Treatment and prognosis of the obesity hypoventilation syndrome
- Thomas J Martin, MD
Thomas J Martin, MD
- Associate Professor of Medicine
- Virginia Tech-Carilion School of Medicine
- Assistant Professor of Medicine
- Edward Via Virginia College of Osteopathic Medicine
- Associate Professor of Clinical Medicine
- University of Virginia School of Medicine
Obesity hypoventilation syndrome (OHS; "pickwickian syndrome") exists when an obese individual (body mass index [BMI] >30kg/m2) has awake alveolar hypoventilation (arterial carbon dioxide tension [PaCO2] >45 mmHg), which cannot be attributed to other conditions (eg, neuromuscular disease). Untreated OHS is a progressive disorder that is associated with significant morbidity such that prompt recognition and treatment is critical.
The treatment and prognosis of OHS are reviewed here. The clinical manifestations, diagnosis, complications, and pathogenesis of OHS are discussed separately. (See "Clinical manifestations and diagnosis of obesity hypoventilation syndrome" and "Epidemiology and pathogenesis of obesity hypoventilation syndrome".)
FIRST LINE THERAPY
Noninvasive positive airway pressure (PAP) together with weight loss are the initial first line therapies for patients with OHS. A comprehensive and multidisciplinary approach utilizing experts in obesity, sleep, and pulmonary medicine is recommended.
Positive airway pressure — All patients with OHS have some form of sleep disordered breathing, typically obstructive sleep apnea (OSA; 90 percent) or sleep-related hypoventilation (10 percent), warranting treatment with noninvasive PAP. PAP therapy should not be delayed while the patient tries to lose weight. Selection of the appropriate mode of PAP (eg, continuous PAP [CPAP], bilevel PAP [BPAP], volume cycled or hybrid modes of noninvasive ventilation)  and approach to initiating PAP therapy in patients with OHS are described in detail separately. (See "Noninvasive positive airway pressure therapy of the obesity hypoventilation syndrome".)
Obesity hypoventilation plus obstructive sleep apnea (continuous positive airway pressure) — CPAP is the typical mode chosen for treatment for OHS plus OSA, while BPAP, usually in the spontaneous–timed mode, is indicated in those who fail CPAP. (See "Noninvasive positive airway pressure therapy of the obesity hypoventilation syndrome", section on 'Patients with obesity hypoventilation and obstructive sleep apnea'.)
- Berry RB, Chediak A, Brown LK, et al. Best clinical practices for the sleep center adjustment of noninvasive positive pressure ventilation (NPPV) in stable chronic alveolar hypoventilation syndromes. J Clin Sleep Med 2010; 6:491.
- Sugerman HJ, Fairman RP, Sood RK, et al. Long-term effects of gastric surgery for treating respiratory insufficiency of obesity. Am J Clin Nutr 1992; 55:597S.
- Nguyen NT, Hinojosa MW, Smith BR, et al. Improvement of restrictive and obstructive pulmonary mechanics following laparoscopic bariatric surgery. Surg Endosc 2009; 23:808.
- 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.
- Boone KA, Cullen JJ, Mason EE, et al. Impact of Vertical Banded Gastroplasty on Respiratory Insufficiency of Severe Obesity. Obes Surg 1996; 6:454.
- Lumachi F, Marzano B, Fanti G, et al. Relationship between body mass index, age and hypoxemia in patients with extremely severe obesity undergoing bariatric surgery. In Vivo 2010; 24:775.
- Lumachi F, Marzano B, Fanti G, et al. Hypoxemia and hypoventilation syndrome improvement after laparoscopic bariatric surgery in patients with morbid obesity. In Vivo 2010; 24:329.
- Dávila-Cervantes A, Domínguez-Cherit G, Borunda D, et al. Impact of surgically-induced weight loss on respiratory function: a prospective analysis. Obes Surg 2004; 14:1389.
- Thomas PS, Cowen ER, Hulands G, Milledge JS. Respiratory function in the morbidly obese before and after weight loss. Thorax 1989; 44:382.
- Piper AJ, Grunstein RR. Obesity hypoventilation syndrome: mechanisms and management. Am J Respir Crit Care Med 2011; 183:292.
- Masa JF, Corral J, Alonso ML, et al. Efficacy of Different Treatment Alternatives for Obesity Hypoventilation Syndrome. Pickwick Study. Am J Respir Crit Care Med 2015; 192:86.
- Rapoport DM, Garay SM, Epstein H, Goldring RM. Hypercapnia in the obstructive sleep apnea syndrome. A reevaluation of the "Pickwickian syndrome". Chest 1986; 89:627.
- Camacho M, Teixeira J, Abdullatif J, et al. Maxillomandibular advancement and tracheostomy for morbidly obese obstructive sleep apnea: a systematic review and meta-analysis. Otolaryngol Head Neck Surg 2015; 152:619.
- El Solh AA, Jaafar W. A comparative study of the complications of surgical tracheostomy in morbidly obese critically ill patients. Crit Care 2007; 11:R3.
- Fattahi T, Chafin C, Bunnell A. Tracheostomy in the Morbidly Obese: Difficulties and Challenges. J Oral Maxillofac Surg 2017; 75:1372.
- Darrat I, Yaremchuk K. Early mortality rate of morbidly obese patients after tracheotomy. Laryngoscope 2008; 118:2125.
- Cordes SR, Best AR, Hiatt KK. The impact of obesity on adult tracheostomy complication rate. Laryngoscope 2015; 125:105.
- Khoo SG, Rajaretnam N. Surgical tracheostomy in morbidly obese patients: technical considerations and a two-flap technique for access. J Laryngol Otol 2012; 126:435.
- Gross ND, Cohen JI, Andersen PE, Wax MK. 'Defatting' tracheotomy in morbidly obese patients. Laryngoscope 2002; 112:1940.
- Sugerman HJ. Surgery for morbid obesity. Surgery 1993; 114:865.
- Sugerman HJ, Baron PL, Fairman RP, et al. Hemodynamic dysfunction in obesity hypoventilation syndrome and the effects of treatment with surgically induced weight loss. Ann Surg 1988; 207:604.
- Wijesinghe M, Williams M, Perrin K, et al. The effect of supplemental oxygen on hypercapnia in subjects with obesity-associated hypoventilation: a randomized, crossover, clinical study. Chest 2011; 139:1018.
- Hollier CA, Harmer AR, Maxwell LJ, et al. Moderate concentrations of supplemental oxygen worsen hypercapnia in obesity hypoventilation syndrome: a randomised crossover study. Thorax 2014; 69:346.
- Mokhlesi B, Tulaimat A, Evans AT, et al. Impact of adherence with positive airway pressure therapy on hypercapnia in obstructive sleep apnea. J Clin Sleep Med 2006; 2:57.
- Sutton FD Jr, Zwillich CW, Creagh CE, et al. Progesterone for outpatient treatment of Pickwickian syndrome. Ann Intern Med 1975; 83:476.
- Lyons HA, Huang CT. Therapeutic use of progesterone in alveolar hypoventilation associated with obesity. Am J Med 1968; 44:881.
- Raurich JM, Rialp G, Ibáñez J, et al. Hypercapnic respiratory failure in obesity-hypoventilation syndrome: CO₂ response and acetazolamide treatment effects. Respir Care 2010; 55:1442.
- Powers MA. Obesity hypoventilation syndrome: bicarbonate concentration and acetazolamide. Respir Care 2010; 55:1504.
- Jones JB, Wilhoit SC, Findley LJ, Suratt PM. Oxyhemoglobin saturation during sleep in subjects with and without the obesity-hypoventilation syndrome. Chest 1985; 88:9.
- Orr WC, Imes NK, Martin RJ. Progesterone therapy in obese patients with sleep apnea. Arch Intern Med 1979; 139:109.
- Strohl KP, Hensley MJ, Saunders NA, et al. Progesterone administration and progressive sleep apneas. JAMA 1981; 245:1230.
- Cook WR, Benich JJ, Wooten SA. Indices of severity of obstructive sleep apnea syndrome do not change during medroxyprogesterone acetate therapy. Chest 1989; 96:262.
- Anttalainen U, Saaresranta T, Vahlberg T, Polo O. Short-term medroxyprogesterone acetate in postmenopausal women with sleep-disordered breathing: a placebo-controlled, randomized, double-blind, parallel-group study. Menopause 2014; 21:361.
- SHARP JT, HENRY JP, SWEANY SK, et al. THE TOTAL WORK OF BREATHING IN NORMAL AND OBESE MEN. J Clin Invest 1964; 43:728.
- Vasilakis C, Jick H, del Mar Melero-Montes M. Risk of idiopathic venous thromboembolism in users of progestagens alone. Lancet 1999; 354:1610.
- Douketis JD, Julian JA, Kearon C, et al. Does the type of hormone replacement therapy influence the risk of deep vein thrombosis? A prospective case-control study. J Thromb Haemost 2005; 3:943.
- Martinez FJ, Bermudez-Gomez M, Celli BR. Hypothyroidism. A reversible cause of diaphragmatic dysfunction. Chest 1989; 96:1059.
- Ladenson PW, Goldenheim PD, Ridgway EC. Prediction and reversal of blunted ventilatory responsiveness in patients with hypothyroidism. Am J Med 1988; 84:877.
- MASSUMI RA, WINNACKER JL. SEVERE DEPRESSION OF THE RESPIRATORY CENTER IN MYXEDEMA. Am J Med 1964; 36:876.
- Skatrud J, Iber C, Ewart R, et al. Disordered breathing during sleep in hypothyroidism. Am Rev Respir Dis 1981; 124:325.
- Randerath W, Verbraecken J, Andreas S, et al. Definition, discrimination, diagnosis and treatment of central breathing disturbances during sleep. Eur Respir J 2017; 49.
- Sugerman HJ, Fairman RP, Baron PL, Kwentus JA. Gastric surgery for respiratory insufficiency of obesity. Chest 1986; 90:81.
- Korenkov M, Shah S, Sauerland S, et al. Impact of laparoscopic adjustable gastric banding on obesity co-morbidities in the medium- and long-term. Obes Surg 2007; 17:679.
- Haines KL, Nelson LG, Gonzalez R, et al. Objective evidence that bariatric surgery improves obesity-related obstructive sleep apnea. Surgery 2007; 141:354.
- Lettieri CJ, Eliasson AH, Greenburg DL. Persistence of obstructive sleep apnea after surgical weight loss. J Clin Sleep Med 2008; 4:333.
- Greenburg DL, Lettieri CJ, Eliasson AH. Effects of surgical weight loss on measures of obstructive sleep apnea: a meta-analysis. Am J Med 2009; 122:535.
- Grunstein RR, Stenlöf K, Hedner JA, et al. Two year reduction in sleep apnea symptoms and associated diabetes incidence after weight loss in severe obesity. Sleep 2007; 30:703.
- Dixon JB, Schachter LM, O'Brien PE, et al. Surgical vs conventional therapy for weight loss treatment of obstructive sleep apnea: a randomized controlled trial. JAMA 2012; 308:1142.
- Pérez 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.
- Borel JC, Burel B, Tamisier R, et al. Comorbidities and mortality in hypercapnic obese under domiciliary noninvasive ventilation. PLoS One 2013; 8:e52006.
- Castro-Añón O, Pérez de Llano LA, De la Fuente Sánchez S, et al. Obesity-hypoventilation syndrome: increased risk of death over sleep apnea syndrome. PLoS One 2015; 10:e0117808.
- Berg G, Delaive K, Manfreda J, et al. The use of health-care resources in obesity-hypoventilation syndrome. Chest 2001; 120:377.
- Nowbar S, Burkart KM, Gonzales R, et al. Obesity-associated hypoventilation in hospitalized patients: prevalence, effects, and outcome. Am J Med 2004; 116:1.
- Jennum P, Kjellberg J. Health, social and economical consequences of sleep-disordered breathing: a controlled national study. Thorax 2011; 66:560.
- Budweiser S, Riedl SG, Jörres RA, et al. Mortality and prognostic factors in patients with obesity-hypoventilation syndrome undergoing noninvasive ventilation. J Intern Med 2007; 261:375.
- Priou P, Hamel JF, Person C, et al. Long-term outcome of noninvasive positive pressure ventilation for obesity hypoventilation syndrome. Chest 2010; 138:84.
- FIRST LINE THERAPY
- Positive airway pressure
- - Obesity hypoventilation plus obstructive sleep apnea (continuous positive airway pressure)
- - Obesity hypoventilation plus sleep-related hypoventilation (bilevel positive airway pressure)
- Weight loss
- - Lifestyle modifications
- SECOND LINE THERAPY
- Treatments targeted at obesity hypoventilation and sleep disordered breathing
- - Tracheostomy
- Additional treatments targeted at weight loss
- - Bariatric surgery
- - Medication
- THERAPIES OF LIMITED VALUE OR ASSOCIATED WITH HARM
- Oxygen alone
- Respiratory stimulants
- SUPPORTIVE THERAPIES
- Avoidance of alcohol and sedatives
- Treatment of comorbid conditions
- SUMMARY AND RECOMMENDATIONS