Pacing the diaphragm: Patient selection, evaluation, implantation, and complications
- Donald W Marion, MD
Donald W Marion, MD
- Senior Consultant
- Defense and Veterans Brain Injury Center
- Section Editor
- Jeremy M Shefner, MD, PhD
Jeremy M Shefner, MD, PhD
- Section Editor — Neuromuscular Disease
- Professor and Chair of Neurology, Barrow Neurological Institute
- Professor of Neurology, University of Arizona, Phoenix
- Clinical Professor of Neurology, Creighton University
Diaphragmatic pacing can be used in patients with ventilatory failure due to severe weakness or paralysis of the diaphragm as a means to eliminate or reduce the need for ventilatory support. Intact phrenic nerve function is required for effective pacing. The typical approach has been to pace the diaphragm via stimulation of the phrenic nerve at the level of the neck or thorax. However, pacing of the phrenic nerve at the level of the diaphragm may also be used in some patients.
Patient selection, evaluation for pacemaker candidacy, implantation technique, postoperative recovery, and complications of pacemaker placement will be reviewed here. The etiology, diagnosis, and management of patients with bilateral and unilateral diaphragmatic paralysis are discussed separately. (See "Causes and diagnosis of bilateral diaphragmatic paralysis" and "Causes and diagnosis of unilateral diaphragmatic paralysis and eventration in adults" and "Treatment of diaphragmatic paralysis" and "Diaphragmatic paralysis in the newborn".)
SELECTION OF POTENTIAL CANDIDATES
Diaphragmatic pacing is typically a second line therapy for patients with ventilatory failure due to bilateral paralysis or severe paresis of the diaphragm. This population of patients has traditionally been ventilated invasively with a mechanical ventilator, or noninvasively with positive pressure support. However, diaphragmatic pacing can be used in a select group of patients who cannot tolerate, have a desire to be liberated from, or have a desire to delay the need for noninvasive or invasive ventilatory support, the details of which are discussed in the sections below. (See 'Upper cervical spinal cord injury (above C3)' below and 'Amyotrophic lateral sclerosis' below and 'Other patient populations' below.)
Importantly, paralysis/paresis of the diaphragm can be due to muscle, nerve, or central nervous system disease, and only patients with intact phrenic nerve function are amenable to pacing. Notably, diaphragmatic pacing should be avoided in patients with evidence of a denervated diaphragm. (See 'Upper cervical spinal cord injury (above C3)' below and 'Amyotrophic lateral sclerosis' below and 'Other patient populations' below.)
It should be recognized by physicians and patients that only small case series have demonstrated benefits from diaphragmatic pacing including a reduction or a delay in the need for ventilatory support. Randomized trials are needed to validate these and other clinically important outcomes, including the effect of pacing on lung function and overall survival, compared with standard forms of ventilatory support (invasive or noninvasive ventilation) . (See "Continuous noninvasive ventilatory support for patients with respiratory muscle dysfunction" and "Practical aspects of nocturnal noninvasive ventilation in neuromuscular and chest wall disease" and "Types of noninvasive nocturnal ventilatory support in neuromuscular and chest wall disease" and "Overview of tracheostomy" and "Management and prognosis of patients requiring prolonged mechanical ventilation" and 'Making the decision to pace' below.)
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- SELECTION OF POTENTIAL CANDIDATES
- Upper cervical spinal cord injury (above C3)
- Other patient populations
- PATIENTS WITH UNCLEAR BENEFIT
- Amyotrophic lateral sclerosis
- Requirements for successful pacing
- Apnea or severe hypoventilation due to diaphragmatic dysfunction
- Intact phrenic nerve function
- - Percutaneous nerve conduction studies
- - Intraoperative nerve stimulation
- General medical issues
- Timing of evaluation
- MAKING THE DECISION TO PACE
- SURGICAL TECHNIQUE
- Selection of approach/pacemaker type
- - Patients with upper level spinal cord injury
- - Other patient populations
- - Cervical approach
- - Thoracic approach
- - Diaphragmatic approach
- Intraoperative anesthesia
- Immediate postoperative period
- CONDITIONING OF THE DIAPHRAGM
- Initial pacemaker settings
- Conditioning regimen
- General complications of pacemaker surgery
- Complications of cervical pacemakers
- Complications of thoracic pacemakers
- Complications of direct diaphragmatic pacemakers
- SUMMARY AND RECOMMENDATIONS