Skip to main content
PMC home page
Thorax logoLink to Thorax
. 2005 Dec;60(12):1019–1024. doi: 10.1136/thx.2004.037424

Randomised controlled trial of non-invasive ventilation (NIV) for nocturnal hypoventilation in neuromuscular and chest wall disease patients with daytime normocapnia

S Ward 1, M Chatwin 1, S Heather 1, A Simonds 1
PMCID: PMC1747266  PMID: 16299118

Abstract

Background: Long term non-invasive ventilation (NIV) reduces morbidity and mortality in patients with neuromuscular and chest wall disease with hypercapnic ventilatory failure, but preventive use has not produced benefit in normocapnic patients with Duchenne muscular dystrophy. Individuals with nocturnal hypercapnia but daytime normocapnia were randomised to a control group or nocturnal NIV to examine whether nocturnal hypoventilation is a valid indication for NIV.

Methods: Forty eight patients with congenital neuromuscular or chest wall disease aged 7–51 years and vital capacity <50% predicted underwent overnight respiratory monitoring. Twenty six with daytime normocapnia and nocturnal hypercapnia were randomised to either nocturnal NIV or to a control group without ventilatory support. NIV was started in the control group if patients fulfilled preset safety criteria.

Results: Peak nocturnal transcutaneous carbon dioxide tension (TcCO2) did not differ between the groups, but the mean (SD) percentage of the night during which TcCO2 was >6.5 kPa decreased in the NIV group (–57.7 (26.1)%) but not in controls (–11.75 (46.1)%; p = 0.049, 95% CI –91.5 to –0.35). Mean (SD) arterial oxygen saturation increased in the NIV group (+2.97 (2.57)%) but not in controls (–1.12 (2.02)%; p = 0.024, 95% CI 0.69 to 7.5). Nine of the 10 controls failed non-intervention by fulfilling criteria to initiate NIV after a mean (SD) of 8.3 (7.3) months.

Conclusion: Patients with neuromuscular disease with nocturnal hypoventilation are likely to deteriorate with the development of daytime hypercapnia and/or progressive symptoms within 2 years and may benefit from the introduction of nocturnal NIV before daytime hypercapnia ensues.

Full Text

The Full Text of this article is available as a PDF (91.2 KB).

Selected References

These references are in PubMed. This may not be the complete list of references from this article.

  1. Annane D., Chevrolet J. C., Chevret S., Raphael J. C. Nocturnal mechanical ventilation for chronic hypoventilation in patients with neuromuscular and chest wall disorders. Cochrane Database Syst Rev. 2000;(2):CD001941–CD001941. doi: 10.1002/14651858.CD001941. [DOI] [PubMed] [Google Scholar]
  2. Bach J. R., Ishikawa Y., Kim H. Prevention of pulmonary morbidity for patients with Duchenne muscular dystrophy. Chest. 1997 Oct;112(4):1024–1028. doi: 10.1378/chest.112.4.1024. [DOI] [PubMed] [Google Scholar]
  3. Bach J. R. Mechanical insufflation-exsufflation. Comparison of peak expiratory flows with manually assisted and unassisted coughing techniques. Chest. 1993 Nov;104(5):1553–1562. doi: 10.1378/chest.104.5.1553. [DOI] [PubMed] [Google Scholar]
  4. Bourke S. C., Shaw P. J., Gibson G. J. Respiratory function vs sleep-disordered breathing as predictors of QOL in ALS. Neurology. 2001 Dec 11;57(11):2040–2044. doi: 10.1212/wnl.57.11.2040. [DOI] [PubMed] [Google Scholar]
  5. Eagle Michelle, Baudouin Simon V., Chandler Colin, Giddings David R., Bullock Robert, Bushby Kate. Survival in Duchenne muscular dystrophy: improvements in life expectancy since 1967 and the impact of home nocturnal ventilation. Neuromuscul Disord. 2002 Dec;12(10):926–929. doi: 10.1016/s0960-8966(02)00140-2. [DOI] [PubMed] [Google Scholar]
  6. Hill N. S. Noninvasive positive pressure ventilation in neuromuscular disease. Enough is enough! Chest. 1994 Feb;105(2):337–338. doi: 10.1378/chest.105.2.337. [DOI] [PubMed] [Google Scholar]
  7. Janssens J. P., Perrin E., Bennani I., de Muralt B., Titelion V., Picaud C. Is continuous transcutaneous monitoring of PCO2 (TcPCO2) over 8 h reliable in adults? Respir Med. 2001 May;95(5):331–335. doi: 10.1053/rmed.2001.1045. [DOI] [PubMed] [Google Scholar]
  8. Jeppesen J., Green A., Steffensen B. F., Rahbek J. The Duchenne muscular dystrophy population in Denmark, 1977-2001: prevalence, incidence and survival in relation to the introduction of ventilator use. Neuromuscul Disord. 2003 Dec;13(10):804–812. doi: 10.1016/s0960-8966(03)00162-7. [DOI] [PubMed] [Google Scholar]
  9. Kleopa K. A., Sherman M., Neal B., Romano G. J., Heiman-Patterson T. Bipap improves survival and rate of pulmonary function decline in patients with ALS. J Neurol Sci. 1999 Mar 15;164(1):82–88. doi: 10.1016/s0022-510x(99)00045-3. [DOI] [PubMed] [Google Scholar]
  10. Leger P., Bedicam J. M., Cornette A., Reybet-Degat O., Langevin B., Polu J. M., Jeannin L., Robert D. Nasal intermittent positive pressure ventilation. Long-term follow-up in patients with severe chronic respiratory insufficiency. Chest. 1994 Jan;105(1):100–105. doi: 10.1378/chest.105.1.100. [DOI] [PubMed] [Google Scholar]
  11. Lyall R. A., Donaldson N., Fleming T., Wood C., Newsom-Davis I., Polkey M. I., Leigh P. N., Moxham J. A prospective study of quality of life in ALS patients treated with noninvasive ventilation. Neurology. 2001 Jul 10;57(1):153–156. doi: 10.1212/wnl.57.1.153. [DOI] [PubMed] [Google Scholar]
  12. Marcus C. L., Omlin K. J., Basinki D. J., Bailey S. L., Rachal A. B., Von Pechmann W. S., Keens T. G., Ward S. L. Normal polysomnographic values for children and adolescents. Am Rev Respir Dis. 1992 Nov;146(5 Pt 1):1235–1239. doi: 10.1164/ajrccm/146.5_Pt_1.1235. [DOI] [PubMed] [Google Scholar]
  13. Mehta S., Hill N. S. Noninvasive ventilation. Am J Respir Crit Care Med. 2001 Feb;163(2):540–577. doi: 10.1164/ajrccm.163.2.9906116. [DOI] [PubMed] [Google Scholar]
  14. Mellies U., Ragette R., Dohna Schwake C., Boehm H., Voit T., Teschler H. Long-term noninvasive ventilation in children and adolescents with neuromuscular disorders. Eur Respir J. 2003 Oct;22(4):631–636. doi: 10.1183/09031936.03.00044303a. [DOI] [PubMed] [Google Scholar]
  15. Milross M. A., Piper A. J., Norman M., Becker H. F., Willson G. N., Grunstein R. R., Sullivan C. E., Bye P. T. Low-flow oxygen and bilevel ventilatory support: effects on ventilation during sleep in cystic fibrosis. Am J Respir Crit Care Med. 2001 Jan;163(1):129–134. doi: 10.1164/ajrccm.163.1.2005130. [DOI] [PubMed] [Google Scholar]
  16. Pinto A. C., Evangelista T., Carvalho M., Alves M. A., Sales Luís M. L. Respiratory assistance with a non-invasive ventilator (Bipap) in MND/ALS patients: survival rates in a controlled trial. J Neurol Sci. 1995 May;129 (Suppl):19–26. doi: 10.1016/0022-510x(95)00052-4. [DOI] [PubMed] [Google Scholar]
  17. Ragette R., Mellies U., Schwake C., Voit T., Teschler H. Patterns and predictors of sleep disordered breathing in primary myopathies. Thorax. 2002 Aug;57(8):724–728. doi: 10.1136/thorax.57.8.724. [DOI] [PMC free article] [PubMed] [Google Scholar]
  18. Raphael J. C., Chevret S., Chastang C., Bouvet F. Randomised trial of preventive nasal ventilation in Duchenne muscular dystrophy. French Multicentre Cooperative Group on Home Mechanical Ventilation Assistance in Duchenne de Boulogne Muscular Dystrophy. Lancet. 1994 Jun 25;343(8913):1600–1604. doi: 10.1016/s0140-6736(94)93058-9. [DOI] [PubMed] [Google Scholar]
  19. Simonds A. K., Elliott M. W. Outcome of domiciliary nasal intermittent positive pressure ventilation in restrictive and obstructive disorders. Thorax. 1995 Jun;50(6):604–609. doi: 10.1136/thx.50.6.604. [DOI] [PMC free article] [PubMed] [Google Scholar]
  20. Simonds A. K., Muntoni F., Heather S., Fielding S. Impact of nasal ventilation on survival in hypercapnic Duchenne muscular dystrophy. Thorax. 1998 Nov;53(11):949–952. doi: 10.1136/thx.53.11.949. [DOI] [PMC free article] [PubMed] [Google Scholar]
  21. Simonds A. K., Ward S., Heather S., Bush A., Muntoni F. Outcome of paediatric domiciliary mask ventilation in neuromuscular and skeletal disease. Eur Respir J. 2000 Sep;16(3):476–481. doi: 10.1034/j.1399-3003.2000.016003476.x. [DOI] [PubMed] [Google Scholar]
  22. Vianello A., Bevilacqua M., Salvador V., Cardaioli C., Vincenti E. Long-term nasal intermittent positive pressure ventilation in advanced Duchenne's muscular dystrophy. Chest. 1994 Feb;105(2):445–448. doi: 10.1378/chest.105.2.445. [DOI] [PubMed] [Google Scholar]
  23. Wallgren-Pettersson Carina, Bushby Kate, Mellies Uwe, Simonds Anita, ENMC 117th ENMC workshop: ventilatory support in congenital neuromuscular disorders -- congenital myopathies, congenital muscular dystrophies, congenital myotonic dystrophy and SMA (II) 4-6 April 2003, Naarden, The Netherlands. Neuromuscul Disord. 2004 Jan;14(1):56–69. doi: 10.1016/j.nmd.2003.09.003. [DOI] [PubMed] [Google Scholar]

Articles from Thorax are provided here courtesy of BMJ Publishing Group

RESOURCES