Management of Diaphragmatic Dysfunction/Phrenic Nerve Injury in CVICU
Non-invasive ventilation (NIV) should be the first-line ventilatory support for patients with diaphragmatic dysfunction or phrenic nerve injury in the CVICU, as it is well-tolerated and extubation from invasive mechanical ventilation may be difficult in these patients. 1
Diagnostic Assessment
- Diaphragmatic ultrasound is recommended as a bedside tool to assess diaphragmatic function, particularly in patients being weaned from mechanical ventilation 2
- Transdiaphragmatic pressure (Pdi) measurement requires simultaneous recordings of esophageal and gastric pressures to assess diaphragmatic contribution to breathing 2
- Phrenic nerve stimulation techniques (electrical or magnetic) provide objective assessment of diaphragm function independent of patient effort 3
- A negative gastric pressure to transdiaphragmatic pressure ratio (Pga/Pdi) indicates severe diaphragmatic dysfunction or paralysis 2
Acute Management Algorithm
Initial Respiratory Support
- Initiate NIV promptly in patients with diaphragmatic dysfunction showing signs of respiratory distress or hypercapnia 1
- Avoid excessive oxygen administration in isolation as it can worsen hypercapnia; target oxygen saturation of 88-92% in adults or above 92% for children 1
- Monitor CO2 levels closely with transcutaneous monitoring or arterial blood gases 1
- Consider controlled ventilation modes as patient triggering may be ineffective 1
Monitoring and Escalation Criteria
Maintain low threshold for enhanced monitoring and critical care input if any of the following are present 1:
- Hypoxemia (saturations <95%)
- Hypercapnia (>45 mmHg/6 kPa)
- Elevated respiratory rate
- Signs of respiratory fatigue
Warning signs requiring immediate intervention include 1:
- Difficulty achieving adequate oxygenation
- Rapid desaturation during breaks from NIV
- Presence of bulbar dysfunction with more profound hypoxemia
Ventilation Settings
- For patients with neuromuscular disease (NMD), use low levels of pressure support 1
- Apply PEEP in the range of 5-10 cmH2O to increase residual volume and reduce oxygen dependency 1
- Consider volume control (or volume assured) modes when high inflation pressures are required 1
Special Considerations
Invasive Mechanical Ventilation
- If NIV fails, do not delay intubation unless escalation to invasive mechanical ventilation is not desired by the patient or deemed inappropriate 1
- In the setting of single organ respiratory failure with diaphragmatic dysfunction, the prospects of recovery are good and invasive ventilation should be considered when NIV is unsuccessful 1
- Plan extubation carefully and perform in a specialist center with NIV and mechanical insufflator-exsufflator (MI-E) support available following extubation 1
Weaning Considerations
- Diaphragmatic dysfunction is a major cause of weaning failure 2
- Before initiating weaning, ensure the precipitant cause of respiratory failure is treated, pH is normalized, and chronic hypercapnia is corrected 1
- Physiotherapy treatment is recommended before and after extubation to reduce weaning duration and risk of extubation failure 1
- Consider prophylactic NIV after extubation for patients at high risk of reintubation 1
Advanced Treatment Options
- For persistent bilateral diaphragmatic dysfunction, consider referral for evaluation of diaphragm pacing 3, 4
- Diaphragm pacing generates breathing using the patient's own diaphragm as the respiratory pump and may be optimal for ventilatory support during wakefulness in carefully selected patients 3
- For unilateral diaphragmatic paralysis causing significant symptoms, surgical plication of the diaphragm may be considered 5, 6
- Phrenic nerve reconstruction combined with diaphragm pacing may offer greater functional recovery than pacing alone in bilateral diaphragmatic dysfunction 4
Long-term Management
- Following an episode of acute hypercapnic respiratory failure, nocturnal NIV should usually be continued pending discussion with a home ventilation service 1
- Advance care planning, particularly around the potential future use of invasive mechanical ventilation, is recommended in patients with progressive neuromuscular disease 1
- Ensure an individualized emergency healthcare plan is in place and discussed with the patient and family for future hospital admissions 1
Pitfalls and Caveats
- Patients with diaphragmatic dysfunction may not display typical signs of respiratory distress such as labored breathing and accessory muscle use 1
- Incorrect placement of the gastric balloon in the lower esophagus can mimic severe diaphragmatic dysfunction when measuring transdiaphragmatic pressure 2
- Recruitment of abdominal muscles during expiration followed by sudden relaxation at inspiration can also mimic diaphragmatic dysfunction 2
- Senior staff should be involved in decision-making, in conjunction with home mechanical ventilation specialists, especially when the appropriateness of invasive mechanical ventilation is questioned 1