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 neuromuscular or chest wall disease" 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.)
- McDermott CJ, Maguire C, Cooper CL, et al. Protocol for diaphragm pacing in patients with respiratory muscle weakness due to motor neurone disease (DiPALS): a randomised controlled trial. BMC Neurol 2012; 12:74.
- Elefteriades JA, Hogan JF, Handler A, Loke JS. Long-term follow-up of bilateral pacing of the diaphragm in quadriplegia. N Engl J Med 1992; 326:1433.
- Glenn WW, Hogan JF, Loke JS, et al. Ventilatory support by pacing of the conditioned diaphragm in quadriplegia. N Engl J Med 1984; 310:1150.
- Elefteriades JA, Quin JA. Diaphragm pacing. Ann Thorac Surg 2002; 73:691.
- Le Pimpec-Barthes F, Gonzalez-Bermejo J, Hubsch JP, et al. Intrathoracic phrenic pacing: a 10-year experience in France. J Thorac Cardiovasc Surg 2011; 142:378.
- Elefteriades JA, Quin JA, Hogan JF, et al. Long-term follow-up of pacing of the conditioned diaphragm in quadriplegia. Pacing Clin Electrophysiol 2002; 25:897.
- Garrido-Garcia H, Mazaira Alvarez J, Martín Escribano P, et al. Treatment of chronic ventilatory failure using a diaphragmatic pacemaker. Spinal Cord 1998; 36:310.
- Morgan JA, Morales DL, John R, et al. Endoscopic, robotically assisted implantation of phrenic pacemakers. J Thorac Cardiovasc Surg 2003; 126:582.
- Khong P, Lazzaro A, Mobbs R. Phrenic nerve stimulation: the Australian experience. J Clin Neurosci 2010; 17:205.
- Romero FJ, Gambarrutta C, Garcia-Forcada A, et al. Long-term evaluation of phrenic nerve pacing for respiratory failure due to high cervical spinal cord injury. Spinal Cord 2012; 50:895.
- Onders RP, Elmo M, Khansarinia S, et al. Complete worldwide operative experience in laparoscopic diaphragm pacing: results and differences in spinal cord injured patients and amyotrophic lateral sclerosis patients. Surg Endosc 2009; 23:1433.
- Posluszny JA Jr, Onders R, Kerwin AJ, et al. Multicenter review of diaphragm pacing in spinal cord injury: successful not only in weaning from ventilators but also in bridging to independent respiration. J Trauma Acute Care Surg 2014; 76:303.
- Onders RP, Ponsky TA, Elmo M, et al. First reported experience with intramuscular diaphragm pacing in replacing positive pressure mechanical ventilators in children. J Pediatr Surg 2011; 46:72.
- Onders RP, Khansarinia S, Weiser T, et al. Multicenter analysis of diaphragm pacing in tetraplegics with cardiac pacemakers: positive implications for ventilator weaning in intensive care units. Surgery 2010; 148:893.
- Onders RP, Elmo MJ, Ignagni AR. Diaphragm pacing stimulation system for tetraplegia in individuals injured during childhood or adolescence. J Spinal Cord Med 2007; 30 Suppl 1:S25.
- DiMarco AF, Takaoka Y, Kowalski KE. Combined intercostal and diaphragm pacing to provide artificial ventilation in patients with tetraplegia. Arch Phys Med Rehabil 2005; 86:1200.
- Ali A, Flageole H. Diaphragmatic pacing for the treatment of congenital central alveolar hypoventilation syndrome. J Pediatr Surg 2008; 43:792.
- Lassman AB, Mayer SA. Paroxysmal apnea and vasomotor instability following medullary infarction. Arch Neurol 2005; 62:1286.
- Yasuma F, Sakamoto M, Okada T, Abe K. Eight-year follow-up study of a patient with central alveolar hypoventilation treated with diaphragm pacing. Respiration 1998; 65:313.
- Brouillette RT, Ilbawi MN, Hunt CE. Phrenic nerve pacing in infants and children: a review of experience and report on the usefulness of phrenic nerve stimulation studies. J Pediatr 1983; 102:32.
- Sardenberg RA, Secaf LB, Pinotti AC, et al. Diaphragmatic pacing: unusual indication with successful application. J Bras Pneumol 2011; 37:697.
- Diep B, Wang A, Kun S, et al. Diaphragm Pacing without Tracheostomy in Congenital Central Hypoventilation Syndrome Patients. Respiration 2015; 89:534.
- Smith BK, Fuller DD, Martin AD, et al. Diaphragm Pacing as a Rehabilitative Tool for Patients With Pompe Disease Who Are Ventilator-Dependent: Case Series. Phys Ther 2016; 96:696.
- Onders RP, Elmo M, Kaplan C, et al. Extended use of diaphragm pacing in patients with unilateral or bilateral diaphragm dysfunction: a new therapeutic option. Surgery 2014; 156:776.
- Taslimuddin M, Islam Q, Islam S. Breathing pacemakers in poliomyelitis - A case report. Indian J Physical Med and Rehab 2003; 14:1.
- Onders RP, Carlin AM, Elmo M, et al. Amyotrophic lateral sclerosis: the Midwestern surgical experience with the diaphragm pacing stimulation system shows that general anesthesia can be safely performed. Am J Surg 2009; 197:386.
- DiPALS Writing Committee, DiPALS Study Group Collaborators, McDermott CJ, et al. Safety and efficacy of diaphragm pacing in patients with respiratory insufficiency due to amyotrophic lateral sclerosis (DiPALS): a multicentre, open-label, randomised controlled trial. Lancet Neurol 2015; 14:883.
- Gonzalez-Bermejo J, Morélot-Panzini C, Tanguy ML, et al. Early diaphragm pacing in patients with amyotrophic lateral sclerosis (RespiStimALS): a randomised controlled triple-blind trial. Lancet Neurol 2016; 15:1217.
- Onders RP, Elmo M, Kaplan C, et al. Final analysis of the pilot trial of diaphragm pacing in amyotrophic lateral sclerosis with long-term follow-up: diaphragm pacing positively affects diaphragm respiration. Am J Surg 2014; 207:393.
- Sanli A, Sengun IS, Tertemiz KC, et al. Importance of diaphragm thickness in amyotrophic lateral sclerosis patients with diaphragm pacing system implantation. Surg Endosc 2016; 30:154.
- Shaw RK, Glenn WW, Hogan JF, Phelps ML. Electrophysiological evaluation of phrenic nerve function in candidates for diaphragm pacing. J Neurosurg 1980; 53:345.
- Alshekhlee A, Onders RP, Syed TU, et al. Phrenic nerve conduction studies in spinal cord injury: applications for diaphragmatic pacing. Muscle Nerve 2008; 38:1546.
- McCauley RG, Labib KB. Diaphragmatic paralysis evaluated by phrenic nerve stimulation during fluoroscopy or real-time ultrasound. Radiology 1984; 153:33.
- DiMarcoAF. Diaphragm pacing in patients with spinal cord injury. Top Spinal Cord Inj Rehabil 1999; 5:6.
- Boon AJ, Sekiguchi H, Harper CJ, et al. Sensitivity and specificity of diagnostic ultrasound in the diagnosis of phrenic neuropathy. Neurology 2014; 83:1264.
- Layachi L, Georges M, Gonzalez-Bermejo J, et al. Diaphragm pacing failure secondary to deteriorated chest wall mechanics: When a good diaphragm does not suffice to take a good breath in. Respir Med Case Rep 2015; 15:20.
- Shaul DB, Danielson PD, McComb JG, Keens TG. Thoracoscopic placement of phrenic nerve electrodes for diaphragmatic pacing in children. J Pediatr Surg 2002; 37:974.
- DiMarco AF. Phrenic nerve stimulation in patients with spinal cord injury. Respir Physiol Neurobiol 2009; 169:200.
- Carter RE, Donovan WH, Halstead L, Wilkerson MA. Comparative study of electrophrenic nerve stimulation and mechanical ventilatory support in traumatic spinal cord injury. Paraplegia 1987; 25:86.
- Lieberman JS, Corkill G, Nayak NN, et al. Serial phrenic nerve conduction studies in candidates for diaphragm pacing. Arch Phys Med Rehabil 1980; 61:528.
- Versteegh MI, Braun J, Voigt PG, et al. Diaphragm plication in adult patients with diaphragm paralysis leads to long-term improvement of pulmonary function and level of dyspnea. Eur J Cardiothorac Surg 2007; 32:449.
- Bach JR, O'Connor K. Electrophrenic ventilation: a different perspective. J Am Paraplegia Soc 1991; 14:9.
- Adler D, Gonzalez-Bermejo J, Duguet A, et al. Diaphragm pacing restores olfaction in tetraplegia. Eur Respir J 2009; 34:365.
- Gonzalez-Bermejo J, Morélot-Panzini C, Salachas F, et al. Diaphragm pacing improves sleep in patients with amyotrophic lateral sclerosis. Amyotroph Lateral Scler 2012; 13:44.
- Breathing pacemakers. http://www.averylabs.com (Accessed on March 20, 2012).
- Morgan JA, Ginsburg ME, Sonett JR, et al. Advanced thoracoscopic procedures are facilitated by computer-aided robotic technology. Eur J Cardiothorac Surg 2003; 23:883.
- Story D, Mariampillai E, Nikfarjam M, et al. Anaesthetic aspects of implanting diaphragmatic pacing in patients with spinal cord injury. Anaesth Intensive Care 2010; 38:740.
- Tedde ML, Vasconcelos Filho P, Hajjar LA, et al. Diaphragmatic pacing stimulation in spinal cord injury: anesthetic and perioperative management. Clinics (Sao Paulo) 2012; 67:1265.
- Niazi AU, Mocon A, Varadi RG, et al. Ondine's curse: anesthesia for laparoscopic implantation of a diaphragm pacing stimulation system. Can J Anaesth 2011; 58:1034.
- Devine A, Watt JW. Anaesthesia and diaphragmatic pacing in patients with tetraplegia. A review of peri-operative management in patients over a 10-year period. Eur J Anaesthesiol 1996; 13:553.
- Chervin RD, Guilleminault C. Diaphragm pacing for respiratory insufficiency. J Clin Neurophysiol 1997; 14:369.
- Onders RP. Functional electrical stimuation: restoration of respiratory function. In: Handbook of clinical neurology: Spinal cord injury, 3rd Ed, Verhaagen J, Mc Donald J. (Eds), Elsevier BV, 2012. Vol 109, p.275.
- Rezania K, Gottlieb O, Guralnick A, et al. Venous thromboembolism after diaphragm pacing in amyotrophic lateral sclerosis. Muscle Nerve 2014; 50:863.
- 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