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Medical Policy Diaphragmatic/Phrenic Nerve Stimulation and Diaphragm Pacing Systems Table of Contents • Policy: Commercial • Coding Information
• Information Pertaining to All Policies
• Policy: Medicare • Description
• References
• Authorization Information • Policy History
• Endnotes Policy Number: 593

BCBSA Reference Number: N/A Related Policies

Phrenic Nerve Stimulation for Central Sleep Apnea, #955

Policy1 Commercial Members: Managed Care (HMO and POS), PPO, and Indemnity

Diaphragmatic/Phrenic Stimulation Diaphragmatic/phrenic nerve stimulation with an FDA-approved device is considered MEDICALLY NECESSARY as an alternative to invasive mechanical ventilation for individuals who are 18 years of age or older when ALL of the following criteria are met:

a. The individual has ventilatory failure from stable, high spinal cord injury or ventilatory failure from central alveolar hypoventilation syndrome; and b. The individual cannot breathe spontaneously for 4 continuous hours or more without use of a mechanical ventilator; and c. Diaphragm movement with stimulation is visible under fluoroscopy; and d. Stimulation of the diaphragm either directly or through the phrenic nerve results in sufficient muscle activity to accommodate independent breathing without the support of a ventilator for at least 4 continuous hours a day; and e. Individual has normal chest anatomy, a normal level of consciousness, and has the ability to participate in and complete the training and rehabilitation associated with the use of the device; and f. Bilateral clinically acceptable phrenic nerve function is demonstrated with electromyography recordings and nerve conduction times.

Diaphragmatic Stimulation Diaphragm stimulation with an FDA approved diaphragm pacing system is considered MEDICALLY NECESSARY as an alternative to invasive mechanical ventilation in individuals who are 18 years of age or older when ALL of the following criteria are met:

2 a. The individual has ventilatory failure from stable, high spinal cord injury or ventilatory failure from central alveolar hypoventilation syndrome or ventilatory failure from motor neuron disease, for example amyotrophic lateral sclerosis; and b. The individual cannot breathe spontaneously for 4 continuous hours or more without use of a mechanical ventilator; and c. Diaphragm movement with stimulation is visible under fluoroscopy; and d. Stimulation of the diaphragm directly results in sufficient muscle activity to accommodate independent breathing without the support of a ventilator for at least 4 continuous hours a day; and e. Individual has normal chest anatomy, a normal level of consciousness, and has the ability to participate in and complete the training and rehabilitation associated with the use of the device.

Diaphragmatic/phrenic nerve stimulation devices and Diaphragm Pacing Systems are considered NOT MEDICALLY NECESSARY when: • The individual can breathe spontaneously for 4 continuous hours or more without use of a mechanical ventilator; or • The respiratory insufficiency is temporary.

Diaphragmatic/phrenic nerve stimulation and Diaphragm Pacing Systems are considered INVESTIGATIONAL and NOT MEDICALLY NECESSARY for all other indications including, but not limited to: • Underlying cardiac, pulmonary or chest wall disease is present which is significant enough to prevent spontaneous breathing off a ventilator for more than 4 hours even with the use of the phrenic nerve or diaphragm pacemaker device; or • In individuals with intact phrenic nerve and diaphragm function; or • For treatment of any other condition where the phrenic nerve and diaphragm are intact (for example, chronic obstructive lung disease, restrictive lung disease, singultus [hiccups]); or • For adolescents, children and infants; or • When the above criteria are not met.

Prior Authorization Information
Inpatient • For services described in this policy, precertification/preauthorization IS REQUIRED for all products if the procedure is performed inpatient.
Outpatient • For services described in this policy, see below for products where prior authorization might be required if the procedure is performed outpatient.

Outpatient Commercial Managed Care (HMO and POS) Prior authorization is not required. Commercial PPO and Indemnity Prior authorization is not required.

CPT Codes / HCPCS Codes / ICD Codes
Inclusion or exclusion of a code does not constitute or imply member coverage or provider reimbursement. Please refer to the member’s contract benefits in effect at the time of service to determine coverage or non-coverage as it applies to an individual member.

Providers should report all services using the most up-to-date industry-standard procedure, revenue, and diagnosis codes, including modifiers where applicable.

CPT Codes There is no specific CPT code for this service.

Description The NeuRx DPS RA/4 Respiratory Stimulation System is implanted through minimally invasive laparoscopic surgery and provides electrical stimulation to muscles and nerves that run through the

3 diaphragm. This eliminates any direct contact with the phrenic nerve, allows all circuitry and electronics to remain outside the body, and provides direct, selective activation to each hemidiaphragm. According to manufacturer information, when stimulated by the NeuRx DPS, the diaphragm contracts, mimicking natural breathing and allowing air to fill the upper and lower parts of the lungs, rather than forcing air in with a mechanical ventilator. The device uses four electrodes implanted in the muscle of the diaphragm to electronically stimulate contraction; this stimulation allows the user to inhale. The DPS is lightweight and battery powered, eliminating the need for an external power source. Similar to the NeuRx DPS system, the Mark IV system is connected to the phrenic nerve by electrodes in the neck or chest area. The device consists of a surgically implanted receiver and electrodes which are connected to an external transmitter for transmitting the stimulating pulses across the skin to the implanted receiver.

Summary Based on humanitarian device exemptions, diaphragmatic/phrenic nerve stimulation is considered medically necessary for patients with high spinal cord injuries to allow freedom from mechanical ventilation for at least 4 hours daily and is indicated for patients with ALS to delay the need for mechanical ventilation. Patients must meet the medical necessity criteria in the Policy section of this document.

Policy History Date Action 6/2022 Annual policy review. Policy updated with literature review through June 2022.
References added.
1/2021 Medicare information removed. See MP #132 Medicare Advantage Management for local coverage determination and national coverage determination reference.
5/2020 Annual policy review. Policy updated with literature review through April 2020, references added. Policy statements unchanged. 7/2019 Phrenic Nerve Stimulation for the treatment of central sleep apnea was transferred to policy #955.
11/2018 Annual policy review. Investigational policy statements clarified. Policy statements unchanged.
1/2018 Medically necessary criteria revised. Effective 1/1/2018. 9/2017 Annual policy review. Policy statements unchanged.
8/2015 Coding information clarified. 9/2013 New policy describing medically necessary and investigational indications. Effective 9/1/2013.

Information Pertaining to All Blue Cross Blue Shield Medical Policies Click on any of the following terms to access the relevant information: Medical Policy Terms of Use Managed Care Guidelines Indemnity/PPO Guidelines Clinical Exception Process Medical Technology Assessment Guidelines

References

1. Peter J Wijkstra, Hans van der Aa, Sijbrand HofkerDiaphragm et al. Pacing in Patients with Spinal Cord Injury: A European Experience. Respiration. 2022;101(1):18-24.
2. Onders RP, Khansarinia S, Ingvarsson PE et al. Diaphragm pacing in spinal cord injury can significantly decrease mechanical ventilation in multicenter prospective evaluation. Artif Organs. 2022 Feb 28.
3. Abraham WT, Jagielski D, Oldenburg O, et al. Phrenic nerve stimulation for the treatment of central sleep apnea. JACC Heart Fail. 2015; 3(5):360-369.
4. Ali A, Flageole H. Diaphragmatic pacing for the treatment of congenital central alveolar hypoventilation syndrome. J Pediatr Surg. 2008; 43(5):792-796.

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5. Alshekhlee A, Onders RP, Syed TU, et al. Phrenic nerve conduction studies in spinal cord injury: applications for diaphragmatic pacing. Muscle Nerve. 2008; 38(6):1546-1552.
6. Costanzo MR, Ponilowski P, Coats A, et al. Phrenic nerve stimulation to treat patients with central sleep apnoea and heart failure. Eur J Heart Fail. 2018b; 20(12):1746-1754.
7. Costanzo MR, Ponikowski P, Javaheri S, et al. Transvenous neurostimulation for central sleep apnoea: a randomised controlled trial. Lancet. 2016; 388(10048):974-982.
8. Contanzo MR, Ponikowski P, Javaheri S, et al. Sustained 12 month benefit of phrenic nerve stimulation for central sleep apnea. Am J Cardiol. 2018a; 121(11):1400-1408.
9. DiMarco AF, Onders RP, Ignagni A, et al. Phrenic nerve pacing via intramuscular diaphragm electrodes in tetraplegic subjects. Chest. 2005; 127(2):671-678.
10. DiPALS Writing Committee. 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. 2015; 14(9):883-892.
11. 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(6):897-906.
12. Garrido-Garcia H, Mazaira Alvarez J, Martin Escribano P, et al. Treatment of chronic ventilatory failure using a diaphragmatic pacemaker. Spinal Cord. 1998; 36(5):310-314.
13. 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(1):44-54.
14. 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(12):1217-1227.
15. Hirschfeld S, Exner G, Luukkaala T, Baer GA. Mechanical ventilation or phrenic nerve stimulation for treatment of spinal cord injury-induced respiratory insufficiency. Spinal Cord. 2008; 46(11):738-742.
16. Jagielski D, Ponikowski P, Augostini R, et al. Transvenous stimulation of the phrenic nerve for the treatment of central sleep apnoea: 12 months' experience with the Remedē® System. Eur J Heart Fail. 2016; 18(11):1386-1393.
17. Krieger LM, Krieger AJ. The intercostal to phrenic nerve transfer: an effective means of reanimating the diaphragm in patients with high cervical spine injury. Plastic and Recon Surg. 2000; 105(4):1255-
19. Mahajan KR, Bach JR, Saporito L, Perez N. Diaphragm pacing and noninvasive respiratory management of amyotrophic lateral sclerosis/motor neuron disease. Muscle Nerve. 2012; 46(6):851-
21. McDermott CJ, Bradburn MJ, Maguire C, et al. DiPALS: Diaphragm pacing in patients with amyotrophic lateral sclerosis - a randomised controlled trial. health technol assess. 2016; 20(45):1-
23. 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-S29.
24. Onders RP, Elmo M, Khansarinia S, et al. Complete worldwide operative experience in laparoscopic diaphragm pacing: results and difference in spinal cord injured patients and amyotrophic lateral sclerosis patients. Surg Endosc. 2009; 23(7):1433-1440.
25. 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(4):893-897; discussion 897-898.
26. 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(1):72-76.
27. 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(2):303-309.
28. 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(12):895-898.
29. Shaul DB, Danielson PD, McComb JG, Keens TG. Thorascopic placement of phrenic nerve electrodes for diaphragmatic pacing in children. J Pediatr Surg. 2002; 37(7):974-978.
30. American Thoracic Society (ATS). ATS clinical policy statement: congenital central hypoventilation syndrome: genetic basis, diagnosis and management. October 2009. Available at: http://www.thoracic.org/statements/resources/pldd/congenital-central-hypoventilation-syndrome.pdf. Accessed on September 4, 2019.

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28. Centers for Medicare and Medicaid Services. National Coverage Determination for Phrenic Nerve Stimulators. NCD 160.19. Longstanding coverage determination; Effective date not posted. Available at: https://www.cms.gov/medicare-coverage-database/details/ncd- details.aspx?NCDId=244&ncdver=1&bc=AAAAQAAAAAAA&. Accessed on September 4, 2019.
29. Miller RG, Jackson CE, Kasarskis EJ, et al. Practice parameter update: The care of the patient with amyotrophic lateral sclerosis: drug, nutritional, and respiratory therapies (an evidence-based review). Report of the Quality Standards Subcommittee of the American Academy of Neurology (AAN). Neurology. 2009; 73(15):1218-1226.
30. U.S. Food and Drug Administration (FDA). Part 882 Neurological devices. Sec. 882.5830. Implanted diaphragmatic/phrenic nerve stimulator. April 8, 1986. Revised April 1, 2018. Available at: http://www.accessdata.fda.gov/scripts/cdrh/cfdocs/cfCFR/CFRSearch.cfm?FR=882.5830. Accessed on September 4, 2019.
31. U.S. Food and Drug Administration. Humanitarian Device Exemption Database. NeuRx DPS RA/4 Respiratory Stimulation System (Synapse Biomedical, Inc., Oberlin, OH). Summary of Safety and Probable Benefit. No. H070003. Rockville, MD: FDA. June 17, 2008. Available at: https://www.accessdata.fda.gov/scripts/cdrh/cfdocs/cfhde/hde.cfm?id=375540. Accessed on September 4, 2019.
32. U.S. Food and Drug Administration (FDA). Humanitarian Device Exemption Database. NeuRx DPS Diaphragm Pacing System (Synapse Biomedical, Inc., Oberlin, OH). Summary of Safety and Probable Benefit. No. H100006. Rockville, MD: FDA. September 28, 2011. Available at: https://www.accessdata.fda.gov/scripts/cdrh/cfdocs/cfhde/hde.cfm?id=375558. Accessed on September 4, 2019.
33. U.S. Food and Drug Administration (FDA). Center for Devices and Radiological Health (CDRH) Premarket Approvals for the Avery Breathing Pacemaker System Mark IV (Avery Biomedical Device, Inc., Commack, NY). Summary of Safety and Effectiveness. No. P860026. Rockville, MD: FDA. February 25, 1987; updated 2003. Available at: https://www.accessdata.fda.gov/scripts/cdrh/cfdocs/cfpma/pma.cfm?id=P860026S006. Accessed on September 4, 2019.

34. U.S. Food and Drug Administration (FDA). Center for Devices and Radiological Health (CDRH). Premarket Notification Database. Remedē System (Respicardia, Inc., Minnetonka, MN). Summary of Safety and Effectiveness. No. P160039. Rockville, MD: FDA. October 6, 2017. Available at: https://www.accessdata.fda.gov/cdrh_docs/pdf16/P160039b.pdf. Accessed on September 4, 2019.

    Endnotes

    1 Based on expert opinion