Coverage Policy Manual
Policy #: 1998084
Category: Surgery
Initiated: February 1998
Last Review: October 2018
  Electrophrenic Pacemaker (Diaphragmatic Pacemaker)

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.
 
Regulatory Status
The NeuRx DPS™ RA/4 Respiratory Stimulation System is FDA approved under the Humanitarian Device Exemption process for patients age 18 years and older. The device is “intended for use in patients with stable, high spinal cord injuries with stimulatable diaphragms, but lack control of their diaphragms. The device is indicated to allow the patients to breathe without the assistance of a mechanical ventilator for at least 4 continuous hours a day” (FDA, 2008).
 
On September 28, 2011, the FDA approved the NeuRx Diaphragm Pacing System™ under the Humanitarian Device Exemption (HDE) program. This Humanitarian Use Device (HUD) is indicated for use in patients 21 years of age or older with amyotrophic lateral sclerosis (ALS) with a stimulatable diaphragm as demonstrated by voluntary contraction or phrenic nerve conduction studies, and who are experiencing chronic hypoventilation but are not progressed to an FVC less than 45% predicted.
 
An HUD is a device that is intended to be used to treat or diagnose a condition affecting less than 4,000 individuals in the United States per year. Approval for an HUD is obtained by submitting an HDE application to the FDA.  HDE approval allows marketing of the HUD. However, the HUD may only be allowed to be used in facilities that have a local institutional review board (IRB) that has approved the use of the HUD to treat or diagnose a specific disease or condition (U.S. Department of Health & Human Services).
 

Policy/
Coverage:
Effective January 2017
 
Meets Primary Coverage Criteria Or Is Covered For Contracts Without Primary Coverage Criteria
  
The use of an electrophrenic pacemaker meets primary coverage criteria for effectiveness and is covered for the following patients with permanent, severe hypoventilation caused by brain or high cervical cord lesions:
 
    • Quadriplegia (high C3 or above); OR
    • Central alveolar hypoventilation syndrome.
 
The use of an electrophrenic pacemaker meets primary coverage criteria that there be scientific evidence of effectiveness in improving health outcomes in patients with amyotrophic lateral sclerosis meeting the following criteria:
 
    • Have a stimulatable diaphragm (both right and left portions) as demonstrated by voluntary contraction or phrenic nerve conduction studies; AND
    • Are experiencing chronic hypoventilation.
    • Are unable to breathe on their own
    • The device is being implanted at a center that has received IRB approval for the implantation of this device in the treatment of amyotrophic lateral sclerosis.
 
Does Not Meet Primary Coverage Criteria Or Is Investigational For Contracts Without Primary Coverage Criteria
 
The use of electrophrenic pacemakers does not meet member benefit certificate primary coverage criteria that there be scientific evidence of effectiveness in improving health outcomes when any of the following conditions apply:
 
    • Patient can subsist independently of a mechanical respirator
    • Respiratory insufficiency is temporary
    • Pre-operative screening tests that 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.)
    • Chronic obstructive pulmonary disease
    • Treatment of hiccups
 
For contracts without primary coverage criteria, electrophrenic pacemakers are considered investigational when any of the following conditions apply:
 
    • Patient can subsist independently of a mechanical respirator
    • Respiratory insufficiency is temporary
    • Pre-operative screening tests that 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.)
    • Chronic obstructive pulmonary disease
    • Treatment of hiccups
 
Investigational services are specific contract exclusions in most member benefit certificates of coverage.
 
Effective January 2012- December 2016
The use of an electrophrenic pacemaker meets primary coverage criteria for effectiveness and is covered for the following patients with permanent, severe hypoventilation caused by brain or high cervical cord lesions:
    • Quadriplegia (high C3 or above); OR
    • Central alveolar hypoventilation syndrome.
 
The use of an electrophrenic pacemaker meets primary coverage criteria that there be scientific evidence of effectiveness in improving health outcomes in patients 21 years of age or older with amyotrophic lateral sclerosis meeting the following criteria:
    • Have a stimulatable diaphragm (both right and left portions) as demonstrated by voluntary contraction or phrenic nerve conduction studies; AND
    • Are experiencing chronic hypoventilation.
    • Are unable to breathe on their own
    • The device is being implanted at a center that has received IRB approval for the implantation of this device in the treatment of amyotrophic lateral sclerosis.
 
The use of electrophrenic pacemakers does not meet member benefit certificate primary coverage criteria that there be scientific evidence of effectiveness in improving health outcomes when any of the following conditions apply:
        • Patient can subsist independently of a mechanical respirator
        • Respiratory insufficiency is temporary
        • Pre-operative screening tests that 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.)
        • Chronic obstructive pulmonary disease
        • Treatment of hiccups
For contracts without primary coverage criteria, electrophrenic pacemakers are considered investigational when any of the following conditions apply:
        • Patient can subsist independently of a mechanical respirator
        • Respiratory insufficiency is temporary
        • Pre-operative screening tests that 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.)
        • Chronic obstructive pulmonary disease
        • Treatment of hiccups
Investigational services are specific contract exclusions in most member benefit certificates of coverage.
 
Effective prior to January 2012
The use of an electrophrenic pacemaker meets primary coverage criteria for effectiveness and is covered for patients with permanent, severe hypoventilation caused by brain or high cervical cord lesions:
Quadriplegia (high C3 or above) Central alveolar hypoventilation syndrome.
 
The use of electrophrenic pacemakers when/for:
    • Patient can subsist independently of a mechanical respirator
    • Respiratory insufficiency is temporary
    • Pre-operative screening tests that 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.)
    • Chronic obstructive pulmonary disease
    • Treatment of hiccups
is not covered based on benefit certificate primary coverage criteria that there be scientific evidence of effectiveness.
 
For contracts without primary coverage criteria, electrophrenic pacemakers when/for:
    • Patient can subsist independently of a mechanical respirator
    • Respiratory insufficiency is temporary
    • Pre-operative screening tests that 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.)
    • Chronic obstructive pulmonary disease
    • Treatment of hiccups
is considered investigational and/or medically unnecessary.  Investigational and medically unnecessary services are an exclusion in the member certificate of coverage.

Rationale:
2008 Update
A review of peer reviewed medical literature from July 2005 through August 2008 revealed a small number of papers addressing diaphragmatic pacing, but nothing that altered the policy coverage.
 
2014 Update
A search of the MEDLINE database did not reveal any new randomized controlled trials on the use of diaphragmatic pacing. No new information was identified that would prompt a change in the coverage statement.
 
2015 Update
A literature search conducted through January 2015 did not reveal any new information that would prompt a change in the coverage statement.
 
2016 Update
A literature search conducted through January 2016 did not reveal any new information that would prompt a change in the coverage statement. The key identified literature is summarized below.
 
No RCTs were identified in the literature. Some nonrandomized comparative studies and case series were identified, these are reviewed next.
 
Nonrandomized Controlled Trials
The largest study is from Reynolds and colleagues, who in 2015 reported 1-year follow-up of 50 MSA and 50 LNF patients matched for disease severity (Reynolds, 2015). To be included in the study, patients had (1) objective evidence of GERD, defined as an abnormal pH study, presence of biopsy proven Barrett esophagus, or esophagitis grade B or greater; (2) proton pump inhibitor (PPI) therapy for a minimum or 6 months; and (3) normal esophageal motility. Some of the patients had been included in previous reports. At 1 year after surgery, the 2 groups had similar GERD-HRQL (Health-Related Quality-of-Life) scores (4.2 MSA, 4.3 LNF; maximum, 50) and PPI use (17% of MSA, 8.5% of LNF). There was no difference in the number of patients reporting mild gas and bloating (27.6% MSA, 27.6% LNF), but more LNF patients reported severe gas and bloating (10.6% vs 0%, p=0.028). More LNF patients were unable to belch (8.5% of MSA and 25.5% of LNF, p=0.028) or vomit when needed (4.3% of MSA and 21.3% of LNF, p<0.002).
 
In 2014, Riegler and colleagues published 1-year results of an industry-sponsored multicenter registry (NCT01624506) that included a comparison with laparoscopic fundoplication (Reigler, 2014). The report included 202 MSA and 47LF (Nissen or Toupet) patients out of a planned enrollment of 734 patients. The choice of procedure was made by the surgeon at the time of laparoscopy, taking into account the presence of a large hiatal hernia along with other factors. In addition to having a greater frequency of large hiatal hernias (>3 cm, 45.7% vs 1.6%), the LF group was older and had a greater frequency of Barrett esophagus (19.1% vs 1.0%, p<0.001). Consistent with the greater severity of symptoms, patients who underwent LF had greater regurgitation and fewer patients who discontinued PPIs after treatment. Excessive gas and abdominal bloating (31.9% vs. 10.0%) and inability to vomit (55.6% vs 8.7%) were significantly higher after LF compared with MSA. Improvements in GERD-HRQL were similar for the two groups. Follow-up to 3 years in a larger number of patients is ongoing.
 
Single-Arm Studies
Bonavina and colleagues published longer follow-up from some of the patients in the pilot and multi-center registry studies in 2013 (Bonavina, 2013).  This study included a consecutive series of 100 patients who received MSA for GERD at their institution and were followed for a median of 3 years (range, 378 days to 6 years). Thirty of the patients had data beyond 5 years. The median GERD-HRQL improved from 24 off PPIs to 2 (p<0.001), and freedom from daily dependence on PPIs was achieved in 85% of patients. The time that esophageal pH was less than 4 decreased from 8.0% to 3.2% (p<0.001). Although 3 patients had the device removed for persistent GERD, odynophagia, or dysphagia, no occurrences of device migrations or erosions were observed during follow-up.
 
Ongoing and Unpublished Clinical Trials
Some currently unpublished trials that might influence this policy are listed below:
 
Ongoing:
(NCT02505945) an industry-sponsored or cosponsored trial.  The CALIBER Study Randomized Controlled Trial of LINX Versus Double-Dose Proton Pump Inhibitor Therapy for Reflux Disease; planned enrollment 150; completion date April 2017.
 
(NCT01940185) an industry-sponsored or cosponsored trial.  A post-Approval Study of the Lynx® Reflux Management System; planned enrollment 200; completion date September 2019.
 
2017 Update
A literature search conducted using the MEDLINE database through September 2017 did not reveal any new information that would prompt a change in the coverage statement.
 
2018 Update
A literature search was conducted through September 2018.  There was no new information identified that would prompt a change in the coverage statement.  

CPT/HCPCS:
64575Incision for implantation of neurostimulator electrode array; peripheral nerve (excludes sacral nerve)
64585Revision or removal of peripheral neurostimulator electrode array

References: Bonavina L, Saino G, Bona D, et al.(2013) One hundred consecutive patients treated with magnetic sphincter augmentation for gastroesophageal reflux disease: 6 years of clinical experience from a single center. J Am Coll Surg. Oct 2013;217(4):577-585. PMID 23856355

Gonzalez-Bermejo J, Morelot-Panzini C, Salacha F, et al.(2011) Diaphragm pacing improves sleep in patients with amyotrophic lateral sclerosis. Amyotroph Lateral Scler. 2011 Oct 24. [Epub ahead of print].

Onders RP, Elmo M, Khansarinia S, et al.(2009) Complete worldwide operative experience in laparospcopic diaphragm pacing: results and differences in spinal cord injured patients and amyotrophic lateral sclerosis patients. Surg Endosc. 2009 Jul;23(7):1433-40. Epub 2008 Dec 6.

Reynolds JL, Zehetner J, Wu P, et al.(2015) Laparoscopic Magnetic Sphincter Augmentation vs Laparoscopic Nissen Fundoplication: A Matched-Pair Analysis of 100 Patients. Am Coll Surg. Jul 2015;221(1):123-128. PMID 26095560

Riegler M, Schoppman SF, Bonavina L, et al.(2015) Magnetic sphincter augmentation and fundoplication for GERD in clinical practice: one-year results of a multicenter, prospective observational study. Surg Endosc. May 2015;29(5):1123-1129. PMID 25171881

Schmiesing CA, Lee J, Morton JM, et al.(2010) Laparoscopic diaphragmatic pacer placement-a potential new treatment for ALS patients: a brief description of the device and anesthetic issues. J Clin Anesth. 2010 Nov; 22(7):549-552.

U.S. Department of Health & Human Services. U. S. Food and Drug Administration.(2012) Medical Devices. Humanitarian Device Exemption. Accessed at http://www.fda.gov. Last accessed Jan. 2012.


Group specific policy will supersede this policy when applicable. This policy does not apply to the Wal-Mart Associates Group Health Plan participants or to the Tyson Group Health Plan participants.
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