (2013) Effects of lack of pulsatility on pulmonary endothelial function in the Fontan circulation. J Thorac Cardiovasc Surg 146:522–529
Latus H, Lederle A, Khalil M, Kerst G, Schranz D, Apitz C (2019) Evaluation of pulmonary endothelial function in Fontan patients. J Thorac Cardiovasc Surg 158:523-531.e1
Constantine A, Dimopoulos K, Jenkins P, Tulloh RMR, Condliffe R, Jansen K, Chung NAY, Oliver J, Parry H, Fitzsimmons S, Walker N, Wort SJ, Papaioannou V, von Klemperer K, Clift P (2022) Use of pulmonary arterial hypertension therapies in patients with a fontan circulation: current practice across the united kingdom. J Am Heart Assoc 11(1):e023035
Cedars AM, Saef J, Peterson LR, Coggan AR, Novak EL, Kemp D, Ludbrook PA (2016) Effect of Ambrisentan on exercise capacity in adult patients after the Fontan procedure. Am J Cardiol 117:1524–1532. https://doi.org/10.1016/j.amjcard.2016.02.024
Giardini A, Balducci A, Specchia S, Gargiulo G, Bonvicini M, Picchio FM (2008) Effect of sildenafil on haemodynamic response to exercise and exercise capacity in Fontan patients. Eur Heart J 29:1681–1687
Goldberg DJ, Zak V, Goldstein BH, Schumacher KR, Rhodes J, Penny DJ, Petit CJ, Ginde S, Menon SC, Kim S-H, Kim GB, Nowlen TT, DiMaria MV, Frischhertz BP, Wagner JB, McHugh KE, McCrindle BW, Shillingford AJ, Sabati AA, Yetman AT, John AS, Richmond ME, Files MD, Payne RM, Mackie AS, Davis CK, Shahanavaz S, Hill KD, Garg R, Jacobs JP, Hamstra MS, Woyciechowski S, Rathge KA, McBride MG, Frommelt PC, Russell MW, Urbina EM, Yeager JL, Pemberton VL, Stylianou MP, Pearson GD, Paridon SM (2020) Results of the FUEL trial. Circulation 141:641–651
Rhodes J, Ubeda-Tikkanen A, Clair M, Fernandes SM, Graham DA, Milliren CE, Daly KP, Mullen MP, Landzberg MJ (2013) Effect of inhaled iloprost on the exercise function of Fontan patients: a demonstration of concept. Int J Cardiol 168:2435–2440
PubMed PubMed Central Google Scholar
Hebert A, Mikkelsen UR, Thilen U, Idorn L, Jensen AS, Nagy E, Hanseus K, Sørensen KE, Søndergaard L (2014) 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) stud. Circulation 130:2021–2030
Schuuring MJ, Vis JC, van Dijk APJ, van Melle JP, Vliegen HW, Pieper PG, Sieswerda GT, de Bruin-Bon R, Mulder BJM, Bouma BJ (2013) Impact of bosentan on exercise capacity in adults after the Fontan procedure: a randomized controlled trial. Eur J Heart Fail 15:690–698
Goldberg DJ, French B, McBride MG, Marino BS, Mirarchi N, Hanna BD, Wernovsky G, Paridon SM, Rychik J (2011) Impact of oral sildenafil on exercise performance in children and young adults after the Fontan operation a randomized, double-blind, placebo-controlled. Crossover Trial Circ 123:1185–1193
Kosmidis D, Arvanitaki A, Farmakis IT, Liakos A, Giannopoulos A, Ziakas A, Giannakoulas G (2024) Pulmonary vasodilators and exercise in Fontan circulation: a systematic review and meta-analysis. Heart 110:552–559
Brown JW, Ruzmetov M, Vijay P, Rodefeld MD, Turrentine MW (2005) Pulmonary arteriovenous malformations in children after the Kawashima operation. Ann Thorac Surg 80:1592–1596
Henaine R, Vergnat M, Mercier O, Serraf A, De MV, Ninet J, Bacha EA (2013) Hemodynamics and arteriovenous malformations in cavopulmonary anastomosis: the case for residual antegrade pulsatile flow. J Thorac Cardiovasc Surg 146:1359–1365. https://doi.org/10.1016/j.jtcvs.2013.02.036
Vettukattil JJ (2017) Is the hepatic factor a mirna that maintains the integrity of pulmonary microvasculature by inhibiting the vascular endothelial growth factor? Curr Cardiol Rev 13:244–250
CAS PubMed PubMed Central Google Scholar
Field-Ridley A, Heljasvaara R, Pihlajaniemi T, Adatia I, Sun C, Keller RL, Gong WH, Datar S, Oishi P, Fineman JR (2013) Endostatin, an inhibitor of angiogenesis, decreases after bidirectional superior cavopulmonary anastamosis. Pediatr Cardiol 34:291–295
Krimly A, Jain CC, Egbe A, Alzahrani A, Al NK, Albert-Brotons D, Veldtman GR (2021) The pulmonary vascular bed in patients with functionally univentricular physiology and a Fontan circulation. Cardiol Young 31:1241–1250
Inai K, Nakanishi T, Nakazawa M (2005) Clinical correlation and prognostic predictive value of neurohumoral factors in patients late after the Fontan operation. Am Heart J 150:588–594
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