Hostname: page-component-76fb5796d-22dnz Total loading time: 0 Render date: 2024-04-26T21:06:42.425Z Has data issue: false hasContentIssue false
  • English
  • Français

New insights into the aspects of pulmonary diffusing capacity in Fontan patients

Published online by Cambridge University Press:  03 April 2013

Lars Idorn ,
Birgitte Hanel ,
Annette S. Jensen ,
Klaus Juul ,
Jesper I. Reimers ,
Kim G. Nielsen  and
Lars Søndergaard
Lars Idorn*
Affiliation:
Department of Cardiology, Rigshospitalet, Copenhagen, Denmark
Birgitte Hanel
Affiliation:
Department of Paediatrics and Adolescent Medicine, Section of Paediatric Pulmonary Service, Rigshospitalet, Copenhagen, Denmark
Annette S. Jensen
Affiliation:
Department of Cardiology, Rigshospitalet, Copenhagen, Denmark
Klaus Juul
Affiliation:
Department of Paediatrics and Adolescent Medicine, Section of Pediatric Cardiology, Rigshospitalet, Copenhagen, Denmark
Jesper I. Reimers
Affiliation:
Department of Paediatrics and Adolescent Medicine, Section of Pediatric Cardiology, Rigshospitalet, Copenhagen, Denmark
Kim G. Nielsen
Affiliation:
Department of Paediatrics and Adolescent Medicine, Section of Paediatric Pulmonary Service, Rigshospitalet, Copenhagen, Denmark
Lars Søndergaard
Affiliation:
Department of Cardiology, Rigshospitalet, Copenhagen, Denmark
*
Correspondence to: Dr L. Idorn, MD, Department of Cardiology, Blegdamsvej 9, section 2014, DK 2100, Rigshospitalet, Copenhagen East, Denmark. Tel: +0045 35450807; Fax: +0045 35452230; E-mail: lars.idorn@rh.regionh.dk
Get access Rights & Permissions [Opens in a new window]

Abstract

Background: Patients with a functionally univentricular heart, palliated a.m. Fontan, consequently have non-pulsatile pulmonary blood flow and are known to have a reduced pulmonary diffusing capacity. However, the cause of this reduction remains unclear. We aimed to assess the possible determinants in the aetiology of a reduced diffusing capacity and also to assess whether it could be increased. Furthermore, we aimed to search for predictors of a reduced diffusing capacity. Material and methods: A total of 87 Fontan patients (mean age 16.3 ± 7.6 years) performed advanced pulmonary function tests and maximal cycle ergometer tests. A total of 10 Fontan patients and nine matched controls performed a supine pulmonary function test after a supine rest. Results: In the sitting pulmonary function test, the mean z-scores were: diffusing capacity, 2.38 ± 1.20; pulmonary capillary blood volume, 2.04 ± 0.80; and alveolar capillary membrane diffusing capacity, 0.14 ± 0.84. In the supine compared with the sitting pulmonary function test, the diffusing capacity increased by 51.7 ± 11.9% in the Fontan group and by 23.3 ± 17.7% in the control group (p < 0.001); moreover, the pulmonary capillary blood volume increased by 48.3 ± 17.4% in the Fontan group and by 20.2 ± 13.9% in the control group (p = 0.001). In a multiple linear regression analysis including the explanatory variables of surgical data and exercise data at rest and peak exercise, the resting cardiac index was an independent predictor of the diffusing capacity (regression coefficient: 0.18, p < 0.001). Conclusions: The pulmonary diffusing capacity was reduced in Fontan patients because of a reduced pulmonary capillary blood volume, whereas the alveolar capillary membrane diffusing capacity was preserved. The diffusing capacity was highly increasable in Fontan patients compared with controls, and the resting cardiac index was an independent predictor of the diffusing capacity.

Keywords

Fontan procedurecongenital heart diseasepulmonary diffusing capacityexercise test
Type
Original Articles
Information
Cardiology in the Young , Volume 24 , Issue 2 , April 2014 , pp. 311 - 320
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.)

References

1. 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
2. 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
3. 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
4. Presson, RG Jr, Baumgartner, WA Jr, Peterson, AJ, Glenny, RW, Wagner, WW Jr. Pulmonary capillaries are recruited during pulsatile flow. J Appl Physiol 2002; 92: 1183–1190.CrossRefGoogle ScholarPubMed
5. Hakim, TS. Flow-induced release of EDRF in the pulmonary vasculature: site of release and action. Am J Physiol 1994; 267 (Pt 2): H363–H369.Google Scholar
6. Yin, Z, Wang, Z, Zhu, H, Zhang, R, Wang, H, Li, X. Experimental study of effect of Fontan circuit on pulmonary microcirculation. Asian Cardiovasc Thorac Ann 2006; 14: 183–188.CrossRefGoogle ScholarPubMed
7. Zongtao, Y, Huishan, W, Zengwei, W, et al. Experimental study of nonpulsatile flow perfusion and structural remodeling of pulmonary microcirculation vessels. Thorac Cardiovasc Surg 2010; 58: 468–472.Google Scholar
8. Miller, MR, Hankinson, J, Brusasco, V, et al. Standardisation of spirometry. Eur Respir J 2005; 26: 319–338.CrossRefGoogle ScholarPubMed
9. MacIntyre, N, Crapo, RO, Viegi, G, et al. Standardisation of the single-breath determination of carbon monoxide uptake in the lung. Eur Respir J 2005; 26: 720–735.CrossRefGoogle ScholarPubMed
10. Guenard, H, Varene, N, Vaida, P. Determination of lung capillary blood volume and membrane diffusing capacity in man by the measurements of NO and CO transfer. Respir Physiol 1987; 70: 113–120.Google Scholar
11. Stanojevic, S, Wade, A, Stocks, J, et al. Reference ranges for spirometry across all ages: a new approach. Am J Respir Crit Care Med 2008; 177: 253–260.Google Scholar
12. Zapletal, A, Samanek, M, Paul, T. Lung Function in Children and Adolescents. Methods, Reference values, 22nd edn. Karger, Basel, 1987.Google Scholar
13. Stam, H, van den Beek, A, Grunberg, K, Stijnen, T, Tiddens, HA, Versprille, A. Pulmonary diffusing capacity at reduced alveolar volumes in children. Pediatr Pulmonol 1996; 21: 84–89.Google Scholar
14. Cotes, JE, Chinn, DJ, Quanjer, PH, Roca, J, Yernault, JC. Standardization of the measurement of transfer factor (diffusing capacity). 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: 41–52.Google Scholar
15. Crapo, RO, Morris, AH, Gardner, RM. Reference values for pulmonary tissue volume, membrane diffusing capacity, and pulmonary capillary blood volume. Bull Eur Physiopathol Respir 1982; 18: 893–899.Google Scholar
16. Lang, CC, Karlin, P, Haythe, J, Tsao, L, Mancini, DM. Ease of noninvasive measurement of cardiac output coupled with peak VO2 determination at rest and during exercise in patients with heart failure. Am J Cardiol 2007; 99: 404–405.Google Scholar
17. Cooper, DM, Weiler-Ravell, D. Gas exchange response to exercise in children. Am Rev Respir Dis 1984; 129 (Pt 2): S47–S48.Google Scholar
18. Cooper, CB, Storer, TW. Exercise Testing and Interpretation: A Practical Approach. Cambridge University Press, Cambridge, United Kingdom, 2001, p 278.Google Scholar
19. Morgan, VL, Graham, TP Jr, Roselli, RJ, Lorenz, CH. Alterations in pulmonary artery flow patterns and shear stress determined with three-dimensional phase-contrast magnetic resonance imaging in Fontan patients. J Thorac Cardiovasc Surg 1998; 116: 294–304.Google Scholar
20. Wang, H, Yin, Z, Wang, Z, et al. The mid-term follow-up of pulmonary perfusion in patients after extracardiac total cavopulmonary connection. Nucl Med Commun 2012; 33: 148–154.CrossRefGoogle ScholarPubMed
21. Matsushita, T, Sano, T, Okada, S. Postural change and pulmonary ventilation–perfusion distribution after Fontan operation. Pediatr Int 2000; 42: 226–227.Google Scholar
22. Barber, G, Di, ST, Child, JS, et al. Hemodynamic responses to isolated increments in heart rate by atrial pacing after a Fontan procedure. Am Heart J 1988; 115: 837–841.Google Scholar
23. Gewillig, M, Brown, SC, Eyskens, B, et al. The Fontan circulation: who controls cardiac output? Interact Cardiovasc Thorac Surg 2010; 10: 428–433.Google Scholar
24. Dasi, LP, Krishnankuttyrema, R, Kitajima, HD, et al. Fontan hemodynamics: importance of pulmonary artery diameter. J Thorac Cardiovasc Surg 2009; 137: 560–564.CrossRefGoogle ScholarPubMed
25. Khambadkone, S, Li, J, de Leval, MR, Cullen, S, Deanfield, JE, Redington, AN. Basal pulmonary vascular resistance and nitric oxide responsiveness late after Fontan-type operation. Circulation 2003; 107: 3204–3208.Google Scholar
26. Beghetti, M. Fontan and the pulmonary circulation: a potential role for new pulmonary hypertension therapies. Heart 2010; 96: 911–916.Google Scholar
27. 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.Google Scholar
28. 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.Google Scholar
29. 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.CrossRefGoogle ScholarPubMed
30. Tamhane, RM, Johnson, RL Jr, Hsia, CC. Pulmonary membrane diffusing capacity and capillary blood volume measured during exercise from nitric oxide uptake. Chest 2001; 120: 1850–1856.CrossRefGoogle ScholarPubMed
31. Black, MD, van Son, JA, Haas, GS. Extracardiac Fontan operation with adjustable communication. Ann Thorac Surg 1995; 60: 716–718.CrossRefGoogle ScholarPubMed
32. Rodefeld, MD, Frankel, SH, Giridharan, GA. Cavopulmonary assist: (em)powering the univentricular Fontan circulation. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 2011; 14: 45–54.Google Scholar