Hostname: page-component-7dd5485656-j7khd Total loading time: 0 Render date: 2025-10-28T21:21:54.541Z Has data issue: false hasContentIssue false
  • English
  • Français

Exercise capacity in the Fontan circulation

Published online by Cambridge University Press:  09 January 2014

David J. Goldberg ,
Catherine M. Avitabile ,
Michael G. McBride  and
Stephen M. Paridon
David J. Goldberg*
Affiliation:
Division of Cardiology, The Perelman School of Medicine, The University of Pennsylvania, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, United States of America Department of Pediatrics, The Perelman School of Medicine, The University of Pennsylvania, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, United States of America
Catherine M. Avitabile
Affiliation:
Division of Cardiology, The Perelman School of Medicine, The University of Pennsylvania, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, United States of America
Michael G. McBride
Affiliation:
Division of Cardiology, The Perelman School of Medicine, The University of Pennsylvania, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, United States of America
Stephen M. Paridon
Affiliation:
Division of Cardiology, The Perelman School of Medicine, The University of Pennsylvania, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, United States of America Department of Pediatrics, The Perelman School of Medicine, The University of Pennsylvania, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, United States of America
*
Correspondence to: D. J. Goldberg, Division of Cardiology, The Children's Hospital of Philadelphia, 34th Street and Civic Center Blvd, Philadelphia, PA 19104, United States of America. Tel: 267-426-8143; Fax: 267-425-6108; E-mail: goldbergda@email.chop.edu
Get access Rights & Permissions [Opens in a new window]

Abstract

The Fontan operation can create a stable circulation from childhood through early adulthood. However, the absence of a sub-pulmonary pumping chamber leads to a physiology in which exercise capacity is limited and decreases with age starting in adolescence. The limitation in exercise capacity is more pronounced at peak levels of exercise, but is still present during more modest levels of activity. The underlying causes of exercise impairment relate to both central cardiovascular factors (oxygen delivery) and peripheral factors (oxygen extraction). Interventions to improve cardiac preload and to improve lean muscle mass may help to improve exercise capacity and, perhaps, will alter the “natural history” of the progressive decline.

Keywords

Congenital heart diseaseexerciseFontan

Information

Type
Original Article
Information
Cardiology in the Young , Volume 23 , Issue 6: HeartWeek 2013 , December 2013 , pp. 824 - 830
Copyright
Copyright © Cambridge University Press 2013 

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

Article purchase

Temporarily unavailable

References

1.Fontan, F, Baudet, E. Surgical repair of tricuspid atresia. Thorax 1971; 26: 240–248.CrossRefGoogle ScholarPubMed
2.Kreutzer, G, Galindez, E, Bono, H, De Palma, C, Laura, JP. An operation for the correction of tricuspid atresia. J Thorac Cardiovasc Surg 1973; 66: 613–621.CrossRefGoogle ScholarPubMed
3.Driscoll, DJ, Danielson, GK, Puga, FJ, Schaff, HV, Heise, CT, Staats, BA. Exercise tolerance and cardiorespiratory response to exercise after the Fontan operation for tricuspid atresia or functional single ventricle. J Am Coll Cardiol 1986; 7: 1087–1094.CrossRefGoogle ScholarPubMed
4.Harrison, DA, Liu, P, Walters, JE, et al. Cardiopulmonary function in adult patients late after Fontan repair. J Am Coll Cardiol 1995; 26: 1016–1021.CrossRefGoogle ScholarPubMed
5.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: 785–790.CrossRefGoogle ScholarPubMed
6.Driscoll, DJ, Durongpisitkul, K. Exercise testing after the Fontan operation. Pediat Cardiol 1999; 20: 57–59; discussion 60.CrossRefGoogle ScholarPubMed
7.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: 99–107.CrossRefGoogle ScholarPubMed
8.Fernandes, SM, McElhinney, DB, Khairy, P, Graham, DA, Landzberg, MJ, Rhodes, J. Serial cardiopulmonary exercise testing in patients with previous Fontan surgery. Pediat Cardiol 2010; 31: 175–180.CrossRefGoogle ScholarPubMed
9.Giardini, A, Hager, A, Pace Napoleone, C, Picchio, FM. Natural history of exercise capacity after the Fontan operation: a longitudinal study. Ann Thorac Surg 2008; 85: 818–821.CrossRefGoogle ScholarPubMed
10.Diller, GP, Giardini, A, Dimopoulos, K, et al. Predictors of morbidity and mortality in contemporary Fontan patients: results from a multicenter study including cardiopulmonary exercise testing in 321 patients. Eur Heart J 2010; 31: 3073–3083.CrossRefGoogle ScholarPubMed
11.Diller, GP, Dimopoulos, K, Okonko, D, et al. Exercise intolerance in adult congenital heart disease: comparative severity, correlates, and prognostic implication. Circulation 2005; 112: 828–835.CrossRefGoogle ScholarPubMed
12.Argiento, P, Chesler, N, Mule, M, et al. Exercise stress echocardiography for the study of the pulmonary circulation. Eur Respir J 2010; 35: 1273–1278.CrossRefGoogle Scholar
13.Stickland, MK, Welsh, RC, Petersen, SR, et al. Does fitness level modulate the cardiovascular hemodynamic response to exercise? J Appl Physiol 2006; 100: 1895–1901.CrossRefGoogle ScholarPubMed
14.Whitehead, KK, Pekkan, K, Kitajima, HD, Paridon, SM, Yoganathan, AP, Fogel, MA. Nonlinear power loss during exercise in single-ventricle patients after the Fontan: insights from computational fluid dynamics. Circulation 2007; 116: I165–I171.CrossRefGoogle ScholarPubMed
15.Haykowsky, MJ, Timmons, MP, Kruger, C, McNeely, M, Taylor, DA, Clark, AM. Meta-analysis of aerobic interval training on exercise capacity and systolic function in patients with heart failure and reduced ejection fractions. Am J Cardiol 2013; 111: 1466–1469.CrossRefGoogle ScholarPubMed
16.Haykowsky, MJ, Brubaker, PH, Stewart, KP, Morgan, TM, Eggebeen, J, Kitzman, DW. Effect of endurance training on the determinants of peak exercise oxygen consumption in elderly patients with stable compensated heart failure and preserved ejection fraction. J Am Coll Cardiol 2012; 60: 120–128.CrossRefGoogle ScholarPubMed
17.Avitabile, CM, Brodsky, JL, Leonard, MB, et al. Abnormalities in bone density, bone structure and muscle cross-sectional area after Fontan palliation (Abstract from ACC.13 – The 62nd Annual Scientific Sessions and Expo). J Am Coll Cardiol 2012; 61: E486.CrossRefGoogle Scholar
18.Haggerty, CM, de Zelicourt, DA, Restrepo, M, et al. Comparing pre- and post-operative Fontan hemodynamic simulations: implications for the reliability of surgical planning. Ann Biomed Eng 2012; 40: 2639–2651.CrossRefGoogle ScholarPubMed
19.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: 1185–1193.CrossRefGoogle Scholar
20.Giardini, A, Balducci, A, Specchia, S, Gargiulo, G, Bonvicini, M, Picchio, FM. Effect of sildenafil on haemodynamic response to exercise and exercise capacity in Fontan patients. Eur Heart J 2008; 29: 1681–1687.CrossRefGoogle ScholarPubMed
21.Morchi, GS, Ivy, DD, Duster, MC, Claussen, L, Chan, KC, Kay, J. Sildenafil increases systemic saturation and reduces pulmonary artery pressure in patients with failing Fontan physiology. Congenit Heart Dis 2009; 4: 107–111.CrossRefGoogle ScholarPubMed
22.Ovaert, C, Thijs, D, Dewolf, D, et al. The effect of bosentan in patients with a failing Fontan circulation. Cardiol Young 2009; 19: 331–339.CrossRefGoogle ScholarPubMed
23.Schuuring, MJ, Vis, JC, van Dijk, AP, et al. Impact of bosentan on exercise capacity in adults after the Fontan procedure: a randomized controlled trial. Eur J Heart Fail 2013; 15: 690–698.CrossRefGoogle ScholarPubMed
24.Hebert, A, Jensen, AS, Idorn, L, Sorensen, KE, Sondergaard, L. The effect of bosentan on exercise capacity in Fontan patients; rationale and design for the TEMPO study. BMC Cardiovas Dis 2013; 13: 36.CrossRefGoogle ScholarPubMed
25.Kouatli, AA, Garcia, JA, Zellers, TM, Weinstein, EM, Mahony, L. Enalapril does not enhance exercise capacity in patients after Fontan procedure. Circulation 1997; 96: 1507–1512.CrossRefGoogle Scholar
26.O'Byrne, ML, Mercer-Rosa, L, Ingall, E, McBride, MG, Paridon, S, Goldmuntz, E. Habitual exercise correlates with exercise performance in patients with conotruncal abnormalities. Pediat Cardiol 2013; 34: 853–860.CrossRefGoogle ScholarPubMed
27.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 Childh 2007; 92: 509–514.CrossRefGoogle ScholarPubMed
28.Longmuir, PE, Russell, JL, Corey, M, Faulkner, G, McCrindle, BW. Factors associated with the physical activity level of children who have the Fontan procedure. Am Heart J 2011; 161: 411–417.CrossRefGoogle ScholarPubMed
29.Cordina, RL, O'Meagher, S, Karmali, A, et al. Resistance training improves cardiac output, exercise capacity and tolerance to positive airway pressure in Fontan physiology. Int J Cardiol 2012 [Epub ahead of print].Google ScholarPubMed
30.Kitzman, DW, Brubaker, PH, Morgan, TM, Stewart, KP, Little, WC. Exercise training in older patients with heart failure and preserved ejection fraction: a randomized, controlled, single-blind trial. Circ Heart Fail 2010; 3: 659–667.CrossRefGoogle ScholarPubMed
31.Edelmann, F, Gelbrich, G, Dungen, HD, et al. Exercise training improves exercise capacity and diastolic function in patients with heart failure with preserved ejection fraction: results of the Ex-DHF (exercise training in diastolic heart failure) pilot study. J Am Coll Cardiol 2011; 58: 1780–1791.CrossRefGoogle ScholarPubMed