Hostname: page-component-8448b6f56d-c4f8m Total loading time: 0 Render date: 2024-04-19T17:53:25.955Z Has data issue: false hasContentIssue false
  • English
  • Français

Reduced pulmonary function in children with the Fontan circulation affects their exercise capacity

Published online by Cambridge University Press:  26 May 2006

Iren Lindbak Matthews ,
Per Morten Fredriksen ,
Per G. Bjørnstad ,
Erik Thaulow  and
Morten Gronn
Iren Lindbak Matthews
Affiliation:
Paediatric Pulmonology and Allergology Unit, Paediatric Department, Rikshospitalet University Hospital, Oslo, Norway
Per Morten Fredriksen
Affiliation:
Physiotherapy Department, Rikshospitalet University Hospital, Oslo, Norway
Per G. Bjørnstad
Affiliation:
Paediatric Cardiology Unit, Paediatric Department, Rikshospitalet University Hospital, Oslo, Norway
Erik Thaulow
Affiliation:
Paediatric Cardiology Unit, Paediatric Department, Rikshospitalet University Hospital, Oslo, Norway
Morten Gronn
Affiliation:
Neonatology Unit, Paediatric Department, Rikshospitalet University Hospital, Oslo, Norway
Get access Rights & Permissions [Opens in a new window]

Abstract

Most children with functionally univentricular hearts nowadays are treated surgically by creating a total cavopulmonary connection. In the resulting Fontan circulation, the venous return and the pulmonary arterial bed are coupled in series, bypassing the heart. This gives the potential for interaction between the abnormal circulation and function of the lungs. In this study, we investigated the pattern of impairment of pulmonary function, and its relation to decreased exercise capacity.

We performed spirometry in 33 (85 percent) of 39 eligible Norwegian children, aged from 8 to 16, with a total cavopulmonary connection, along with whole body plethysmography, the carbon monoxide single breath test, and a peak treadmill exercise test. The single breath test showed a mean corrected diffusing capacity of 66.5 percent of predicted, giving a z score of minus 2.88. The mean residual volume measured by whole body plethysmography was 146.8 percent, equivalent to a z score of 2.46, whereas the mean residual volume measured by the single breath test was 102.4 percent of predicted, this being the same as a z score of 0.43. The mean peak treadmill exercise test was 70.0 percent of predicted, equivalent with a z score of minus 3.07. Mean forced vital capacity was 85.7 percent of predicted, the equivalent z score being minus 0.92. Lung function correlated with the peak treadmill exercise test.

We have shown, therefore, that children with the Fontan circulation have reduced diffusing capacity, possibly caused by the abnormal circulation through the lungs. The difference between residual volume measured by plethysmography and the single breath test implies trapping of air. The correlation of parameters for lung function with peak consumption of oxygen during exercise indicates that the abnormalities of pulmonary function may affect physical capacity.

Keywords

Functionally univentricular heartCongenital heart diseasediffusing capacitylung function
Type
Original Article
Information
Cardiology in the Young , Volume 16 , Issue 3 , June 2006 , pp. 261 - 267
Copyright
© 2006 Cambridge University Press

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.)

References

Fontan F, Mounicot FB, Baudet E, Simonneau J, Gordo J, Gouffrant JM. [“Correction” of tricuspid atresia. 2 cases “corrected” using a new surgical technic]. Ann Chir Thorac Cardiovasc 1971; 10: 39–47.Google Scholar
Joshi VM, Carey A, Simpson P, Paridon SM. Exercise performance following repair of hypoplastic left heart syndrome: A comparison with other types of Fontan patients. Pediatr Cardiol 1997; 18: 357–360.Google Scholar
Nir A, Driscoll DJ, Mottram CD, et al. Cardiorespiratory response to exercise after the Fontan operation: a serial study. J Am Coll Cardiol 1993; 22: 216–220.Google Scholar
Weipert J, Koch W, Haehnel JC, Meisner H. Exercise capacity and mid-term survival in patients with tricuspid atresia and complex congenital cardiac malformations after modified Fontan-operation. Eur J Cardiothorac Surg 1997; 12: 574–580.Google Scholar
Zajac A, Tomkiewicz L, Podolec P, Tracz W, Malec E. Cardiorespiratory response to exercise in children after modified fontan operation. Scand Cardiovasc J 2002; 36: 80–85.Google Scholar
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.Google Scholar
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
Quanjer PH, Tammeling GJ, Cotes JE, Pedersen OF, Peslin R, Yernault JC. Lung volumes and forced ventilatory flows. Report Working Party Standardization of Lung Function Tests, European Community for Steel and Coal. Official Statement of the European Respiratory Society. Eur Respir J Suppl 1993; 16: 5–40.Google Scholar
Zapletal A, Samanek M, Paul T. Lung function in Children and Adolescents. Methods, Reference values. 22 ed. Karger, Basel; 1987.
Stam H, van den BA, Grunberg K, Stijnen T, Tiddens HA, Versprille A. Pulmonary diffusing capacity at reduced alveolar volumes in children. Pediatric Pulmonology 1996; 21: 84–89.Google Scholar
Fredriksen PM, Ingjer F, Nystad W, Thaulow E. Aerobic endurance testing of children and adolescents–a comparison of two treadmill-protocols. Scand J Med Sci Sports 1998; 8: 203–207.Google Scholar
Pettersen SA, Fredriksen PM, Ingjer E. The correlation between peak oxygen uptake (VO2 peak) and running performance in children and adolescents. Aspects of different units. Scand J Med Sci Sports 2001; 11: 223–228.Google Scholar
He Q, Albertsson-Wikland K, Karlberg J. Population-based body mass index reference values from Goteborg, sweden: birth to 18 years of age. Acta Paediatr 2000; 89: 582–592.Google Scholar
Knudtzon J, Waaler PE, Skjaerven R, Solberg LK, Steen J. [New Norwegian percentage charts for height, weight and head circumference for age groups 0–17 years]. Tidsskr Nor Laegeforen 1988; 108: 2125–2135.Google Scholar
Waaler PE. Anthropometric studies in Norwegian children. Acta Paediatr Scand Suppl 1983; 308: 1–41.Google Scholar
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
Bridges ND, Lock JE, Mayer Jr JE, Burnett J, Castaneda AR. Cardiac catheterization and test occlusion of the interatrial communication after the fenestrated Fontan operation. J Am Coll Cardiol 1995; 25: 1712–1717.Google Scholar
Levy M, Danel C, Tamisier D, Vouhe P, Leca F. Histomorphometric analysis of pulmonary vessels in single ventricle for better selection of patients for the Fontan operation. J Thorac Cardiovasc Surg 2002; 123: 263–270.Google Scholar
Rosenthal DN, Friedman AH, Kleinman CS, Kopf GS, Rosenfeld LE, Hellenbrand WE. Thromboembolic complications after Fontan operations. Circulation 1995; 92: II287–II293.Google Scholar
Svanes C, Omenaas E, Jarvis D, Chinn S, Gulsvik A, Burney P. Parental smoking in childhood and adult obstructive lung disease: results from the European Community Respiratory Health Survey. Thorax 2004; 59: 295–302.Google Scholar
Rizzi M, Sergi M, Andreoli A, Pecis M, Bruschi C, Fanfulla F. Environmental tobacco smoke may induce early lung damage in healthy male adolescents. Chest 2004; 125: 1387–1393.Google Scholar
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.Google Scholar
Driscoll DJ, Durongpisitkul K. Exercise testing after the Fontan operation. Pediatr Cardiol 1999; 20: 57–59.Google Scholar
Fredriksen PM, Therrien J, Veldtman G, Warsi MA, Liu P, Siu S et al. Lung function and aerobic capacity in adult patients following modified Fontan procedure. Heart 2001; 85: 295–299.Google Scholar
Fredriksen PM, Kahrs N, Blaasvaer S, et al. Effect of physical training in children and adolescents with congenital heart disease. Cardiol Young 2000; 10: 107–114.Google Scholar
Redington AN, Penny D, Shinebourne EA. Pulmonary blood flow after total cavopulmonary shunt. Br Heart J 1991; 65: 213–217.Google Scholar
Dimopoulou I, Tsintzas OK, Daganou M, Cokkinos DV, Tzelepis GE. Contribution of lung function to exercise capacity in patients with chronic heart failure. Respiration 1999; 66: 144–149.Google Scholar