What is the treatment for central hypoventilation?

Treatment of Central Hypoventilation

Central hypoventilation requires lifelong mechanical ventilatory support, as pharmacologic stimulants are ineffective and the condition does not resolve spontaneously. 1

Primary Treatment: Mechanical Ventilatory Support

The cornerstone of management is securing the airway and ensuring optimal ventilation and oxygenation through artificial ventilation. 1 The American Thoracic Society emphasizes that oxygen supplementation alone is inadequate and dangerous, as it improves cyanosis but allows persistent hypoventilation and leads to pulmonary hypertension. 1

Ventilation Modality Selection

For infants and young children with congenital central hypoventilation syndrome (CCHS), positive pressure ventilation via tracheostomy is recommended in the first several years of life to ensure optimal neurocognitive outcomes. 1 This approach provides the most reliable ventilatory support during the critical developmental period. 1

Available ventilation options include:

• Positive pressure ventilators via tracheostomy - preferred for infants and young children 1
• Bilevel positive airway pressure (BiPAP) - considered only after 6-8 years of age in stable patients requiring support only during sleep 1
• Diaphragm pacing - an option for select patients 1
• Negative pressure ventilators (chest shell, cuirass, wrap) - less commonly used 1

Noninvasive Positive Pressure Ventilation (NPPV) Settings

When NPPV is appropriate (older children, sleep-only support), the American Academy of Sleep Medicine provides specific titration parameters: 1

Initial settings:

• Minimum starting IPAP: 8 cm H₂O 1
• Minimum starting EPAP: 4 cm H₂O 1
• Maximum IPAP: 30 cm H₂O for patients ≥12 years; 20 cm H₂O for patients <12 years 1
• Minimum pressure support (PS): 4 cm H₂O; maximum 20 cm H₂O 1

Titration approach:

• Increase PS every 5 minutes if tidal volume is low (<6-8 mL/kg) 1
• Increase PS if arterial PCO₂ remains ≥10 mm Hg above goal for ≥10 minutes 1
• Goal PCO₂ should be ≤ awake PCO₂ 1

Backup rate (spontaneous-timed mode) is mandatory for central hypoventilation: 1

• Set backup rate equal to or slightly less than spontaneous sleeping respiratory rate (minimum 10 bpm) 1
• Increase in 1-2 bpm increments every 10 minutes if goals not met 1
• Set IPAP time to provide inspiratory time between 30-40% of cycle time 1

Monitoring Requirements

Continuous monitoring with pulse oximetry and end-tidal CO₂ (PETCO₂) is mandatory during all sleep periods and recommended for periodic checks during wakefulness. 1 Standard apnea/bradycardia monitors are contraindicated as they cannot detect hypoventilation and provide false reassurance. 1

Target parameters:

• SpO₂: ≥95% 1
• PETCO₂: 30-50 mm Hg (ideally 35-40 mm Hg) 1
• Alarm settings: SpO₂ ≤85%, PETCO₂ ≥55 mm Hg 1

Continuous care by a registered nurse trained in ventilator management is required at home to prevent hypoxia and its neurocognitive sequelae, as patients with central hypoventilation lack typical signs of respiratory distress (retractions, increased work of breathing, dyspnea sensation). 1

Ventilator Philosophy: No Weaning

Weaning from the ventilator is not a realistic goal and should not be attempted. 1 Patients cannot be "trained" to breathe adequately. 1 Ventilator settings should completely meet ventilatory demands to optimize quality of life and preserve energy for academic and physical activities. 1 Some centers use "ventilator ladders" in conjunction with continuous monitoring to maintain precise gas exchange control. 1

Comprehensive Physiologic Assessment

Biannual (initially) then annual in-hospital comprehensive physiologic testing is required to assess ventilatory needs during varying activity levels and sleep stages. 1 These multi-day evaluations at specialized CCHS centers should include: 1

• Respiratory inductance plethysmography (chest, abdomen, sum)
• Continuous ECG and hemoglobin saturation monitoring
• End-tidal CO₂ monitoring
• Sleep state staging
• Blood pressure and temperature monitoring
• Endogenous and exogenous gas challenges
• Autonomic testing

Critical Safety Measures

Avoid all sedative medications and CNS depressants, as they worsen hypoventilation. 1 Metabolic imbalances, particularly chronic metabolic alkalosis, further inhibit central respiratory drive and must be corrected. 1

Swimming requires careful supervision regardless of tracheostomy presence, and underwater swimming contests are absolutely prohibited as patients lack asphyxia perception and will not surface appropriately. 1

Emergency preparedness is mandatory:

• Power generator for home use 1
• Placement on emergency list for local power company and fire department 1

Supplemental Oxygen Use

Supplemental oxygen may be added only when:

• Awake SpO₂ <88%, OR 1
• PS and respiratory rate optimized but SpO₂ remains <90% for ≥5 minutes 1

Start at 1 L/minute and increase in 1 L/minute increments every 5 minutes until SpO₂ >90%. 1 Never use oxygen as monotherapy - it masks hypoventilation while allowing dangerous CO₂ retention. 1

Follow-Up Care

Close follow-up by appropriately trained healthcare providers is essential after NPPV initiation to establish effective utilization patterns, remediate side effects, and assess ventilation/oxygenation adequacy. 1

Common Pitfalls

• Never rely on clinical appearance alone - patients lack typical respiratory distress signs despite severe hypoxemia/hypercarbia 1
• Do not use apnea/bradycardia monitors - they cannot detect hypoventilation and miss characteristic sinus pauses 1
• Avoid attempting ventilator weaning - this is not a goal in central hypoventilation management 1
• Never use oxygen without mechanical ventilation - this leads to pulmonary hypertension and does not address hypoventilation 1