Description:
The electrophrenic pacemaker is an implanted electrode and receiver with a pocket or table-top size external transmitter. The device electrically stimulates the phrenic nerves to contract the diaphragm rhythmically, which causes breathing.

Policy:
The use of an electrophrenic pacemaker is considered MEDICALLY NECESSARY for the following patients with permanent, severe hypoventilation caused by brain or high cervical cord lesions:

• Quadriplegia (high C3 or above)
• Central alveolar hypoventilation syndrome

Electrophrenic pacemakers are considered NOT MEDICALLY NECESSARY when:

• Patient can subsist independently of a mechanical respirator.
• Respiratory insufficiency is temporary.

The use of the electrophrenic pacemaker is considered INVESTIGATIONAL for the following conditions:

• Chronic obstructive pulmonary disease
• Young children and infants
• Treatment of hiccups

Policy Guidelines:
Electrophrenic pacemakers are contraindicated in the following situations:

• Preoperative screening tests do not demonstrate that phrenic nerves, lungs and diaphragm can sustain ventilation by electrical stimulation.
• Patient has another serious disorder that might affect nerve conduction (e.g., tumors, vascular disease, diabetes, multiple sclerosis, etc.).

Rationale:  
Since 1972, phrenic nerve stimulation has been an option for assisting the ventilation of patients with chronic paralytic respiratory insufficiency. Garrido-Garcia et al. conducted a study of patients with chronic ventilatory failure who were treated with phrenic nerve stimulation; they concluded that complete stable ventilation can be achieved using diaphragmatic pacing, and that it improves the prognosis and life quality of these patients. Girsch et al. conducted a similar study on children who had either central alveolar hypoventilation or total ventilatory insufficiency due to high cervical cord or brain stem lesion(s); they also concluded that, when chosen for the correct indications, phrenic nerve stimulation can increase the quality of life and should be preferred to positive pressure mechanical ventilation.

Implantation of a phrenic nerve stimulator is not without risks and adverse effects, and, therefore, should not be used for patients who can subsist independently of a mechanical respirator or who have temporary respiratory insufficiency. 

A search of peer-reviewed literature failed to reveal studies to support phrenic nerve stimulation as treatment of hiccups.

References:

1. Garrido-Garcia H, Alvarez J, Escribano P, Ganuza J, Banda F, Gambarrutta C, Garcia ME, Labarta C, Arroyo O, de la Cruz F, Gutierrez R, Moreno J. Treatment of chronic ventilatory failure using a diaphragmatic pacemaker. Spinal Cord 1998;36:310-314.
2. Glenn WWL. Fundamental considerations in pacing of the diaphragm for chronic ventilatory insufficiency: A multi-center study. PACE Nov 1988;11:2121-2127.
3. Onders RP, Aiyar H, Mortimer JT: Dept. of Surg, University Hospitals of Cleveland and Case Western Reserve University Ohio. "Characterization of the human diaphragm muscle with respect to the phrenic nerve motor points for diaphragmatic pacing". Am Surg. 2004 Mar; 70(3): 241-7.
4. Electrophrenic Pacemaker. Chicago, Illinois: Blue Cross Blue Shield Association – Technology Evaluation Center Assessment Program (1989 November) 39599.0-39599.1.
5. Talonen, P.P., Baer, G.A., et al.  Neurophysiological and technical considerations for the design of an implantable phrenic nerve stimulator. Medical and Biological Engineering and Computing (1990 January) 28(1): 31-37.
6. Bach, J.R. and K. O’Connor.  Electrophrenic Ventilation: a different perspective. Journal of the American Paraplegia Society (1991 January) 14(1): 9-17.
7. Brule, J.F., Leriche, B., et al.  Patients with high spinal cord injuries: evaluation of diaphragmatic function, indication of electrophrenic ventilation. Agressologie (1993) 34 (Spec No 2): 90-92.
8. Krieger, A.J., Gropper, M.R., et al.  Electrophrenic respiration after intercostal to phrenic nerve anastomosis in a patient with anterior spinal artery syndrome: technical case report. Neurosurgery (1994 October) 35(4): 760-764.
9. Staton, G., and R. Ingram.  Disorders of the Chest Wall. Scientific American, Inc.   (1994 January) 1 - 12.
10. Fritz, U., Braunn, U., et al. Implantation of a phrenic stimulator in central respiratory paralysis.  Anesthetist (1995 December) 44(12): 880 - 883.
11. Girsch, W., Koller, R., et al. Vienna phrenic pacemaker—experience with diaphragm pacing in children.  European Journal of Pediatric Surgery (1996 June) 6(3):140-3.
12. Garrido-Garcia, H., Mazaira Alvarez, J., et al. Spinal Cord (1998 May) 36(5):310-4.
13. Centers for Medicare and Medicaid Services – Phrenic Nerve Stimulator. Pub. No. 100-3, Manual Section No. 160.19.  Long-standing NCD, effective date not posted. http://www.cms.hhs.gov (Accessed April 26, 2006)
14. Electrophrenic Pacemaker. Chicago, Illinois: Blue Cross Blue Shield Association Medical Policy Reference Manual (2005 January) Surgery 7.01.37.

Coding Section

Procedure and diagnosis codes on Medical Policy documents are included only as a general reference tool for each policy. They may not be all-inclusive.

This medical policy was developed through consideration of peer-reviewed medical literature generally recognized by the relevant medical community, U.S. FDA approval status, nationally accepted standards of medical practice and accepted standards of medical practice in this community, Blue Cross Blue Shield Association technology assessment program (TEC) and other nonaffiliated technology evaluation centers, reference to federal regulations, other plan medical policies and accredited national guidelines.

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