Change in lung function of patients with cystic fibrosis underwent lung transplantation

The aim of this study was to evaluate changes in lung ventilation and lung diffusing capacity (DLCO) of cystic fibrosis (CF) patients during 1 year after lung transplantation. Methods. Complex lung function was monitored in CF patients underwent bilateral lung transplantation in 2012 – 2016. Results. CF patients (n = 12; mean age, 26.4 ± 4.4 years) were included in the study. Median follow-up after the lung transplantation was 19 (12–57) months. Consistent improvement in lung function parameters and DLCO was observed in 3 months after lung transplantation and maintained during 1 year. The ratio of forced expiratory volume in 1 sec to forced vital capacity was higher the normal level and the bronchial resistance was lower the cut-off values, i.e. the patients did not have bronchial obstruction. The total lung capacity (TLC) decreased to normal according to the anthropometric characteristics of the recipient. Meanwhile, change in the TLC structure, such as non-significant reduction in the vital capacity (VC) together with increase in the functional residual capacity (FRC), residual volume (RV) and RV/TLC ratio, have been developed. Consistent increase in FRC and RV could be explained by changes in the shape and the elasticity of the chest wall after the surgery. DLCO also improved, but was still slightly decreased. Conclusion. The lung function and DLCO improved in a year after bilateral lung transplantation in CF patients. Effects of different factors on postoperative lung function parameters need to be further investigated.

1. Krasovskiy S.A., Chernyak A.V., Kashirskaya N.Yu. et al. Cystic fibrosis in Russia: development of the National Register. Pediatriya. Zhurnal imeni G.N.Speranskogo. 2014; 93 (4): 44–55 (in Russian).

2. Kashirskaya N.Yu., Krasovskiy S.A., Chernyak A.V. et al. Change in the life expectancy of cystic fibrosis patients in Moscow and a relationship with therapy: a retrospective analysis, 1993 – 2013. Voprosy sovremennoy pediatrii. 2015; 14 (4): 503–508. DOI: 10.15690/vsp.v14.i4.1390 (in Russian).

3. Kondrat'eva E.I. Innovative therapeutic methods for cystic fibrosis. Vrach. 2016; 2: 77–81 (in Russian).

4. Stephenson A.L., Math J.S., Berthiaume Y. et al. Clinical and demographic factors associated with post–lung transplantation survival in individuals with cystic fibrosis. J. Heart Lung Transplant. 2015; 34 (9): 1139–1145. DOI: 10.1016/j.healun.2015.05.003.

5. Miller M.R., Hankinson J., Brusasco V. et al. Standardisation of spirometry. Eur. Respir. J. 2005; 26 (2): 319–337. DOI: 10.1183/09031936.05.00034805.

6. Chuchalin A.G., Aysanov Z.R., Chikina S.Yu. et al. Federal Clinical Guidelines of Russian Respiratory Society on Spirometry. Pul'monologiya. 2014; (6): 11–23 (in Russian).

7. Wanger J., Clausen J.L., Coates A. et al. Standardisation of the measurement of lung volumes. Eur. Respir. J. 2005; 26 (3): 511–522. DOI: 10.1183/09031936.05.00035005.

8. Macintyre N., Crapo R.O., Viegi G. et al. Standardisation of the single-breath determination of carbon monoxide uptake in the lung. Eur. Respir. J. 2005; 26 (4): 720–735. DOI: 10.1183/09031936.05.00034905.

9. Cooper J.D., Billingham M., Egan T. et al. A working formulation for the standardization of nomenclature and for clinical staging of chronic dysfunction in lung allografts. J. Heart Lung Transplant. 1993; 12 (5): 713–716.

10. Estenne M., Maurer J.R., Boehler A. et al. Bronchiolitis obliterans syndrome 2001: an update of the diagnostic criteria. J. Heart Lung Transplant. 2002; 21 (3): 297–310.

11. Pellegrino R., Viegi G., Brusasco V. et al. Interpretative strategies for lung function tests. Eur. Respir. J. 2005; 26 (5): 948–968. DOI: 10.1183/09031936.05.00035205.

12. Chacon R.A., Corris P.A., Dark J.H., Gibson G.J. Respiratory mechanics after heart-lung and bilateral lung transplantation. Thorax. 1997; 52 (8): 718–722.

13. Arens R., McDonough J.M., Zhao H. et al. Altered lung mechanics after double-lung transplantation. Am. J. Respir. Crit. Care Med. 1998; 158 (5): 1403–1409. DOI: 10.1164/ajrccm.158.5.9711086.

14. Estenne M., Ketelbant P., Primo G., Yernault J.C. Human heart-lung transplantation: physiologic aspects of the denervated lung and post-transplant obliterative bronchiolitis. Am. Rev. Respir. Dis. 1987; 135 (4): 976–978. DOI: 10.1164/arrd.1987.135.4.976.

15. Griffith B.P., Hardesty R.L., Trento A. et al. Heart-lung transplantation: lessons learned and future hopes. Ann. Thorac. Surg. 1987; 43: 6–16.

16. Burke C.M., Theodore J., Baldwin J.C. et al. Twenty eight cases of human heart-lung transplantation. Lancet. 1986; 1 (8480): 517–519.

17. Tamm M., Higenbottam T.W., Dennis C.M. et al. Donor and recipient predicted lung volume and lung size after heart-lung transplantation. Am. J. Respir. Crit. Care Med. 1994; 150 (2): 403–407. DOI: 10.1164/ajrccm.150.2.8049822.

18. Guignon I., Cassart M., Gevenois PA. et al. Persistent hyperinflation after heart-lung transplantation for cystic fibrosis. Am. J. Respir. Crit. Care Med. 1995; 151 (2, Pt 1): 534–540. DOI: 10.1164/ajrccm.151.2.7842217.

19. Pinet C., Estenne M. Effect of preoperative hyperinflation on static lung volumes after lung transplantation. Eur. Respir. J. 2000; 16 (3): 482–485.

20. van Muylem A., Paiva M., Estenne M. Involvement of peripheral airways during methacholineinduced bronchoconstriction after lung transplantation. Am. J. Respir. Crit. Care Med. 2001; 164 (7): 1200–1203. DOI: 10.1164/ajrccm.164.7.2102113.

21. Williams T.J., Patterson G.A., McClean P.A. et al. Maximal exercise testing in single and double lung transplant recipients. Am. Rev. Respir. Dis. 1992; 145 (1): 101–105. DOI: 10.1164/ajrccm/145.1.101.