Table 1.

Etiology of hypoxia in patients with SCD

Mechanism of hypoxiaCauses in SCD
Hypoventilation SDB (OSA, upper airway obstruction)
Thoracic splinting due to chronic pain
Restrictive pulmonary disease
Reduced chest excursion due to hepatomegaly
Central hypoventilation (eg, due to excessive use of narcotics) 
Diffusion impairment SCD-associated interstitial lung fibrosis
PH
Pulmonary vascular disease 
Shunt Intracardiac shunt (eg, ventriculoseptal defect)
Extracardiac shunt
Arterial-venous malformations
Intrapulmonary shunt (eg, due to consolidation or atelectasis resulting in decreased perfusion to affected area) 
Ventilation-perfusion inequality Chronic VTE
ACS
Plastic bronchitis
Obstructive lung disease without asthma
Chronic airway inflammation due to asthma 
Mechanism of hypoxiaCauses in SCD
Hypoventilation SDB (OSA, upper airway obstruction)
Thoracic splinting due to chronic pain
Restrictive pulmonary disease
Reduced chest excursion due to hepatomegaly
Central hypoventilation (eg, due to excessive use of narcotics) 
Diffusion impairment SCD-associated interstitial lung fibrosis
PH
Pulmonary vascular disease 
Shunt Intracardiac shunt (eg, ventriculoseptal defect)
Extracardiac shunt
Arterial-venous malformations
Intrapulmonary shunt (eg, due to consolidation or atelectasis resulting in decreased perfusion to affected area) 
Ventilation-perfusion inequality Chronic VTE
ACS
Plastic bronchitis
Obstructive lung disease without asthma
Chronic airway inflammation due to asthma 

OSA, obstructive sleep apnea; SDB, sleep-disordered breathing.

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