Basics
Description
- Cheyne-Stokes respiration (CSR) was initially described by Cheyne in 1818 and then by Stokes in 1854.
- Characterized by cyclic variations in minute ventilation
- Periods of hyperpnea alternate with periods of hypopnea and apnea. Sleep is interrupted with frequent arousals. These arousals usually occur during early non-REM sleep (stages 1 and 2), resulting in reduced REM sleep.
- Commonly occurs in patients with CHF and neurologic disorders (cerebral hemorrhage infarct or embolism, meningitis, trauma, tumor)
- Also can be seen with lactic acidosis, diabetic ketoacidosis, and uremic coma and altitude acclimatization.
- Mechanism responsible is unclear: However, several theories have been proposed.
- System(s) affected: Respiratory; Cardiovascular
- Synonym(s): Cyclic respiration; Periodic breathing
Can be seen in premature and full-term infants.
Epidemiology
Males > Females
Incidence
40-50% of patients with chronic CHF, 25% of cases of cerebral embolism, and 10% of cerebral infarcts
Risk Factors
Predisposing disorders such as:
- CHF
- Neurologic disorders
- Renal failure
- High altitude
- Narcotics
Pathophysiology
- CSR is thought to result from interaction of several factors including oxygen and carbon dioxide levels in the blood, increased sensitivity of chemo receptors, failing heart/increased circulatory time, O2 and CO2 stores in the body, lung gas stores, metabolic rate, DLco, and PCWP.
- Heart failure causes pulmonary edema/increased pulmonary venous pressures. This leads to stimulation of "J" receptors and hyperventilation during nights and also at daytime. As a result of this Paco2 falls below the apnea threshold thus triggering central apneas. This apnea causes increase in Paco2 and decrease in Pao2. This is sensed by the carotid body, which initiates a new cycle of hyperventilation in an attempt to restore normal Paco2 levels. However, this results in overshoot leading to initiation of another apneic episode.
- Naughton et al. studied heart failure patients and showed that CSR in these patients is initiated by a period of hyperventilation and not apnea.
- High chemoreceptor sensitivity promotes exaggerated ventilatory response, leading to overcorrection and instability in blood gases.
- Smaller lung volumes also favor instability, as they cannot effectively dampen the changes in Paco2 and Pao2.
- OSA and CSA may coexist. The principal defect in OSA is with pharyngeal muscle structure and function. In CSA, the principal defect is in ventilatory control. In OSA, ventilatory efforts continue during apnea, and awakenings lead to airway patency and resumed breathing, whereas in CSA there are no ventilatory efforts during apnea.
Diagnosis
Signs and symptoms:
- Daytime sleepiness
- Fatigue
- Paroxysmal nocturnal dyspnea
- Snoring
- Witnessed apnea
- Dyspnea
- Orthopnea
History
History of one of the following:
- CHF
- Neurologic disorder (cerebral hemorrhage, infarct or embolism, meningitis, tumor, trauma)
- Renal failure/uremic coma
- Acidosis (lactic acidosis, diabetic ketoacidosis)
Tests
Polysomnography can verify diagnosis and rule out obstructive sleep apnea.
Lab
Blood gas may reveal a respiratory alkalosis.
Differential Diagnosis
- Obstructive sleep apnea
- Ataxic breathing
- Neurogenic hyperventilation
- Neurogenic hypoventilation
Treatment
- Currently, there is no consensus or enough evidence to recommend definitive treatment for all patients with CSA.
- Treatment should be focused on the underlying disorder.
- In heart failure patients, general recommendations for treatment of heart failure such as β-blockers, ACEI, biventricular pacing should be considered.
- Aggressive treatment of heart failure with medications such as ACEI results in significant reduction in episodes of oxygen desaturations.
- Treatment of anemia with agents such as iron and erythropoietin has been shown to reduce AHI.
- Positive-pressure support such as CPAP, BPAP, ASV have been shown to be beneficial. However, large randomized clinical trials are further needed to assess their long-term benefit. Based on the current available evidence, it seems reasonable to consider PAP in patients with severe daytime somnolence or patients who are at high risk of suffering from cardiovascular events due to hemodynamic changes during CSR.
- Data on the long-term use of supplemental oxygen is inconsistent.
- High-frequency jet ventilation as a possible treatment is also under study.
Medication
- Respiratory stimulants, such as theophylline has been used. But its long term safety profile is not known. Benefit of treatment should be weighed against the potential of inducing ventricular arrhythmia in a poorly functioning heart
- Acetazolamide acts by stimulating the respiratory center by causing mild metabolic acidosis. It also may cause central and peripheral chemoreceptor depression.
- A small study also showed that temazepam may be helpful.
- None of the above drugs are recommended as 1st-line agents.
- Contraindications: Narcotics
- Precautions: Refer to manufacturer's profile of each drug.
- Theophylline: May increase risk of arrhythmias
- Acetazolamide: Causes a metabolic acidosis
- Significant possible interactions: Many drugs can affect theophylline levels.
Additional Treatment
Issues for Referral
- Patients with CSR have increased mortality if a respiratory alkalosis exists.
- In patients with CHF, the development of CSR is associated with increased mortality independent of LV function.
Ongoing Care
Patient Education
Physical training may increase exercise tolerance and decrease abnormal ventilation.
Additional Reading
1Aldabal L, BaHammam AS. Cheyne-Stokes respiration in patients with heart failure. Lung. 03 December 2009.2Baum GL, Crapo JD, Celli BR Textbook of Pulmonary Medicine, 6th ed. Philadelphia: Lippincott-Raven, 1998.3Crystal RG, West JB, Weibel ER The lung. Philadelphia: Lippincott Raven, 1997.4George RB, Matthay MA, Light RW Chest Medicine: Essentials of Pulmonary and Critical Care Medicine, 3rd ed. Baltimore: Williams & Wilkins, 1995.5Lange RL, Hecht HH. The mechanism of Cheyne-Stokes respiration. J Clin Invest. 1962;41(1).6Lorenzi-Filho G, Genta PR. A new straw in the genesis of Cheyne-Stokes respiration. Chest. 2008;134(1):7-97Lorenzi-Filho G, Genta PR, Figueiredo AC. Cheyne-Stokes respiration in patients with congestive heart failure: Causes and consequences. Clinics. 2005;60:333-344. [View Abstract]8Naughton M, Benard D, Tam A. Role of hyperventilation in the pathogenesis of central sleep apneasin patients with congestive heart failure. Am Rev Respir Dis. 1993;148:330-338. [View Abstract]9Piepoli MF. Aetiology and pathophysiological implications of oscillatory ventilation at rest and during exercise in chronic heart failure. Do Cheyne and Stokes have an important message for modern-day patients with heart failure? Eur Heart J. 1999;20:946-953. [View Abstract]10Quaranta AJ, D'Alonzo GE, Krachman SL. Cheyne-Stokes respiration during sleep in congestive heart failure. Chest. 1997;111:467-473. [View Abstract]11Sin DD, Fitzgerald F, Parker JD, Newton G. Risk factors for central and obstructive sleep apnea in 450 men and women with congestive heart failure. Am J Respir Crit Care Med. 1999;160(4):1101-1106. [View Abstract]12Solin P, Bergin P, Richardson M (1999) Influence of pulmonary capillary wedge pressure on central apnea in heart failure. Circulation. 1999;99(12):1574-1579. [View Abstract]13Szollosi I, Thompson B, Krum H. Impaired pulmonary diffusing capacity and hypoxia in heart failure correlates with central sleep apnea severity. Chest. 2008;134:67-72. [View Abstract]14Wilcox I, McNamara SG, Wessendorf T. Prognosis and sleep disordered breathing in heart failure. Thorax. 1998;53(Suppl 3):33-36.
Codes
ICD9
786.04 Cheyne-stokes respiration
SNOMED
309155007 Cheyne-Stokes respiration (finding)