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Semin Respir Crit Care Med 2009; 30(3): 359-366
DOI: 10.1055/s-0029-1222444
© Thieme Medical Publishers

Therapy of Hypoventilation

Salman R. Khan1 , Patrick J. Strollo1
  • 1Division of Pulmonary, Allergy, and Critical Care Medicine, University of Pittsburgh Medical Center, Montefiore University Hospital, Pittsburgh, Pennsylvania
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Publication History

Publication Date:
18 May 2009 (online)

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ABSTRACT

Hypoventilation can present as the primary manifestation or as a part of the clinical spectrum in a variety of diseases. It often goes unrecognized by clinicians and health care providers, especially if the presentation is subacute. If untreated, it is associated with increased morbidity and mortality. Some of the consequences of hypoventilation (e.g., cor pulmonale and pulmonary hypertension) may be irreversible. It becomes imperative that conditions commonly associated with hypoventilation (e.g., obesity hypoventilation syndrome, muscular dystrophy, and rigid chest wall diseases) be carefully evaluated and appropriate treatment implemented to prevent these complications. The ability to ventilate patients without invasive procedures is now available. These noninvasive therapies can be successfully implemented and are tolerated well by patients. The noninvasive positive pressure ventilation not only improves nocturnal hypoventilation during sleep but may improve muscle strength during the daytime. This review provides an overview of the treatment of hypoventilation in various diseases with emphasis on noninvasive positive pressure therapy. Treatment needs to be individualized to a given patient and the primary pathology. Success is impacted by the experience of the respiratory team caring for the patient.

KEYWORDS

Hypoventilation - therapy - noninvasive positive pressure ventilation (NIPPV)

REFERENCES

  • 1 Barer G R, Howard P, McCurrie J R. The effect of carbon dioxide and changes in blood pH on pulmonary vascular resistance in cats.  Clin Sci. 1967;  32 361-376
    PubMedGoogle Scholar
  • 2 Kiely D G, Cargill R I, Lipworth B J. Effects of hypercapnia on hemodynamic, inotropic, lusitropic, and electrophysiologic indices in humans.  Chest. 1996;  109 1215-1221
    CrossrefPubMedGoogle Scholar
  • 3 Refsum H E. Relationship between state of consciousness and arterial hypoxemia and hypercapnia in patient with pulmonary insufficiency, breathing air.  Clin Sci. 1963;  25 361-367
    PubMedGoogle Scholar
  • 4 Mellins R B, Balfour Jr H H, Turino G M, Winters R W. Failure of automatic control of ventilation (Ondine's curse): report of an infant born with this syndrome and review of the literature.  Medicine (Baltimore). 1970;  49 487-504
    PubMedGoogle Scholar
  • 5 Bickelmann A G, Burwell C S, Robin E D, Whaley R D. Extreme obesity associated with alveolar hypoventilation: a Pickwickian syndrome.  Am J Med. 1956;  21 811-818
    CrossrefPubMedGoogle Scholar
  • 6 Serpick A A, Baker E L, Woodward T E. Motor system disease: review and discussion of a case presenting with alveolar hypoventilation.  Arch Intern Med. 1965;  115 192-197
    PubMedGoogle Scholar
  • 7 Bergofsky E H, Turino G M, Fishman A P. Cardiorespiratory failure in kyphoscoliosis.  Medicine (Baltimore). 1959;  38 263-317
    PubMedGoogle Scholar
  • 8 Casey K R, Cantillo K O, Brown L K. Sleep-related hypoventilation/hypoxemic syndromes.  Chest. 2007;  131 1936-1948
    CrossrefPubMedGoogle Scholar
  • 9 Gilmartin G S, Tamisier R, Curley M, Weiss J W. Ventilatory, hemodynamic, sympathetic nervous system, and vascular reactivity changes after recurrent nocturnal sustained hypoxia in humans.  Am J Physiol Heart Circ Physiol. 2008;  295 H778-H785
    CrossrefPubMedGoogle Scholar
  • 10 Drinker P, Shaw L A. An apparatus for the prolonged administration of artificial respiration, I: A design for adults and children.  J Clin Invest. 1929;  7 229-247
    PubMedGoogle Scholar
  • 11 Motley H L, Werko L et al.. Observations on the clinical use of intermittent positive pressure.  J Aviat Med. 1947;  18 417-435
    PubMedGoogle Scholar
  • 12 Hillberg R E, Johnson D C. Noninvasive ventilation.  N Engl J Med. 1997;  337 1746-1752
    CrossrefPubMedGoogle Scholar
  • 13 Nava S, Navalesi P, Gregoretti C. Interfaces and humidification for noninvasive mechanical ventilation.  Respir Care. 2009;  54 71-84
    PubMedGoogle Scholar
  • 14 Bickelmann A G, Burwell C S, Robin E D, Whaley R D. Extreme obesity associated with alveolar hypoventilation: a Pickwickian syndrome.  Am J Med. 1956;  21 811-818
    CrossrefPubMedGoogle Scholar
  • 15 Naimark A, Cherniack R M. Compliance of the respiratory system and its components in health and obesity.  J Appl Physiol. 1960;  15 377-382
    PubMedGoogle Scholar
  • 16 Kress J P, Pohlman A S, Alverdy J, Hall J B. The impact of morbid obesity on oxygen cost of breathing (VO(2RESP)) at rest.  Am J Respir Crit Care Med. 1999;  160 883-886
    CrossrefPubMedGoogle Scholar
  • 17 Sampson M G, Grassino K. Neuromechanical properties in obese patients during carbon dioxide rebreathing.  Am J Med. 1983;  75 81-90
    PubMedGoogle Scholar
  • 18 Mokhlesi B, Tulaimat A, Faibussowitsch I, Wang Y, Evans A T. Obesity hypoventilation syndrome: prevalence and predictors in patients with obstructive sleep apnea.  Sleep Breath. 2007;  11 117-124
    CrossrefPubMedGoogle Scholar
  • 19 Jones R L, Nzekwu M M. The effects of body mass index on lung volumes.  Chest. 2006;  130 827-833
    CrossrefPubMedGoogle Scholar
  • 20 Pérez de Llano L A, Golpe R, Piquer M O et al.. Clinical heterogeneity among patients with obesity hypoventilation syndrome: therapeutic implications.  Respiration. 2008;  75 34-39
    CrossrefPubMedGoogle Scholar
  • 21 Pérez de Llano L A, 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
    CrossrefPubMedGoogle Scholar
  • 22 Heinemann F, Budweiser S, Dobroschke J, Pfeifer M. Non-invasive positive pressure ventilation improves lung volumes in the obesity hypoventilation syndrome.  Respir Med. 2007;  101 1229-1235
    CrossrefPubMedGoogle Scholar
  • 23 Delay J M, Sebbane M, Jung B et al.. The effectiveness of noninvasive positive pressure ventilation to enhance preoxygenation in morbidly obese patients: a randomized controlled study.  Anesth Analg. 2008;  107 1707-1713
    CrossrefPubMedGoogle Scholar
  • 24 Chouri-Pontarollo N, Borel J C, Tamisier R, Wuyam B, Levy P, Pépin J L. Impaired objective daytime vigilance in obesity-hypoventilation syndrome: impact of noninvasive ventilation.  Chest. 2007;  131 148-155
    CrossrefPubMedGoogle Scholar
  • 25 Banerjee D, Yee B J, Piper A J, Zwillich C W, Grunstein R R. Obesity hypoventilation syndrome: hypoxemia during continuous positive airway pressure.  Chest. 2007;  131 1678-1684
    CrossrefPubMedGoogle Scholar
  • 26 Berger K I, Ayappa I, Chatr-Amontri B et al.. Obesity hypoventilation syndrome as a spectrum of respiratory disturbances during sleep.  Chest. 2001;  120 1231-1238
    CrossrefPubMedGoogle Scholar
  • 27 Piper A J, Wang D, Yee B J, Barnes D J, Grunstein R R. Randomised trial of CPAP vs bilevel support in the treatment of obesity hypoventilation syndrome without severe nocturnal desaturation.  Thorax. 2008;  63 395-401
    CrossrefPubMedGoogle Scholar
  • 28 Storre J H, Seuthe B, Fiechter R et al.. Average volume-assured pressure support in obesity hypoventilation: a randomized crossover trial.  Chest. 2006;  130 815-821
    CrossrefPubMedGoogle Scholar
  • 29 Budweiser S, Riedl S G, Jörres R A, Heinemann F, Pfeifer M. Mortality and prognostic factors in patients with obesity-hypoventilation syndrome undergoing noninvasive ventilation.  J Intern Med. 2007;  261 375-383
    CrossrefPubMedGoogle Scholar
  • 30 Sugerman H J, Fairman R P, Sood R K, Engle K, Wolfe L, Kellum J M. Long-term effects of gastric surgery for treating respiratory insufficiency of obesity.  Am J Clin Nutr. 1992;  55(2, Suppl) 597S-601S
    PubMedGoogle Scholar
  • 31 Martí-Valeri C, Sabaté A, Masdevall C, Dalmau A. Improvement of associated respiratory problems in morbidly obese patients after open Roux-en-Y gastric bypass.  Obes Surg. 2007;  17 1102-1110
    CrossrefPubMedGoogle Scholar
  • 32 Giraudoux J. Ondine, a Romantic Fantasy in Three Acts. English version by M. Valency. New York; Samuel French 1956
    Google Scholar
  • 33 Severinghaus J W, Mitchell R A. Ondine's curse: failure of respiratory center automaticity while awake.  Clin Res. 1962;  10 122
    PubMedGoogle Scholar
  • 34 Mellins R B, Balfour Jr H H, Turino G M, Winters R W. Failure of automatic control of ventilation (Ondine's curse): report of an infant born with this syndrome and review of the literature.  Medicine (Baltimore). 1970;  49 487-504
    PubMedGoogle Scholar
  • 35 Weese-Mayer D E, Berry-Kravis E M, Zhou L et al.. Idiopathic congenital central hypoventilation syndrome: analysis of genes pertinent to early autonomic nervous system embryologic development and identification of mutations in PHOX2b.  Am J Med Genet A. 2003;  123A 267-278
    CrossrefPubMedGoogle Scholar
  • 36 Amiel J, Laudier B, Attié-Bitach T et al.. Polyalanine expansion and frameshift mutations of the paired-like homeobox gene PHOX2B in congenital central hypoventilation syndrome.  Nat Genet. 2003;  33 459-461
    CrossrefPubMedGoogle Scholar
  • 37 Haddad G G, Mazza N M, Defendini R et al.. Congenital failure of autonomic control of ventilation, gastrointestinal motility and heart rate.  Medicine. 1978;  5 517-526
    PubMedGoogle Scholar
  • 38 Paton J Y, Swaminathan S, Sargent C W, Keens T G. Hypoxic and hypercapnic ventilatory responses in awake children with congenital central hypoventilation syndrome.  Am Rev Respir Dis. 1989;  140 368-372
    PubMedGoogle Scholar
  • 39 Barratt S, Kendrick A H, Buchanan F, Whittle A T. Central hypoventilation with PHOX2B expansion mutation presenting in adulthood.  Thorax. 2007;  62 919-920
    CrossrefPubMedGoogle Scholar
  • 40 American Thoracic Society . Idiopathic congenital central hypoventilation syndrome: diagnosis and management.  Am J Respir Crit Care Med. 1999;  160 368-373
    PubMedGoogle Scholar
  • 41 Oren J, Kelly D H, Shannon D C. Long-term follow-up of children with congenital central hypoventilation syndrome.  Pediatrics. 1987;  80 375-380
    PubMedGoogle Scholar
  • 42 Hartmann H, Jawad M H, Noyes J, Samuels M P, Southall D P. Negative extrathoracic pressure ventilation in central hypoventilation syndrome.  Arch Dis Child. 1994;  70 418-423
    CrossrefPubMedGoogle Scholar
  • 43 Tibballs J, Henning R D. Noninvasive ventilatory strategies in the management of a newborn infant and three children with congenital central hypoventilation syndrome.  Pediatr Pulmonol. 2003;  36 544-548
    CrossrefPubMedGoogle Scholar
  • 44 Ramesh P, Boit P, Samuels M. Mask ventilation in the early management of congenital central hypoventilation syndrome.  Arch Dis Child Fetal Neonatal Ed. 2008;  93 F400-F403
    CrossrefPubMedGoogle Scholar
  • 45 Hunt C E, Brouillette R T, Weese-Mayer D E, Morrow A, Ilbawi M N. Diaphragm pacing in infants and children.  Pacing Clin Electrophysiol. 1988;  11(11 Pt 2) 2135-2141
    CrossrefPubMedGoogle Scholar
  • 46 Flageole H, Adolph V R, Davis G M, Laberge J M, Nguyen L T, Guttman F M. Diaphragmatic pacing in children with congenital central alveolar hypoventilation syndrome.  Surgery. 1995;  118 25-28
    CrossrefPubMedGoogle Scholar
  • 47 Ali A, Flageole H. Diaphragmatic pacing for the treatment of congenital central alveolar hypoventilation syndrome.  J Pediatr Surg. 2008;  43 792-796
    CrossrefPubMedGoogle Scholar
  • 48 Suresh S, Wales P, Dakin C, Harris M A, Cooper D G. Sleep-related breathing disorder in Duchenne muscular dystrophy: disease spectrum in the paediatric population.  J Paediatr Child Health. 2005;  41 500-503
    CrossrefPubMedGoogle Scholar
  • 49 Takasugi T, Ishihara T, Kawamura J et al.. Respiratory disorders during sleep in Duchenne muscular dystrophy [in Japanese].  Nihon Kyobu Shikkan Gakkai Zasshi. 1995;  33 821-828
    PubMedGoogle Scholar
  • 50 Phillips M F, Smith P EM, Carroll N, Edwards R HT, Calverley P M. Nocturnal oxygenation and prognosis in Duchenne muscular dystrophy.  Am J Respir Crit Care Med. 1999;  160 198-202
    PubMedGoogle Scholar
  • 51 Dreher M, Rauter I, Storre J H, Geiseler J, Windisch W. When should home mechanical ventilation be started in patients with different neuromuscular disorders?.  Respirology. 2007;  12 749-753
    CrossrefPubMedGoogle Scholar
  • 52 Soudon P, Steens M, Toussaint M. A comparison of invasive versus noninvasive full-time mechanical ventilation in Duchenne muscular dystrophy.  Chron Respir Dis. 2008;  5 87-93
    CrossrefPubMedGoogle Scholar
  • 53 Simonds A K, Muntoni F, Heather S, Fielding S. Impact of nasal ventilation on survival in hypercapnic Duchenne muscular dystrophy.  Thorax. 1998;  53 949-952
    CrossrefPubMedGoogle Scholar
  • 54 Bach J R, Ishikawa Y, Kim H. Prevention of pulmonary morbidity for patients with Duchenne muscular dystrophy.  Chest. 1997;  112 1024-1028
    CrossrefPubMedGoogle Scholar
  • 55 Ward S, Chatwin M, Heather S, Simonds A K. Randomised controlled trial of non-invasive ventilation (NIV) for nocturnal hypoventilation in neuromuscular and chest wall disease patients with daytime normocapnia.  Thorax. 2005;  60 1019-1024
    CrossrefPubMedGoogle Scholar
  • 56 Velasco M V, Colin A A, Zurakowski D, Darras B T, Shapiro F. Posterior spinal fusion for scoliosis in Duchenne muscular dystrophy diminishes the rate of respiratory decline.  Spine. 2007;  32 459-465
    CrossrefPubMedGoogle Scholar
  • 57 Mulder D W, Howard Jr F M. Patient resistance and prognosis in amyotrophic lateral sclerosis.  Mayo Clin Proc. 1976;  51 537-541
    PubMedGoogle Scholar
  • 58 Bourke S C, Tomlinson M, Williams T L, Bullock R E, Shaw P J, Gibson G J. 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
    CrossrefPubMedGoogle Scholar
  • 59 Bach J R. Amyotrophic lateral sclerosis: prolongation of life by noninvasive respiratory aids.  Chest. 2002;  122 92-98
    CrossrefPubMedGoogle Scholar
  • 60 Bergofsky E H, Turino G M, Fishman A P. Cardiorespiratory failure in kyphoscoliosis.  Medicine (Baltimore). 1959;  38 263-317
    PubMedGoogle Scholar
  • 61 Sawicka E H, Branthwaite M A. Respiration during sleep in kyphoscoliosis.  Thorax. 1987;  42 801-808
    PubMedGoogle Scholar
  • 62 Masa J F, Celli B R, Riesco J A, Sánchez de Cos J, Disdier C, Sojo A. Noninvasive positive pressure ventilation and not oxygen may prevent overt ventilatory failure in patients with chest wall diseases.  Chest. 1997;  112 207-213
    CrossrefPubMedGoogle Scholar
  • 63 Gonzalez C, Ferris G, Diaz J, Fontana I, Nuñez J, Marín J. Kyphoscoliotic ventilatory insufficiency: effects of long-term intermittent positive-pressure ventilation.  Chest. 2003;  124 857-862
    CrossrefPubMedGoogle Scholar
  • 64 Laserna E, Barrot E, Beiztegui A et al.. Non-invasive ventilation in kyphoscoliosis: a comparison of a volumetric ventilator and a BIPAP support pressure device [in Spanish].  Arch Bronconeumol. 2003;  39 13-18
    CrossrefPubMedGoogle Scholar
  • 65 Gustafson T, Franklin K A, Midgren B, Pehrsson K, Ranstam J, Ström K. Survival of patients with kyphoscoliosis receiving mechanical ventilation or oxygen at home.  Chest. 2006;  130 1828-1833
    CrossrefPubMedGoogle Scholar
  • 66 Waldhausen J H, Redding G J, Song K M. Vertical expandable prosthetic titanium rib for thoracic insufficiency syndrome: a new method to treat an old problem.  J Pediatr Surg. 2007;  42 76-80
    CrossrefPubMedGoogle Scholar
  • 67 Cutting G R, Zeitlin P L. Genetics and pathophysiology of cystic fibrosis. In: Chernick V, Boat TF, Wilmott RW, Bush A Kendig's Disorders of the Respiratory Tract in Children. 7th ed. Philadelphia; WB Saunders 2003: 848-901
    Google Scholar
  • 68 Hodson M E, Madden B P, Steven M H, Tsang V T, Yacoub M H. Non-invasive mechanical ventilation for cystic fibrosis patients: a potential bridge to transplantation.  Eur Respir J. 1991;  4 524-527
    PubMedGoogle Scholar
  • 69 Piper A J, Parker S, Torzillo P J, Sullivan C E, Bye P TP. Nocturnal nasal IPPV stabilizes patients with cystic fibrosis and hypercapnic respiratory failure.  Chest. 1992;  102 846-850
    CrossrefPubMedGoogle Scholar
  • 70 Young A C, Wilson J W, Kotsimbos T C, Naughton M T. Randomised placebo controlled trial of non-invasive ventilation for hypercapnia in cystic fibrosis.  Thorax. 2008;  63 72-77
    CrossrefPubMedGoogle Scholar
  • 71 Gozal D. Nocturnal ventilatory support in patients with cystic fibrosis: comparison with supplemental oxygen.  Eur Respir J. 1997;  10 1999-2003
    CrossrefPubMedGoogle Scholar
  • 72 Meduri G U, Conoscenti C C, Menashe P, Nair S. Noninvasive face mask ventilation in patients with acute respiratory failure.  Chest. 1989;  95 865-870
    PubMedGoogle Scholar
  • 73 Wijkstra P J, Lacasse Y, Guyatt G H et al.. A meta-analysis of nocturnal noninvasive positive pressure ventilation in patients with stable COPD.  Chest. 2003;  124 337-343
    CrossrefPubMedGoogle Scholar
  • 74 McEvoy R D, Pierce R J, Hillman D et al.. Nocturnal non-invasive ventilation in stable hypercapnic COPD: a randomized controlled trial.  Thorax. 2009;  , February 12 (Epub ahead of print)
    PubMedGoogle Scholar

Patrick J StrolloJr. M.D. 

Division of Pulmonary, Allergy, and Critical Care Medicine, University of Pittsburgh Medical Center

Montefiore University Hospital, Ste. S643, 3459 Fifth Ave., Pittsburgh, PA 15213

Email: strollopj@upmc.edu