通气不足综合征相关疾病患者的自然病程各不相同,即使经过治疗也不尽相同,具体取决于他们的基础病因。
肥胖-通气不足综合征 (OHS)
与阻塞性睡眠呼吸暂停患者相比,若不加以治疗,即使轻度 OHS 的患者其生活质量也会下降、嗜睡程度会加剧且肺高压会越发严重。此外,与肥胖程度类似但未患 OHS 的患者相比,OHS 患者进入 ICU的概率更高并且更需要机械通气。[104]Nowbar S, Burkart KM, Gonzales R, et al. Obesity-associated hypoventilation in hospitalized patients: prevalence, effects, and outcome. Am J Med. 2004;116:1-7.http://www.ncbi.nlm.nih.gov/pubmed/14706658?tool=bestpractice.com与此相反,经过适当治疗的 OHS 患者的发病率和死亡率都得到了改善。[13]Perez de Llano LA, Golpe R, Ortiz Piquer M, et al. Short-term and long-term effects of nasal intermittent positive pressure ventilation in patients with obesity-hypoventilation syndrome. Chest. 2005;128:587-594.http://journal.publications.chestnet.org/article.aspx?articleid=1083605http://www.ncbi.nlm.nih.gov/pubmed/16100142?tool=bestpractice.com[105]Berg G, Delaive K, Manfreda J, et al. The use of health-care resources in obesity-hypoventilation syndrome. Chest. 2001;120:377-383.http://journal.publications.chestnet.org/article.aspx?articleid=1079910http://www.ncbi.nlm.nih.gov/pubmed/11502632?tool=bestpractice.com[106]Miske LJ, Hickey EM, Kolb SM, et al. Use of the mechanical in-exsufflator in pediatric patients with neuromuscular disease and impaired cough. Chest. 2004;125:1406-1412.http://journal.publications.chestnet.org/data/Journals/CHEST/22007/1406.pdfhttp://www.ncbi.nlm.nih.gov/pubmed/15078753?tool=bestpractice.com
限制性胸部疾病
许多由神经肌肉疾病引发的通气不足综合征患者,其原发疾病的进展将最终影响患者的预后。然而,治疗干预(例如夜间无创通气)能对存活率和生活质量产生显著影响。[43]Lechtzin N, Scott Y, Busse AM, et al. Early use of non-invasive ventilation prolongs survival in subjects with ALS. Amyotroph Lateral Scler. 2007;8:185-188.http://www.ncbi.nlm.nih.gov/pubmed/17538782?tool=bestpractice.com[67]Young HK, Lowe A, Fitzgerald DA, et al. Outcome of noninvasive ventilation in children with neuromuscular disease. Neurology. 2007;68:198-201.http://www.ncbi.nlm.nih.gov/pubmed/17224573?tool=bestpractice.com[68]Piper AJ, Sullivan CE. Effects of long-term nocturnal nasal ventilation on spontaneous breathing during sleep in neuromuscular and chest wall disorders. Eur Respir J. 1996;9:1515-1522.http://erj.ersjournals.com/content/9/7/1515.full.pdf+htmlhttp://www.ncbi.nlm.nih.gov/pubmed/8836668?tool=bestpractice.com[69]Mellies U, Ragette R, Dohna Schwake CD, et al. Longterm noninvasive ventilation in children and adolescents with neuromuscular disorders. Eur Respir J. 2003;22:631-636.http://erj.ersjournals.com/content/22/4/631.fullhttp://www.ncbi.nlm.nih.gov/pubmed/14582916?tool=bestpractice.com[72]Bourke SC, Tomlinson M, Williams TL, et al. Effects of non-invasive ventilation on survival and quality of life in patients with amyotrophic lateral sclerosis: a randomised controlled trial. Lancet Neurol. 2006;5:140-147.http://www.ncbi.nlm.nih.gov/pubmed/16426990?tool=bestpractice.com
COPD
虽然根据疾病严重程度能最终预测慢性阻塞性肺疾病患者的预后,夜间无创通气仍被证实能改善这些患者的气体交换、睡眠质量和生活质量。[95]Krachman SL, Quaranta AJ, Berger TJ, et al. Effects of noninvasive positive pressure ventilation on gas exchange and sleep in COPD patients. Chest. 1997;112:623-628.http://journal.publications.chestnet.org/data/Journals/CHEST/21750/623.pdfhttp://www.ncbi.nlm.nih.gov/pubmed/9315793?tool=bestpractice.com[96]Jones SE, Packham S, Hebden M, et al. Domiciliary nocturnal intermittent positive pressure ventilation in patients with respiratory failure due to severe COPD: long-term follow-up and effect on survival. Thorax. 1998:53:495-498.http://www.ncbi.nlm.nih.gov/pubmed/9713450?tool=bestpractice.com[98]Elliott MW, Simonds AK, Carroll MP, et al. Domiciliary nocturnal nasal intermittent positive pressure ventilation in hypercapnic respiratory failure due to chronic obstructive lung disease: effects on sleep and quality of life. Thorax. 1992;47:342-348.http://www.ncbi.nlm.nih.gov/pubmed/1609376?tool=bestpractice.com 此外,一项研究发现,与单独接受氧疗相比,同时接受无创通气和氧疗的并发高碳酸血症的慢性阻塞性肺疾病患者的存活率提高。[97]McEvoy RD, Pierce JR, Hillman PD, et al. Nocturnal non-invasive nasal ventilation in stable hypercapnic COPD: a randomised controlled trial. Thorax. 2009;64:561-566.http://www.ncbi.nlm.nih.gov/pubmed/19213769?tool=bestpractice.com
陈-施氏呼吸 (CSR)
已经证明尽管在心力衰竭程度相似的情况下,患 CSR 的充血性心力衰竭患者的死亡率高于未患 CSR 的患者。[107]Hanly PJ, Zuberi-Khokhar NS. Increased mortality associated with Cheyne-Stokes respiration in patients with congestive heart failure. Am J Respir Crit Care Med. 1996;153:272-276.http://www.ncbi.nlm.nih.gov/pubmed/8542128?tool=bestpractice.com虽然夜间无创通气已被证实能改善心脏功能并显著改善睡眠障碍性呼吸,但它对未作移植患者的存活率的影响尚不明确。[80]Bradley TD, Logan AG, Kimoff RJ, et al. Continuous positive airway pressure for central sleep apnea and heart failure. N Engl J Med. 2005;353:2025-2033.http://www.nejm.org/doi/full/10.1056/NEJMoa051001#t=articlehttp://www.ncbi.nlm.nih.gov/pubmed/16282177?tool=bestpractice.com[81]Arzt M, Floras JS, Logan AG, et al. Suppression of central sleep apnea by continuous positive airway pressure and transplant-free survival in heart failure. Circulation. 2007;115:3173-3180.http://circ.ahajournals.org/content/115/25/3173.fullhttp://www.ncbi.nlm.nih.gov/pubmed/17562959?tool=bestpractice.com