II. Definitions

  1. Bellow Failure
    1. Respiratory "pump" failure to expand chest and trigger inspiration
    2. Due to insufficient effort or respiratory drive, neuromuscular Impairment, muscle Fatigue, inefficient bellows

III. Background

  1. Respiratory Failure represents a loss of the normal, substantial Ventilatory reserve
    1. In those without Bellows Failure, Minute Ventilation may increase 20 fold over a baseline of 6 l/min

IV. Types: Hypoventilatory Respiratory Failure with Hypercapnia due to Bellows Failure

  1. Defining features
    1. High PaCO2 >50 mmHg
    2. Normal A-a Gradient
      1. Contrast with decompensated COPD in which pCO2 is increased, but A-a Gradient is high
      2. Normal A-a Gradient suggests external cause, with normal lungs and normal alveolar gas exchange
  2. Causes: Compromised lung mechanics
    1. Background
      1. Work of breathing costs increase significantly with impaired lung mechanics
      2. Patients with normal lungs expend only 1 ml oxygen per 1 liter of Minute Ventilation
      3. Patients with impaired lungs may expend 10-20 ml oxygen per 1 liter of Minute Ventilation
      4. Respiratory muscles Fatigue and fail at acute, persistent workloads >40% of maximal workload
    2. Upper airway obstruction
      1. Infection (Epiglottitis, Bacterial Tracheitis, croup)
      2. Adenotonsillar Hypertrophy
      3. Neck Mass
      4. ThyroidGoiter
      5. Obstructive Sleep Apnea
      6. Vocal Cord Paralysis (bilateral)
      7. Laryngeal Foreign Body
    3. Pulmonary muscle Fatigue (Skeletal muscle Fatigues at >40% of maximum load)
      1. Obesity
      2. Supine position
      3. Kyphoscoliosis
      4. Ankylosing Spondylitis
      5. Hypercarbia (fever, Sepsis, burns)
    4. Inefficient breathing
      1. Obstructive Lung Disease (flat diaphragm, high Residual Volume)
        1. Asthma
        2. COPD (Emphysema or Chronic Bronchitis)
      2. Restrictive Lung Disease
        1. Anatomic Dead Space accounts for a large percentage of a fixed, reduced Tidal Volume
    5. Chest Trauma
      1. Pneumothorax
        1. Air in the pleural space prevents negative pressure from forming within the chest
        2. Loss of negative pressure results in lung failing to expand with diaphragmatic excursion
      2. Flail Chest or multiple Rib Fractures
      3. Diaphragmatic Rupture
      4. Hemothorax
    6. Other chest conditions interfering with ventilation
      1. Ascites
      2. Pleural Effusion
      3. Atelectasis
  3. Causes: Loss of Inspiratory Drive (Insufficient Effort)
    1. Drug Overdose or depressant drugs
      1. Opioids
      2. Benzodiazepines
      3. Barbiturates
      4. Procedural Anesthesia (e.g. Propofol)
      5. Phencyclidine (PCP)
    2. Brainstem injury
      1. See Breathing Patterns in Brain Injury
      2. Brainstem Herniation
    3. Severe global CNS injury
      1. Head Trauma
      2. Intracranial Hemorrhage
      3. CNS Infection (Meningitis, Encephalitis, Brain Abscess, West Nile Encephalitis, Poliomyelitis)
      4. Central Sleep Apnea
      5. Central Alveolar Hypoventilation Syndrome (CHS)
    4. CO2 Retention
      1. Blue Bloaters (Chronic Bronchitis)
        1. Obese with hypoventilation despite Hypoxia
        2. Hypercarbia resulting in increased sedation
        3. Cyanotic (polycythemic with increased desaturated Hemoglobin)
  4. Causes: Neuromuscular
    1. Toxins (or other medication adverse effects)
      1. Aminoglycosides
      2. Arsenic
      3. Strychnine
      4. Botulism
    2. Electrolyte and endocrine abnormalities
      1. Hyponatremia
      2. Hypocalcemia
      3. Hypokalemia
      4. Hyperkalemia
      5. Hypomagnesemia
      6. Severe Hypophosphatemia
      7. Hypothyoidism
    3. Nerve dysfunction
      1. Spinal cord injury
      2. Polyneuritis (e.g. Guillain-Barre Syndrome)
      3. Amyotrophic Lateral Sclerosis
      4. Multiple Sclerosis
      5. Nerve Agent Exposure (e.g. Organophosphates)
      6. Phrenic nerve injury
        1. Example: Phrenic Nerve Injury from Birth Trauma
    4. Muscular dysfunction
      1. Congenital Muscular Dystrophy
      2. Myasthenia Gravis
      3. Polymyositis
      4. Tetanus

V. Types: Hypercarbic Respiratory Failure from Diffuse Severe Lung Disease (large Respiratory Dead Space)

  1. Background
    1. Severe, diffuse lung disease with poor gas exchange
      1. pCO2 is easily excreted even through mild-moderately disease alveoli
      2. Hypercarbia requires apnea (Bellows Failure) or diffuse, severely impaired gas exchange
    2. Ventilated lung with poor gas exchange is dead space, wasted ventilation
    3. Perfusion to lung units that receive less ventilation results in blood retaining more CO2 and less O2
    4. Increased respiratory effort cannot fully compensate for increased dead space and CO2 production
  2. Defining features
    1. High PaCO2
      1. May be normal or low in mild to moderate disease compensated with Hyperventilation
      2. However, with decompensation, pCO2 rises
    2. Low PaO2
    3. Increased A-a Gradient
    4. Often improves with Supplemental Oxygen
  3. Causes:
    1. Decompensated Obstructive Lung Disease
      1. Asthma or Bronchospasm
      2. Chronic Obstructive Pulmonary Disease (COPD)
    2. Decompensated Interstitial Lung Disease (e.g. Idiopathic Pulmonary Fibrosis, Sarcoidosis)
    3. Decompensated Cystic Fibrosis

VI. Types: Hypoxemic Respiratory Failure without Hypercarbia

  1. Background
    1. Alveoli fill with fluid (esp. in dependent lung) and are unable to oxygenate
    2. Interstitial fluid results in stiff lungs that ventilate poorly
    3. Small airways collapse
    4. Right to Left intrapulmonary shunt past poorly ventilated lung
    5. Carbon dioxide may still be expired as dead space is not increased (contrast with hypercapnic failure)
      1. CO2 is highly soluble in fluid (contrast with O2) and diffuses well despite fluid filled alveoli
    6. Patient cannot oxygenate despite increased Respiratory Rate and ventilation
      1. Blood in non-edematous lung is fully saturated with oxygen
      2. Blood in edematous lung is not able to oxygenate
  2. Defining features
    1. Low PaCO2
      1. Contrast with Bellows Failure and Decompensated Severe, Diffusely impaired alveolar gas exchange
    2. Low PaO2 <50-60 mmHg on room air
    3. A-a Gradient may be increased
    4. May not improve with Supplemental Oxygen
  3. Causes: Improved with Supplemental Oxygen
    1. Non-specific
    2. May be due to any Hypoxia cause (e.g. mild-moderate COPD, Asthma, CHF, PE)
  4. Causes: Not improved with Supplemental Oxygen (pO2 <50 mmHg despite oxygen)
    1. Suggests Physiologic right to left intrapulmonary shunting (esp. lung edema)
      1. Oxygen and Hyperventilation are unable to compensate for shunted (non-oxygenated) blood
      2. Blood has a fixed ceiling for Oxygen Saturation, above which no further oxygen is absorbed
        1. pO2 increases with FIO2 and alveolar recruitment from diseased, shunted regions
        2. Alveolar recruitment increases with NIPPV (PEEP, CPAP, BiPAP) and Mechanical Ventilation
    2. Cardiac Pulmonary Edema (increased transcapillary pressure)
      1. Left Ventricular Failure
      2. Acute Myocardial Ischemia (left ventricle)
      3. Malignant Hypertension
      4. Mitral Regurgitation or stenosis
    3. Lung Conditions (often with increased capillary permeability)
      1. Severe Lobar Pneumonia
        1. Autoregulatory Vasoconstriction normally decreases perfusion to non-ventilated alveoli
        2. Significant right to left shunt occurs when Vasoconstriction fails to prevent perfusion to infected lung
      2. Pulmonary Contusion
      3. Alveolar Hemorrhage
      4. Acute Respiratory Distress Syndrome (ARDS)
        1. Increased permeability (low pressure edema)
    4. Miscellaneous other causes
      1. Pulmonary Embolism

VII. Types: Miscellaneous Secondary Causes of Hypoxia

IX. Signs

  1. General appearance
    1. Altered Mental Status
    2. Diaphoresis
  2. Increased work of breathing
    1. Accessory muscle use
    2. Intercostal retractions
    3. Tachypnea
    4. Paradoxical breathing patterns
      1. Abdominal wall moves inward with inspiration as respiratory Fatigue occurs
  3. Cardiovascular changes
    1. Mucous membrane and nail bed Cyanosis
    2. Tachycardia
    3. Hypertension

X. Labs

  1. Complete Blood Count
  2. Comprehensive Metabolic Panel
  3. Serum Troponin
  4. Serum Brain Natriuretic Peptide (BNP, NT-proBNP)
  5. D-Dimer
  6. Arterial Blood Gas
    1. See ABG Interpretation
    2. Venous Blood Gas (VBG) is often used instead, but cannot use pO2 based calculations (e.g. A-a Gradient)
    3. A-a Gradient
      1. Distinguishes intrinsic lung causes (e.g. V/Q mismatch) from external causes (e.g. Bellows Failure)
      2. Increased A-a Gradient suggests intrinsic lung cause, whereas A-a Gradient is normal in external causes
    4. Increased pCO2 Causes
      1. Bellows Failure with inadequate ventilation (normal lungs and gas exchange)
        1. Normal A-a Gradient
      2. Severely abnormal lungs with V/Q mismatch and unable to compensate with Minute Ventilation (e.g. COPD)
        1. Increased A-a Gradient

XII. Differential Diagnosis

  1. See Causes above
  2. See Dyspnea Causes
  3. See Tachypnea Causes
  4. See Hypoxia

XIII. Management: General

  1. See Emergency Breathing Management
  2. See Advanced Airway
  3. See Non-Invasive Positive Pressure Ventilation
  4. See Mechanical Ventilation
  5. Specific Approaches
    1. Emergency Management of Asthma Exacerbation (or Status Asthmaticus)
    2. Emergency Management of COPD Exacerbation
    3. Congestive Heart Failure Exacerbation Management
    4. Acute Respiratory Distress Syndrome
  6. Bellows Failure or apnea (increased PaCO2, normal A-a Gradient)
    1. Aggressive management is required (e.g. consider Endotracheal Intubation and Mechanical Ventilation)
    2. Manage immediately reversible causes (e.g. coma cocktail with Naloxone, dextrose)
    3. Consider upper airway obstruction (e.g. Anaphylaxis, Foreign Body Aspiration)
    4. Evaluate Trauma patients for chest wall defects interfering with bellows function (e.g. Flail Chest)
    5. In progressive neuromuscular disorders (e.g. Guillain Barre Syndrome) evaluate for impending Respiratory Failure
      1. Vital Capacity <15-20 ml/kg
      2. Tidal Volume <5 ml/kg
      3. Maximum expiratory force <40 cm H2O (normally >100 cm H2O)
      4. Maximum inspiratory force <30 cm H2O (normally >100 cm H2O)
  7. Hypercarbic Respiratory Failure from Diffuse Severe Lung Disease (large Respiratory Dead Space)
    1. COPD
      1. See Emergency Management of COPD Exacerbation
      2. Conservative management with controlled Oxygen Delivery (avoiding CO2 narcosis)
      3. Bronchodilators and Corticosteroids
      4. Non-Invasive Positive Pressure Ventilation (NIPPV) as needed
      5. Antibiotics for productive or purulent cough and increased Dyspnea or requiring NIPPV or Intubation
      6. Comorbid Right Heart Failure may Compound Presentation
      7. Avoid Endotracheal Intubation and Mechanical Ventilation if possible
        1. Ventilator Weaning may be more difficult
        2. See Mechanical Ventilation for settings
        3. Requires larger Tidal Volumes (e.g. 10 ml/kg) due to large Physiologic Dead Space
        4. Avoid excessive correction of PaCO2
          1. Correct pH, but avoid Respiratory Alkalosis
          2. Allows for pre-existing metabolically compensated hypercarbia
        5. Decrease air trapping by allowing greater time for expiration
          1. Shorten inspiratory time by increasing inspiratory rate
          2. Decrease Respiratory Rate
    2. Asthma
      1. See Emergency Management of Asthma Exacerbation (or Status Asthmaticus)
  8. Pulmonary Edema
    1. See Congestive Heart Failure Exacerbation Management

XIV. Management: Approach to Non-Invasive Positive Pressure Ventilation Selection

  1. Hypoxemic Respiratory Failure (Inadequate oxygenation)
    1. Reflected by Arterial Blood GasPaO2 and Oxygen Saturation
    2. Concepts
      1. Increase oxygen delivered to the lung (esp. FIO2) or
      2. Increase mean airway pressure (or Positive End-Expiratory Pressure)
    3. Interventions
      1. Continuous Positive Airways Pressure (CPAP)
  2. Hypercarbic Respiratory Failure (Inadequate ventilation)
    1. Reflected by Arterial Blood GasPaCO2 and pH
    2. Concepts (increase Minute Ventilation)
      1. Increase Tidal Volume (TV) or
      2. Increase Respiratory Rate (RR)
    3. Interventions
      1. Bilevel Positive Airway Pressure (BiPap)
  3. References
    1. Mallemat and Runde in Herbert (2015) EM:Rap 15(2): 7-8

XV. References

  1. (2016) Fundamental Critical Care Support, p. 46-60
  2. Davies (1986) Acute Respiratory Failure, Cyberlog
  3. Presberg in Noble (2001) Primary Care, p. 705-16

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Related Studies

Ontology: Acute respiratory failure (C0264490)

Definition (NCI) Life-threatening respiratory failure that develops rapidly. Causes include injury, sepsis, drug overdose, and pancreatitis. It manifests with dyspnea and cyanosis and may lead to cardiovascular shock.
Concepts Disease or Syndrome (T047)
ICD9 518.81
ICD10 J96.0
SnomedCT 65710008
English Acute respiratory failure, ARF - Acute respirat failure, ARF, acute respiratory failure (diagnosis), acute respiratory failure, Acute respiratry failure, acute failure respiratory, arf, ARF - Acute respiratory failure, Acute respiratory failure (disorder), Acute Respiratory Failure
Italian Insufficienza respiratoria acuta, Insufficienza renale acuta
Dutch ANF, Acute respiratoire insufficiëntie, acuut ademhalingstekort
French IRA, Défaillance respiratoire aiguë
German ARF, Akute respiratorische Insuffizienz, anderenorts nicht klassifiziert, akute respiratorische Insuffizienz
Portuguese Insuficiência renal aguda, Insuficiência respiratória aguda
Spanish FRA, fallo respiratorio agudo, falla respiratoria aguda (trastorno), falla respiratoria aguda, difucultad respiratoria aguda, dificultad respiratoria aguda (trastorno), dificultad respiratoria aguda, fallo respiratorio agudo (trastorno), Insuficiencia respiratoria aguda
Japanese 急性腎不全, 急性呼吸不全, キュウセイジンフゼン, キュウセイコキュウフゼン
Czech Akutní respirační selhání, Akutní renální selhání
Korean 급성 호흡기능상실
Hungarian Heveny légzési elégtelenség, ARF

Ontology: Respiratory Failure (C1145670)

Definition (MEDLINEPLUS)

Respiratory failure happens when not enough oxygen passes from your lungs into your blood. Your body's organs, such as your heart and brain, need oxygen-rich blood to work well. Respiratory failure also can happen if your lungs can't remove carbon dioxide (a waste gas) from your blood. Too much carbon dioxide in your blood can harm your body's organs.

Diseases and conditions that affect your breathing can cause respiratory failure. Examples include

  • Lung diseases such as COPD (chronic obstructive pulmonary disease), pneumonia, pulmonary embolism, and cystic fibrosis
  • Conditions that affect the nerves and muscles that control breathing, such as spinal cord injuries, muscular dystrophy and stroke
  • Damage to the tissues and ribs around the lungs. An injury to the chest can cause this damage.
  • Drug or alcohol overdose
  • Injuries from inhaling smoke or harmful fumes

Treatment for respiratory failure depends on whether the condition is acute (short-term) or chronic (ongoing) and how severe it is. It also depends on the underlying cause. You may receive oxygen therapy and other treatment to help you breathe.

NIH: National Heart, Lung, and Blood Institute

Definition (NCI) The significant impairment of gas exchange within the lungs resulting in hypoxia, hypercarbia, or both, to the extent that organ tissue perfusion is severely compromised. Causes include chronic obstructive pulmonary disease, asthma, emphysema, acute respiratory distress syndrome, pneumonia, pulmonary edema, pneumothorax, and congestive heart failure. Treatment requires intubation and mechanical ventilation until the time the lungs recover sufficient function.
Definition (NCI_CTCAE) A disorder characterized by impaired gas exchange by the respiratory system resulting in hypoxemia and a decrease in oxygenation of the tissues that may be associated with an increase in arterial levels of carbon dioxide.
Definition (CSP) respiratory function fails to maintain adequate oxygen supply and carbon dioxide removal.
Concepts Disease or Syndrome (T047)
MSH D012131
ICD10 J96.9
SnomedCT 207552005, 207555007, 51395007, 196165003, 158731006, 158734003, 409622000
English Respiratory Failure, Respiratory failure, unspecified, [D]Respiratory failure (context-dependent category), [D]Respiratory failure NOS (context-dependent category), [D]Respiratory failure, [D]Respiratory failure NOS, respiratory failure (diagnosis), respiratory failure, Respiration failure, Failure respiratory, Failure;respiratory, respiratory insufficiency, respiratory insufficiency/failure, [D]Respiratory failure NOS (situation), [D]Respiratory failure (situation), RESPIRATORY FAILURE, FAILURE, RESPIRATORY, Respiratory failure, NOS, Respiratory failure, Respiratory failure (disorder), Respiratory failure NOS
Italian Insufficienza respiratoria, Crisi respiratoria
German Atemversagen, Versagen Atmung, Respiratorische Insuffizienz, ATEMVERSAGEN, Respiratorische Insuffizienz, nicht naeher bezeichnet, respiratorische Insuffizienz
Spanish Fallo de la respiración, [D]falla respiratoria (categoría dependiente del contexto), Paro Respiratoria, [D]falla respiratoria (situación), [D]falla respiratoria, Insuficiencia respiratoria, falla respiratoria (trastorno), falla respiratoria
Japanese 呼吸不全, コキュウフゼン
Portuguese Falência Respiratória, FALENCIA RESPIRATORIA, Insuficiência respiratória
French Défaillance respiratoire, DEFAILLANCE RESPIRATOIRE
Czech Respirační selhání, Selhání respirační, respirační selhání
Korean 상세불명의 호흡기능상실
Hungarian Respiratiós insufficientia, Légzési elégtelenség, Légzési insufficientia
Norwegian Respirasjonssvikt
Dutch Respiratoire insufficiëntie, niet gespecificeerd, respiratoir falen, Ademhalingsinsufficiëntie