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
Further Information

Publication History

Publication Date:
18 May 2009 (online)

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.

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
  • 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
  • 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
  • 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
  • 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
  • 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
  • 7 Bergofsky E H, Turino G M, Fishman A P. Cardiorespiratory failure in kyphoscoliosis.  Medicine (Baltimore). 1959;  38 263-317
  • 8 Casey K R, Cantillo K O, Brown L K. Sleep-related hypoventilation/hypoxemic syndromes.  Chest. 2007;  131 1936-1948
  • 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
  • 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
  • 11 Motley H L, Werko L et al.. Observations on the clinical use of intermittent positive pressure.  J Aviat Med. 1947;  18 417-435
  • 12 Hillberg R E, Johnson D C. Noninvasive ventilation.  N Engl J Med. 1997;  337 1746-1752
  • 13 Nava S, Navalesi P, Gregoretti C. Interfaces and humidification for noninvasive mechanical ventilation.  Respir Care. 2009;  54 71-84
  • 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
  • 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
  • 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
  • 17 Sampson M G, Grassino K. Neuromechanical properties in obese patients during carbon dioxide rebreathing.  Am J Med. 1983;  75 81-90
  • 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
  • 19 Jones R L, Nzekwu M M. The effects of body mass index on lung volumes.  Chest. 2006;  130 827-833
  • 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
  • 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
  • 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
  • 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
  • 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
  • 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
  • 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
  • 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
  • 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
  • 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
  • 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
  • 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
  • 32 Giraudoux J. Ondine, a Romantic Fantasy in Three Acts. English version by M. Valency. New York; Samuel French 1956
  • 33 Severinghaus J W, Mitchell R A. Ondine's curse: failure of respiratory center automaticity while awake.  Clin Res. 1962;  10 122
  • 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
  • 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
  • 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
  • 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
  • 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
  • 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
  • 40 American Thoracic Society . Idiopathic congenital central hypoventilation syndrome: diagnosis and management.  Am J Respir Crit Care Med. 1999;  160 368-373
  • 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
  • 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
  • 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
  • 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
  • 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
  • 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
  • 47 Ali A, Flageole H. Diaphragmatic pacing for the treatment of congenital central alveolar hypoventilation syndrome.  J Pediatr Surg. 2008;  43 792-796
  • 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
  • 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
  • 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
  • 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
  • 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
  • 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
  • 54 Bach J R, Ishikawa Y, Kim H. Prevention of pulmonary morbidity for patients with Duchenne muscular dystrophy.  Chest. 1997;  112 1024-1028
  • 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
  • 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
  • 57 Mulder D W, Howard Jr F M. Patient resistance and prognosis in amyotrophic lateral sclerosis.  Mayo Clin Proc. 1976;  51 537-541
  • 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
  • 59 Bach J R. Amyotrophic lateral sclerosis: prolongation of life by noninvasive respiratory aids.  Chest. 2002;  122 92-98
  • 60 Bergofsky E H, Turino G M, Fishman A P. Cardiorespiratory failure in kyphoscoliosis.  Medicine (Baltimore). 1959;  38 263-317
  • 61 Sawicka E H, Branthwaite M A. Respiration during sleep in kyphoscoliosis.  Thorax. 1987;  42 801-808
  • 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
  • 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
  • 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
  • 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
  • 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
  • 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
  • 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
  • 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
  • 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
  • 71 Gozal D. Nocturnal ventilatory support in patients with cystic fibrosis: comparison with supplemental oxygen.  Eur Respir J. 1997;  10 1999-2003
  • 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
  • 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
  • 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)

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