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Impaired lung function in children and adolescents with Fontan circulation may improve after endurance training

Published online by Cambridge University Press:  04 July 2018

Eva R. Hedlund ,
Henrik Ljungberg ,
Liselott Söderström ,
Bo Lundell  and
Gunnar Sjöberg
Eva R. Hedlund*
Affiliation:
Department of Women’s and Children’s Health, Karolinska Institutet, Stockholm, Sweden
Henrik Ljungberg
Affiliation:
Department of Women’s and Children’s Health, Karolinska Institutet, Stockholm, Sweden
Liselott Söderström
Affiliation:
Department of Women’s and Children’s Health, Karolinska Institutet, Stockholm, Sweden
Bo Lundell
Affiliation:
Department of Women’s and Children’s Health, Karolinska Institutet, Stockholm, Sweden
Gunnar Sjöberg
Affiliation:
Department of Women’s and Children’s Health, Karolinska Institutet, Stockholm, Sweden
*
Author for correspondence: E. R. Hedlund, MD, Karolinska University Hospital, Astrid Lindgren Children’s Hospital Solna, Barnhjärtcentrum, Eugeniavägen 23, C8:34, S-171 76 Stockholm, Sweden. Tel: +46 707421285; Fax: +46 8 51777778; E-mail: eva.rylander-hedlund@sll.se
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Abstract

Objectives

The objective of this research was to study lung function, physical capacity, and effect of endurance training in children and adolescents after Fontan palliation compared with healthy matched controls.

Methods

Fontan patients (n=30) and healthy matched control patients (n=25) performed dynamic and static spirometry, and pulmonary diffusing capacity and maximal oxygen uptake tests, before and after a 12-week endurance training programme and at follow-up after 1 year.

Results

Fontan patients had a restrictive lung pattern, reduced pulmonary diffusing capacity (4.27±1.16 versus 6.61±1.88 mmol/kPa/minute, p<0.001), and a reduced maximal oxygen uptake (35.0±5.1 versus 43.7±8.4 ml/minute/kg, p<0.001) compared with controls. Patients had air trapping with a higher portion of residual volume of total lung capacity compared with controls (26±6 versus 22±5%, p<0.05). Vital capacity increased for patients, from 2.80±0.97 to 2.91±0.95 L, p<0.05, but not for controls after endurance training. The difference in diffusing capacity between patients and controls appeared to be greater with increasing age.

Conclusions

Fontan patients have a restrictive lung pattern, reduced pulmonary diffusing capacity, and reduced maximal oxygen uptake compared with healthy controls. Endurance training may improve vital capacity in Fontan patients. The normal increase in pulmonary diffusing capacity with age and growth was reduced in Fontan patients, which is concerning. Apart from general health effects, exercise may improve lung function in young Fontan patients and should be encouraged.

Keywords

Endurance trainingexercise capacityFontan palliationlung functionpulmonary diffusing capacity
Type
Original Article
Information
Cardiology in the Young , Volume 28 , Issue 9 , September 2018 , pp. 1115 - 1122
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Copyright
© Cambridge University Press 2018 

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Footnotes

Cite this article: Hedlund ER, Ljungberg H, Söderström L, Lundell B, Sjöberg G. (2018) Impaired lung function in children and adolescents with Fontan circulation may improve after endurance training. Cardiology in the Young28: 1115–1122. doi: S1047951118000902

References

1. 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.CrossRefGoogle ScholarPubMed
2. 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.Google Scholar
3. Fontan, FBE. Surgical repair of tricuspid atresia. Thorax 1971; 26: 240–248.Google Scholar
4. Kanakis, MA, Petropoulos, AC, Mitropoulos, FA. Fontan operation. Hellenic J Cardiol 2009; 50: 133–141.Google Scholar
5. Goldberg, DJ, Avitabile, CM, McBride, MG, Paridon, SM. Exercise capacity in the Fontan circulation. Cardiol Young 2013; 23: 824–830.CrossRefGoogle ScholarPubMed
6. 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.Google Scholar
7. 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.Google Scholar
8. 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: 509–514.CrossRefGoogle ScholarPubMed
9. Muller, J, Christov, F, Schreiber, C, Hess, J, Hager, A. Exercise capacity, quality of life, and daily activity in the long-term follow-up of patients with univentricular heart and total cavopulmonary connection. Eur Heart J 2009; 30: 2915–2920.Google Scholar
10. Dulfer, K, Bossers, SS, Utens, EM, et al. Does functional health status predict health-related quality of life in children after Fontan operation? Cardiol Young 2016; 26: 459–468.Google Scholar
11. 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.Google Scholar
12. Knowles, RL, Day, T, Wade, A, et al. Patient-reported quality of life outcomes for children with serious congenital heart defects. Arch Dis Child 2014; 99: 413–419.Google Scholar
13. McCrindle, BW, Williams, RV, Mitchell, PD, et al. Relationship of patient and medical characteristics to health status in children and adolescents after the Fontan procedure. Circulation 2006; 113: 1123–1129.Google Scholar
14. Uzark, K, Jones, K, Slusher, J, Limbers, CA, Burwinkle, TM, Varni, JW. Quality of life in children with heart disease as perceived by children and parents. Pediatrics 2008; 121: e1060–e1067.Google Scholar
15. Rhodes, J, Curran, TJ, Camil, L, et al. Impact of cardiac rehabilitation on the exercise function of children with serious congenital heart disease. Pediatrics 2005; 116: 1339–1345.Google Scholar
16. Sutherland, N, Jones, B, d’Udekem, Y. Should we recommend exercise after the fontan procedure? Heart Lung Circ 2015; 24: 753–768.CrossRefGoogle ScholarPubMed
17. Takken, T, Hulzebos, HJ, Blank, AC, Tacken, MH, Helders, PJ, Strengers, JL. Exercise prescription for patients with a Fontan circulation: current evidence and future directions. Neth Heart J 2007; 15: 142–147.Google Scholar
18. Rhodes, J, Curran, TJ, Camil, L, et al. Sustained effects of cardiac rehabilitation in children with serious congenital heart disease. Pediatrics 2006; 118: e586–e593.Google Scholar
19. Moalla, W, Elloumi, M, Chamari, K, et al. Training effects on peripheral muscle oxygenation and performance in children with congenital heart diseases. Appl Physiol Nutr Metab 2012; 37: 621–630.Google Scholar
20. 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–H117.Google Scholar
21. Idorn, L, Hanel, B, Jensen, AS, et al. New insights into the aspects of pulmonary diffusing capacity in Fontan patients. Cardiol Young 2014; 24: 311–320.CrossRefGoogle ScholarPubMed
22. Larsson, ES, Eriksson, BO, Sixt, R. Decreased lung function and exercise capacity in Fontan patients. A long-term follow-up. Scand Cardiovasc J 2003; 37: 58–63.CrossRefGoogle ScholarPubMed
23. Matthews, IL, Fredriksen, PM, Bjornstad, PG, Thaulow, E, Gronn, M. Reduced pulmonary function in children with the Fontan circulation affects their exercise capacity. Cardiol Young 2006; 16: 261–267.CrossRefGoogle ScholarPubMed
24. Ohuchi, H, Ohashi, H, Takasugi, H, Yamada, O, Yagihara, T, Echigo, S. Restrictive ventilatory impairment and arterial oxygenation characterize rest and exercise ventilation in patients after fontan operation. Pediatr Cardiol 2004; 25: 513–521.Google Scholar
25. Hedenstrom, H, Malmberg, P, Agarwal, K. Reference values for lung function tests in females. Regression equations with smoking variables. Bull Eur Physiopathol Respir 1985; 21: 551–557.Google Scholar
26. Hedenstrom, H, Malmberg, P, Fridriksson, HV. Reference values for lung function tests in men: regression equations with smoking variables. Ups J Med Sci 1986; 91: 299–310.Google Scholar
27. Solymar, L, Aronsson, PH, Bake, B, Bjure, J. Nitrogen single breath test, flow-volume curves and spirometry in healthy children, 7-18 years of age. Eur J Respir Dis 1980; 61: 275–286.Google Scholar
28. Eakin, BL, Finta, KM, Serwer, GA, Beekman, RH. Perceived exertion and exercise intensity in children with or without structural heart defects. J Pediatr 1992; 120: 90–93.Google Scholar
29. Gewillig, M, Brown, SC. The Fontan circulation after 45 years: update in physiology. Heart 2016; 102: 1081–1086.CrossRefGoogle ScholarPubMed
30. Turquetto, ALR, Caneo, LF, Agostinho, DR, et al. Impaired pulmonary function is an additional potential mechanism for the reduction of functional capacity in clinically stable fontan patients. Pediatr Cardiol 2017; 38: 981–990.CrossRefGoogle ScholarPubMed
31. Yin, Z, Wang, H, Wang, Z, et al. Radionuclide and angiographic assessment of pulmonary perfusion after Fontan procedure: comparative interim outcomes. Ann Thorac Surg 2012; 93: 620–625.CrossRefGoogle ScholarPubMed
32. Mettauer, B, Lampert, E, Charloux, A, et al. Lung membrane diffusing capacity, heart failure, and heart transplantation. Am J Cardiol 1999; 83: 62–67.Google Scholar
33. 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: e005750.Google Scholar
34. Zavorsky, GS, Smoliga, JM. The association between cardiorespiratory fitness and pulmonary diffusing capacity. Respir Physiol Neurobiol 2017; 241: 28–35.Google Scholar