Abstract
Purpose
Restrictive respiratory failure is a major cause of morbidity and mortality in neuromuscular diseases (NMD). Home mechanical ventilation (HMV) is used to treat hypoventilation, identified by daytime hypercapnia or nocturnal desaturation. Recently, transcutaneous measure of CO2 (TcCO2) has been increasingly used to detect hypoventilation, using different cut-offs. We aimed to compare the prevalence of hypoventilation in an unselected adult NMD population according to different definitions issued from the literature.
Methods
All consecutive nocturnal capno-oximetries performed between 2010 and 2014 in unventilated adult NMD patients were analysed retrospectively. Concomitant blood gas analysis and lung function data were collected. Patients on oxygen therapy were excluded. Hypoventilation was defined according to eight criteria, based on daytime PaCO2, daytime base excess, nocturnal SpO2 or TcCO2.
Results
Data from 232 patients were analysed (mean age 43.1 ± 15.4 years; 50.0 % women; vital capacity 59.2 ± 24.2 % of predicted). The hypoventilation prevalence was 10.3 to 61.2 %, depending on the used definition. The different definitions showed 49.1 to 94.8 % concordance (Cohen’s kappa for agreement 0.115 to 0.763). Overall agreement between the eight definitions was poor (Light’s kappa 0.267), and agreement between definitions based on nocturnal SpO2 and those based on TcCO2 was even lower (Light’s kappa 0.204).
Conclusions
We found large differences in hypoventilation prevalence according to the used definition. This has practical consequences, as HMV indication relies upon hypoventilation detection. We believe that capno-oximetry should be included in the diagnostic tools used to detect hypoventilation but this requires an update of consensus guidelines to agree upon the best definition.
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References
Simonds AK (2013) Chronic hypoventilation and its management. Eur Respir Rev 22(129):325–332. doi:10.1183/09059180.00003113
Polkey MI, Lyall RA, Moxham J, Leigh PN (1999) Respiratory aspects of neurological disease. J Neurol Neurosurg Psychiatry 66(1):5–15
Norwood F, de Visser M, Eymard B, Lochmuller H, Bushby K, Force EGT (2007) EFNS guideline on diagnosis and management of limb girdle muscular dystrophies. Eur J Neurol 14(12):1305–1312. doi:10.1111/j.1468-1331.2007.01979.x
Rimmer KP, Golar SD, Lee MA, Whitelaw WA (1993) Myotonia of the respiratory muscles in myotonic dystrophy. Am Rev Respir Dis 148(4 Pt 1):1018–1022. doi:10.1164/ajrccm/148.4_Pt_1.1018
Eagle M, Baudouin SV, Chandler C, Giddings DR, Bullock R, Bushby K (2002) Survival in Duchenne muscular dystrophy: improvements in life expectancy since 1967 and the impact of home nocturnal ventilation. Neuromuscul Disord 12(10):926–929
Simonds AK, Muntoni F, Heather S, Fielding S (1998) Impact of nasal ventilation on survival in hypercapnic Duchenne muscular dystrophy. Thorax 53(11):949–952
Oskoui M, Levy G, Garland CJ, Gray JM, O'Hagen J, De Vivo DC, Kaufmann P (2007) The changing natural history of spinal muscular atrophy type 1. Neurology 69(20):1931–1936. doi:10.1212/01.wnl.0000290830.40544.b9
Magnus T, Beck M, Giess R, Puls I, Naumann M, Toyka KV (2002) Disease progression in amyotrophic lateral sclerosis: predictors of survival. Muscle Nerve 25(5):709–714
Annane D, Orlikowski D, Chevret S (2014) Nocturnal mechanical ventilation for chronic hypoventilation in patients with neuromuscular and chest wall disorders. Cochrane Database Syst Rev 12:CD001941. doi:10.1002/14651858.CD001941.pub3
Ward S, Chatwin M, Heather S, Simonds AK (2005) Randomised controlled trial of non-invasive ventilation (NIV) for nocturnal hypoventilation in neuromuscular and chest wall disease patients with daytime normocapnia. Thorax 60(12):1019–1024. doi:10.1136/thx.2004.037424
Bach JR, Goncalves MR, Hon A, Ishikawa Y, De Vito EL, Prado F, Dominguez ME (2013) Changing trends in the management of end-stage neuromuscular respiratory muscle failure: recommendations of an international consensus. Am J Phys Med Rehabil 92(3):267–277. doi:10.1097/PHM.0b013e31826edcf1
[No authors listed] (1999) Clinical indications for noninvasive positive pressure ventilation in chronic respiratory failure due to restrictive lung disease, COPD, and nocturnal hypoventilation—a consensus conference report. Chest 116 (2):521–534
Berry RB, Budhiraja R, Gottlieb DJ, Gozal D, Iber C, Kapur VK, Marcus CL, Mehra R, Parthasarathy S, Quan SF, Redline S, Strohl KP, Davidson Ward SL, Tangredi MM, American Academy of Sleep M (2012) Rules for scoring respiratory events in sleep: update of the 2007 AASM manual for the scoring of sleep and associated events. Deliberations of the Sleep Apnea Definitions Task Force of the American Academy of Sleep Medicine. J Clin Sleep Med 8(5):597–619. doi:10.5664/jcsm.2172
Raphael JC, Chevret S, Chastang C, Bouvet F (1994) 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 343(8913):1600–1604
Hukins CA, Hillman DR (2000) Daytime predictors of sleep hypoventilation in Duchenne muscular dystrophy. Am J Respir Crit Care Med 161(1):166–170. doi:10.1164/ajrccm.161.1.9901057
Nardi J, Prigent H, Adala A, Bohic M, Lebargy F, Quera-Salva MA, Orlikowski D, Lofaso F (2012) Nocturnal oximetry and transcutaneous carbon dioxide in home-ventilated neuromuscular patients. Respir Care 57(9):1425–1430. doi:10.4187/respcare.01658
Janssens JP, Borel JC, Pepin JL (2011) Nocturnal monitoring of home non-invasive ventilation: the contribution of simple tools such as pulse oximetry, capnography, built-in ventilator software and autonomic markers of sleep fragmentation. Thorax 66(5):438–445, 10.1136/thx.2010.139782
Loutfi S (2015) Sleep-disordered breathing in neuromuscular disease. Am J Respir Crit Care Med. doi:10.1164/rccm.201412-2224CI
Landis JR, Koch GG (1977) The measurement of observer agreement for categorical data. Biometrics 33(1):159–174
Pinto AC, Evangelista T, Carvalho M, Alves MA, Sales Luis ML (1995) Respiratory assistance with a non-invasive ventilator (Bipap) in MND/ALS patients: survival rates in a controlled trial. J Neurol Sci 129(Suppl):19–26
Nickol AH, Hart N, Hopkinson NS, Moxham J, Simonds A, Polkey MI (2005) Mechanisms of improvement of respiratory failure in patients with restrictive thoracic disease treated with non-invasive ventilation. Thorax 60(9):754–760. doi:10.1136/thx.2004.039388
Bourke SC, Tomlinson M, Williams TL, Bullock RE, Shaw PJ, Gibson GJ (2006) Effects of non-invasive ventilation on survival and quality of life in patients with amyotrophic lateral sclerosis: a randomised controlled trial. Lancet Neurol 5(2):140–147. doi:10.1016/S1474-4422(05)70326-4
Jackson CE, Rosenfeld J, Moore DH, Bryan WW, Barohn RJ, Wrench M, Myers D, Heberlin L, King R, Smith J, Gelinas D, Miller RG (2001) A preliminary evaluation of a prospective study of pulmonary function studies and symptoms of hypoventilation in ALS/MND patients. J Neurol Sci 191(1–2):75–78
Nishimura Y, Hida W, Taguchi O, Sakurai M, Ichinose M, Inoue H, Takishima T (1989) Respiratory muscle strength and gas exchange in neuromuscular diseases: comparison with chronic pulmonary emphysema and idiopathic pulmonary fibrosis. Tohoku J Exp Med 159(1):57–68
Ries AL (1987) Oximetry—know thy limits. Chest 91(3):316
Fletcher EC, Scott D, Qian W, Luckett RA, Miller CC, Goodnight-White S (1991) Evolution of nocturnal oxyhemoglobin desaturation in patients with chronic obstructive pulmonary disease and a daytime PaO2 above 60 mm Hg. Am Rev Respir Dis 144(2):401–405. doi:10.1164/ajrccm/144.2.401
Berlowitz DJ, Spong J, O'Donoghue FJ, Pierce RJ, Brown DJ, Campbell DA, Catcheside PG, Gordon I, Rochford PD (2011) Transcutaneous measurement of carbon dioxide tension during extended monitoring: evaluation of accuracy and stability, and an algorithm for correcting calibration drift. Respir Care 56(4):442–448. doi:10.4187/respcare.00454
Senn O, Clarenbach CF, Kaplan V, Maggiorini M, Bloch KE (2005) Monitoring carbon dioxide tension and arterial oxygen saturation by a single earlobe sensor in patients with critical illness or sleep apnea. Chest 128(3):1291–1296. doi:10.1378/chest.128.3.1291
Storre JH, Magnet FS, Dreher M, Windisch W (2011) Transcutaneous monitoring as a replacement for arterial PCO(2) monitoring during nocturnal non-invasive ventilation. Respir Med 105(1):143–150. doi:10.1016/j.rmed.2010.10.007
Chhajed PN, Miedinger D, Baty F, Bernasconi M, Heuss LT, Leuppi JD, Tamm M (2010) Comparison of combined oximetry and cutaneous capnography using a digital sensor with arterial blood gas analysis. Scand J Clin Lab Invest 70(1):60–64. doi:10.3109/00365510903450106
Author contributions
AO, MQS, HP, GM, IV, DA, FL and DO: designed the experiment; AO, MQS, HP, DA and DO: conducted the research; AO, GM, IV, FL and DO: analysed the data and performed the statistical analyses; AO, HP and DO: wrote the manuscript; AO and DO: have primary responsibility for the integrity of the data and the accuracy of the data analysis. All authors had full access to all of the data (including statistical reports and tables) in the study, revised the manuscript for important intellectual content and approved the final version of the manuscript.
Conflict of interest
A. Ogna, G. Mroue, I. Vaugier and D. Annane declare that they have no conflict of interest. MA. Quera Salva acts as consultant for Servier France and Vanda Pharmaceuticals for sleep medications. Prof F. Lofaso and H. Prigent have no conflict of interest related to the present work to disclose; the Service de Physiologie-Explorations Fonctionnelles of Garches received research funds from ResMed France, not related to the present work. Pr. D. Orlikowski has no conflict of interest related to the present work. The CIC 14.29 of Garches received research funds from BREAS Medical for a project on end-tidal CO2.
Compliance with ethical standards
The study was conducted in accordance with the Declaration of Helsinki and was approved by the French national regulatory board (CNIL, N.1817118). For this type of study, formal patients’ consent is not required.
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Ogna, A., Quera Salva, MA., Prigent, H. et al. Nocturnal hypoventilation in neuromuscular disease: prevalence according to different definitions issued from the literature. Sleep Breath 20, 575–581 (2016). https://doi.org/10.1007/s11325-015-1247-2
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DOI: https://doi.org/10.1007/s11325-015-1247-2