Medical Policy: 07.01.77 

Original Effective Date: October 2018 

Reviewed: October 2018 

Revised:  

 

Benefit Application:

Benefit determinations are based on the applicable contract language in effect at the time the services were rendered. Exclusions, limitations or exceptions may apply. Benefits may vary based on contract, and individual member benefits must be verified. Wellmark determines medical necessity only if the benefit exists and no contract exclusions are applicable. This medical policy may not apply to FEP. Benefits are determined by the Federal Employee Program.

 

This Medical Policy document describes the status of medical technology at the time the document was developed. Since that time, new technology may have emerged or new medical literature may have been published. This Medical Policy will be reviewed regularly and be updated as scientific and medical literature becomes available.

 

Description:

Patients with high-level vertebrae C1-C3 spinal cord injuries typically experience respiratory muscle paralysis leading to chronic ventilatory insufficiency. The standard therapy for these patients is chronic mechanical ventilation via tracheostomy.

 

Non-invasive ventilation (NIV) such as positive ventilation or bilevel positive airway pressure is currently the first line treatment for amyotrophic lateral sclerosis (ALS) patients experiencing symptoms of respiratory insufficiency. At some point ALS affects the respiratory muscles so severely that bulbar paresis is combined with severe expiratory and inspiratory muscle weakness. There is a significant risk of impending respiratory failure or death and invasive ventilation becomes the only option for survival.

 

Diaphragmatic/phrenic nerve stimulation, is an alternative to mechanical ventilation for a select subgroup of patients. Diaphragmatic/phrenic nerve stimulation, also referred to phrenic pacing, phrenic nerve stimulation, diaphragm pacing, or electrophrenic respiration, is the electrical stimulation of the diaphragm via the phrenic nerve, the major nerve supply to the diaphragm that controls breathing. Patients with partial or complete respiratory insufficiency who have an intact phrenic nerve and diaphragm may be eligible for diaphragmatic/phrenic nerve stimulation. The patient should be alert, mentally competent, motivated and able to complete the training and rehabilitation needed for a successful outcome. Prior to implantation patients may undergo diaphragm electromyography, pulmonary function studies and/or polysomnograpy (i.e. sleep study). Common indications include patients with high quadriplegia (spinal cord injury) at or above C-3, chronic central alveolar hypoventilation syndrome, and amyotrophic lateral sclerosis ALS.

 

Diaphragmatic/Phrenic Nerve Stimulation Devices

Mark IV System

The Avery Breathing Pacemaker System (that is, the Mark IV™ Avery Biomedical Device, Inc., Commack, NY), surgically implanted (i.e. thoracotomy approach) by placing an electrode behind the phrenic nerve, either in the neck or in the chest. The electrode is connected to a radiofrequency receiving (generator) which is implanted just under the skin which are connected to an external transmitter and antennas to send radiofrequency energy to the implanted receivers. The receivers then convert the radio waves into stimulation pulses. These pulses are then sent down the electrodes to the phrenic nerves, causing the diaphragm to contract. This contraction causes the patient to inhale. When the pulses stop, the diaphragm relaxes and the patient exhales. Repetition of this series of pulses produces a normal breathing pattern.

 

Nonrandomized comparative studies, prospective case series and retrospective reviews have reported that the Mark IV device is a safe and effective alternative to invasive mechanical ventilation and is considered an established alternative therapy in appropriate candidates (upper motor neuron respiratory muscle paralysis [spinal cord injury]; central alveolar hypoventilation). Clinical trials with up to ten years follow-up reported success rates of 73%-94% in patients with spinal cord injuries.      

 

NeuRx System (NeuRX DPS and NeuRX DPS RA/4)

The NeuRx system (NeuRx DPS and NeuRx DPS RA/4) (Synapse Biomedical, Inc., Oberlin, OH) is performed laparoscopically to avoid the need for cervical or thoracic access to the phrenic nerve and potential risks of phrenic nerve damage. The system includes four electrodes implanted in the diaphragm to provide muscle stimulation, a fifth electrode implanted under the skin which grounds the system and completes the circuit, an electrode connector which groups the five electrodes exiting the skin into a socket, an external pulse generator (EPG) and removal cable to connect the electrode socket to the EPG. The NeuRx EPG sends electrical signals to the diaphragm i.e. inhalation upon electrical stimulation and exhalation on cessation of stimulation. 

 

The NeuRx DPS received FDA approval under an HDE (Humanitarian Device Exemption) application for use in amyotrophic lateral sclerosis (ALS) patients (see regulatory information below) and  NeuRx DPS RA/4 received FDA approval under an HDE (Humanitarian Device Exemption) for use in patients with stable, high spinal cord injuries (see regulatory information below). In order to receive HDE approval, a manufacturer must first be granted a Humanitarian Use Device (HUD) exemption by demonstrating that the device is designed to treat or diagnose a disease or condition that affects fewer than 4,000 people in the U.S. per year. Although data demonstrating the safety and probable clinical benefit are required for HDE approval, clinical trials evaluating the effectiveness of the device are not required. Following HDE approval, the hospital or health care facility institutional review board (IRB) must also approve the use of the device at the institution before the device may be used in the patient.  

 

Remede System

The Remede system (Respicardia, Inc., Minneapolis, MN) is an implanted nerve stimulator used to treat moderate to severe central sleep apnea (CSA) in adults. The system includes a battery powered pulse generator that is implanted under the skin in the upper chest and thin wire leads that are threaded through veins (transvenous) near the nerve that stimulates breathing (phrenic nerve). The system is programmed using an external system programmer and programming wand. The Remede system delivers a small electrical stimulus to the phrenic nerve while a patient is asleep. This stimulus makes the diaphragm muscle contract, which causes the patient to take a breath. The Remede system has 2 modes, it can be set to generate pulses at a fixed rate (asynchronous therapy) or it can deliver a pulse only when it detects a pause in breathing (synchronous therapy). The physician is able to set the stimulator to deliver the most appropriate therapy for the patient. The system has safeguards to make sure that therapy is only delivered during sleep, for example it works only at the time of day when the patient is expected to be sleeping and it turns on only when the patient is inactive and lying down. 

 

The FDA approval of the Remede system is based on an industry-supported, multicenter, prospective, randomized controlled sham study that aimed to determine the safety and effectiveness for treatment central sleep apnea (CSA) (Costanza et. al., 2016). A total of 151 adult subjects were randomized to receive either medical management and the Remede system (n=73),or medical management and inactive sham Remede system (n=78). The subjects in the study were an average of 65 years old and predominately Caucasian (95%) and males (89%). The primary endpoint was a 50% or greater reduction in AHI from baseline at 6 months, and the AHI was determined using polysomnography. Subjects were evaluated regularly until the end of the trial. After 6 months, the Remede system was activated in the sham group. Effectiveness was based on modified intention to treat (ITT) data at 6 months (n=141). A significant higher number of subjects in the active Remede system group had a 50% or better reduction in AHI from baseline to 6 months post-procedure (p<0.0001). The success rate for the active Remede system group was 51% compared to 11% in the sham group for a total difference of 41% (95% CI, 25% to 51%; p<0.0001). A total of 76% of subjects in the Remede system group reported improvement in quality of life. Safety results were based on intention to treat (ITT) data for 12 months (n=151). There were 7 deaths but none found to be related to the device or treatment. The number of subjects free from serious adverse events (AEs) was 91% (95% CI, 86% to 95%); however, 13 subjects had serious AEs including impending pocket erosion, implant site infection, lead dislodgement, concomitant device interaction, elevated transaminase, extra-respiratory stimulation, implant site hematoma, lad component failure, lead displacement, and non-cardiac chest pain. The number of subjects who experienced non-serious AEs were 48%. Implants were unsuccessful in 5 subjects, and the rate of explants was 5.3% (8/151). The authors concluded that transvenous neurostimulation could provide a treatment option for central sleep apnea. Limitations of the study included low percentage of female subjects and potential referral bias.

 

In 2015, Abraham et. al. evaluated transvenous unilateral phrenic nerve stimulation to treat central sleep apnea (CSA) in a prospective, multicenter, nonrandomized study. Fifty-seven patients with CSA underwent baseline polysomnography followed by transvenous phrenic nerve stimulation system implantation and follow-up. Feasibility was assessed by implantation success rate and therapy delivery. Safety was evaluated by monitoring of device- and procedure-related adverse events. Efficacy was evaluated by changes in the apnea-hypopnea index at 3 months. Quality of life at 6 months was evaluated using a sleepiness questionnaire, patient global assessment, and, in patients with heart failure at baseline, the Minnesota Living With Heart Failure Questionnaire. The study met its primary end point, demonstrating a 55% reduction in apnea-hypopnea index from baseline to 3 months (49.5 ± 14.6 episodes/h vs. 22.4 ± 13.6 episodes/h of sleep; p < 0.0001; 95% confidence interval for change: -32.3 to -21.9). Central apnea index, oxygenation, and arousals significantly improved. Favorable effects on quality of life and sleepiness were noted. In patients with heart failure, the Minnesota Living With Heart Failure Questionnaire score significantly improved. Device- or procedure-related serious adverse events occurred in 26% of patients through 6 months post therapy initiation, predominantly due to lead repositioning early in the study. Therapy was well tolerated. Efficacy was maintained at 6 months. The authors concluded, transvenous unilateral phrenic nerve stimulation appears safe and effective for treating CSA, and these findings should be confirmed in a prospective randomized, controlled trial (NCT01124370).  

 

In 2016, Jagielski et. al. evaluated the 12 month clinical outcomes of patients with central sleep apnea (CSA) treated with unilateral transvenous phrenic nerve stimulation in the prospective, multi-center, non-randomized Remede system pilot study. Forty-seven patients with CSA were treated with the Remede system for a minimum of 3 months. Sleep disordered breathing parameters were evaluated by polysomnography (PSG) as 3, 6 and 12 month follow-up. Sleep symptoms and quality of life were also evaluated, Forty-one patients completed all follow-up PSGs and were included in the analysis. At 12 months, there was sustained improvement compared with baseline in the apnea-hypopnea index, central apnea index and there was sustained improvement in the oxygen desaturation index, rapid eye movement sleep and sleep efficiency. There were continued favorable effects on sleepiness and quality of life. Three deaths unrelated to Remede system therapy and five serious adverse events occurred over 12 months of follow-up. The authors noted the main limitations of the study were the non-randomized, open-label nature of the trial, the small sample size, the small number of women enrolled in the study, and the fact that many of the parameters studied were only exploratory and hypothesis-generating and the results should be confirmed with future larger, randomized, controlled studies.

 

In 2018, Costanza et. al. reported the 12 month results from Remede system pivotal trial (see above) to evaluate the benefits of this therapy for central sleep apnea (CSA). Reproducibility of treatment effect was assessed in the former control group in whom the implanted device was initially inactive for the sixth month and subsequently activated when the randomized control assessments were complete. Patients with moderate-to-severe central sleep apnea implanted with the Remede system were randomized to therapy activation at 1 month (treatment) or after 6 months (control). Sleep indices were assessed from baseline to 12 months in the treatment group and from 6 to 12 months in former controls. In the treatment group, a ≥50% reduction in apnea-hypopnea index occurred in 60% of patients at 6 months (95% confidence interval [CI] 47% to 64%) and 67% (95% CI 53% to 78%) at 12 months. After 6 months of therapy, 55% of former controls (95% CI 43% to 67%) achieved ≥50% reduction in apnea-hypopnea index. Patient Global Assessment was markedly or moderately improved at 6 and 12 months in 60% of treatment patients. Improvements persisted at 12 months. A serious adverse event within 12 months occurred in 13 patients (9%). Phrenic nerve stimulation produced sustained improvements in sleep indices and quality of life to at least 12 months in patients with central sleep apnea. The similar improvement of former controls after 6 months of active therapy confirms benefits are reproducible and reliable.

 

Summary

Based on review of the peer reviewed medical literature the evidence to date is insufficient regarding the safety and effectiveness of the Remede system for the treatment of central sleep apnea (CSA) with only 12 months of outcome data available from single randomized controlled sham study. The device has also not been compared with CPAP or other therapies for central sleep apnea (CSA). Further large, randomized, comparative, controlled studies are needed to determine the safety and efficacy, and the further studies also need to help define optimal patient selection and assess-long term outcomes. The evidence is insufficient to determine the effects of this technology on net health outcomes.   

 

Practice Guidelines and Position Statements

American Academy of Neurology (AAN)

In 2009, the American Academy of Neurology (AAN) issued a practice parameter update on the care of the patient with amyotrophic lateral sclerosis (ALS): drug, nutritional and respiratory therapies an evidence based review. The recommendations in this practice parameter update does not mention diaphragmatic/phrenic nerve stimulation or diaphragm pacing as a treatment. There has been no update to this practice parameter since 2009.

 

American Thoracic Society (ATS)

In 2010, the American Thoracic Society clinical policy statement on congenital central hypoventilation syndrome in their discussion of the diagnosis and management of children with congenital central hypoventilation syndrome (CCHS) which states: diaphragm pacers can be used for daytime support of ambulatory children who require full-time ventilator support, in combination with positive pressure ventilation at night.

 

American Academy of Sleep Medicine (AASM)

In 2016, the American Academy of Sleep Medicine (AASM) issued an updated guideline on the treatment of central sleep apnea syndromes in adults with an evidence based literature review and meta-analysis. This guideline does not include diaphragmatic/phrenic nerve stimulation or diaphragm pacing as a recommended treatment for this condition.

 

American College of Cardiology(ACC)/American Heart Association (AHA)/Heart Failure Society of America (HFSA)

In 2017, ACC/AHA/HFSA issued a focused update of the 2015 ACCP/AHA guideline for the management of heart failure which includes the following recommendations regarding sleep disordered breathing:

  • In patients with NYHA class II-IV heart failure and suspicious of sleep disordered breathing or excessive daytime sleepiness, a formal sleep assessment is reasonable. 
  • In patients with cardiovascular disease and obstructive sleep apnea, CPAP may be reasonable to improve sleep quality and daytime sleepiness. 
  • In patients with NYHA class II-IV heart failure with reduced ejection faction (HFrEF) and central sleep apnea, adoptive servo-ventilation causes harm.

 

This guideline does not include diaphragmatic/phrenic nerve stimulation or diaphragm pacing as a recommended treatment for heart failure management.  

 

Regulatory Status

The Avery Breathing Pacemaker System (that is, the Mark IV™ Avery Biomedical Device, Inc., Commack, NY) is the only other diaphragmatic/phrenic stimulator system cleared for use by the FDA in the United States for ventilator-dependent individuals. The pacemaker is classified as a Class III neurologic therapeutic device requiring premarket approval (PMA). The device is approved “for persons who require chronic ventilatory support because of upper motor neuron respiratory muscle paralysis (RMP) or because of central alveolar hypoventilation (CAH) and whose remaining phrenic nerve, lung, and diaphragm function is sufficient to accommodate electrical stimulation” (FDA, 2003). Clinical trials that have studied the efficacy of this device have been very limited and included small numbers of subjects.

 

FDA clearance for distribution of the NeuRx DPS RA/4 Respiratory Stimulation System was granted under a Humanitarian Device Exemption (HDE) on June 17, 2008. The FDA-approved indications are:

  • 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 and is for use only in patients 18 years of age or older.

 

This FDA approval is subject to the manufacturer developing “an acceptable method of tracking device implantation to individual patient recipients” (FDA, 2008).

 

FDA clearance of the NeuRx device was primarily based on a prospective, nonrandomized, multicenter clinical trial that included 50 subjects throughout the U.S. and Canada (Onders, 2009). In the clinical trial, 98% of subjects with spinal cord injury were able to breathe normally for at least 4 hours following implantation of the device, while 50% have been able to completely eliminate their need for mechanical ventilation.

 

The study inclusion criteria were:

  • Age 18 years or older;
  • Cervical spinal cord injury with dependence on mechanical ventilation;
  • Clinically stable following acute spinal cord injury;
  • Bilateral phrenic nerve function clinically acceptable as demonstrated with electromyography (EMG) recordings and nerve conduction times;
  • Diaphragm movement with stimulation visible under fluoroscopy;
  • Clinically acceptable oxygenation on room air (greater than 90% 02 saturation);
  • Hemodynamically stable;
  • No medical co-morbidities that would interfere with the proper placement or function of the device;
  • Committed primary caregiver;
  • Negative pregnancy test in females of child-bearing potential;
  • Informed consent from the device user or designated representative.

 

Exclusion criteria were:

  • Co-morbid medical conditions that preclude surgery;
  • Active lung disease (obstructive, restrictive or membrane diseases);
  • Active cardiovascular disease;
  • Active brain disease;
  • Hemodynamic instability or low oxygen levels on room air;
  • Hospitalization for or a treated active infection, within the last 3 months;
  • Significant scoliosis or chest deformity;
  • Marked obesity;
  • Anticipated poor compliance with protocol by either the device user or primary caregiver;
  • Currently breastfeeding.

 

On September 28, 2011, the FDA issued an approval under an HDE application for use of the NeuRx DPS Diaphragm Pacing System in:

  • Amyotrophic lateral sclerosis (ALS) patients with a stimulatable diaphragm (both right and left portions) as demonstrated by voluntary contraction or phrenic nerve conduction studies, and who are experiencing chronic hypoventilation (CH), but not progressed to an FVC (forced vital capacity) less than 45% predicted. For use only in patients 21 years of age or older.

 

This approval was based on results of a multicenter, prospective study of the NeuRx Diaphragm Pacing Stimulation (DPS) System of motor-point stimulation for conditioning the diaphragm of subjects with ALS which showed the probable benefit to health from use of the device outweighed the risks of injury or illness from its use (FDA/HDE; SSPB, 2011).

 

The Remede System was approved by the FDA on October 6, 2017 for the treatment of moderate to severe central sleep apnea in adult individuals. The manufacturer describes the device as:

  • An implantable pacemaker-like device that was designed for improving central sleep apnea (CSA) using Respidrive™, a Respiratory Rhythm Management™ algorithm. The Remede system delivers electrical pulses via a proprietary, novel transvenous implantable lead to one of the body’s two phrenic nerves. The Remede system therapy is intended to stimulate the diaphragm to restore a more natural, less disrupted, breathing pattern.

 

Prior Approval:

Not required.

 

Policy:

Diaphragmatic/phrenic nerve stimulation with the Mark IV system as an alternative to mechanical ventilation is considered medically necessary when all of the following criteria is met:

  • The individual has chronic central alveolar hypoventilation syndrome/congenital central hypoventilation syndrome; OR 
  • High quadriplegia at or above C-3; AND    
  • 18 years and older; AND
  • Diaphragm movement with stimulation visable under fluoroscopy or ultrasound; AND
  • Have intact phrenic nerve function; AND
  • 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 this device; AND   
  • Diaphragmatic/phrenic nerve stimulation will allow the individual to breath without the assistance of a mechanical ventilator for at least 4 continuous hours a day.     

 

The NeuRx DPS RA/4 Respiratory Stimulation System as an alternative to mechanical ventilation is considered medically necessary when provided in accordance with the Humanitarian Device Exemption (HDE) specifications of the U.S. Food and Drug Administration when all of the following criteria is met: 

  • 18 years and older; AND
  • High quadriplegia at or above C-3; AND
  • Diaphragm movement with stimulation visable under fluoroscopy or ultrasound; AND
  • Have intact phrenic nerve function; AND
  • 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 this device; AND
  • Diaphragm pacing system will allow the individual to breathe without the assistance of a mechanical ventilator for at least 4 continuous hours a day; AND   
  • This device may only be used in a facility that has an institutional review board (IRB) to oversee the clinical application of this device. The IRB must approve the application of this device to ensure that it will be used in accordance with the FDA labeled indication under HDE (documentation of the IRB approval may be requested to ensure compliance with the FDA labeled indication under HDE). See also medical policy 10.01.14 Humanitarian Use Devices.

 

The NeuRx DPS Diaphragm Pacing System as an alternative to mechanical ventilation is considered medically necessary when provided in accordance with the Humanitarian Device Exemption (HDE) specifications of the U.S. Food and Drug Administration when all of the following criteria is met:

  • 21 years of age or older; AND
  • Amyotrophic lateral sclerosis (ALS); AND  
  • The individual is experiencing chronic hypoventilation, but has not progressed to an FVC (forced vital capacity) less than 45% predicted; AND
  • Diaphragm movement with stimulation visable under fluoroscopy or ultrasound (both right and left portions); AND
  • Have intact phrenic nerve function; AND
  • The 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 this device; AND       
  • Diaphragm pacing system will allow the individual to breathe without the assistance of a mechanical ventilator for at least 4 continuous hours a day; AND   
  • This device may only be used in a facility that has an institutional review board (IRB) to oversee the clinical application of this device. The IRB must approve the application of this device to ensure that it will be used in accordance with the FDA labeled indication under HDE (documentation of the IRB approval may be requested to ensure compliance with the FDA labeled indication under HDE). See also medical policy 10.01.14 Humanitarian Use Devices.

 

Replacement and Revisions

Replacement or revisions of diaphragm/phrenic nerve stimulation and diaphragm pacing systems (generator and/or leads) is considered medically necessary if the individual meets the above criteria, and is no longer under warranty or cannot be repaired.  

 

Diaphragm/phrenic nerve stimulation and diaphragm pacing systems are considered investigational for all other indications including but not limited to the following:

  • When the above criteria is not met
  • In individuals whose phrenic nerve or diaphragm function is not sufficient to achieve adequate diaphragm movement from the electrical stimulation
  • For treatment of any other condition where the phrenic nerve and diaphragm are intact including:
    • Obstructive lung disease
    • Restrictive lung disease
    • Singultus (hiccups)
    • Central sleep apnea    
    • Management of heart failure and treatment of sleep related disorders including but not limited to central sleep apnea
  • 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 phrenic nerve or diaphragm pacemaker device

 

Based on review of the peer reviewed medical literature the evidence is insufficient to determine  the effects of this technology on net health outcomes for indications other than the ones listed above. Further large, randomized, comparative, controlled studies are needed to determine the safety and efficacy, and the further studies also need to help define optimal patient selection and assess-long term outcomes.

 

Policy Guidelines

Diaphragm Fluoroscopy (Sniff Test): a diaphragm fluoroscopy (sniff test) checks how the diaphragm (the muscle that controls breathing) moves when an individual breathes normally and when they inhale quickly. The diaphragm normally moves down when a person inhales, and up when a person exhales. Both the right and left sides of the diaphragm should move in the same direction at the same time. This test shows if there are problems with the phrenic nerve, which controls movement of the diaphragm.    

  • Normal Diaphragmatic Motion:
    • The diaphragm contracts during inspiration: moves downward
    • The diaphragm relaxes during expiration: moves upwards
    • Both hemi-diaphragms move together
  • Abnormal Diaphragmatic Motion
    • The affected hemi-diaphragm does not move downwards during inspiration
    • Paradoxical motion can occur (diaphragm moves opposite to the normal direction of its movements) 
    • Weak response to phrenic nerve stimulation or there is unilateral movements

 

Stimulation of the phrenic nerve may be performed by percutaneously stimulating the phrenic nerve in the neck and assessing diaphragmatic movements.

 

A provider may utilize electromyography (EMG) or nerve conduction studies to assess phrenic nerve function.

 

Procedure Codes and Billing Guidelines:

To report provider services, use appropriate CPT* codes, Alpha Numeric (HCPCS level 2) codes, Revenue codes and / or diagnosis codes.

  • 0424T Insertion or replacement of neurostimulator system for treatment of central sleep apnea; complete system (transvenous placement of right or left stimulation lead, sensing lead, implantable pulse generator) 
  • 0425T Insertion  or replacement of sensing lead only for treatment of central sleep apnea 
  • 0426T Insertion or replacement of stimulation lead only for treatment of central sleep apnea
  • 0427T Insertion or replacement of pulse generator only for treatment of central sleep apnea
  • 0428T Removal of neurostimulator system for treatment for central sleep apnea; pulse generator only 
  • 0429T Removal of sensing lead only for treatment of central sleep apnea
  • 0430T Removal of stimulation lead only for treatment of central sleep apnea 
  • 0431T Removal and replacement of neurostimulator system for treatment of central sleep apnea, pulse generator only 
  • 0432T Repositioning of neurostimulator system for treatment of central sleep apnea; stimulation lead only
  • 0433T Repositioning of sensing lead only for treatment of central sleep apnea 
  • 0434T Interrogation device evaluation implanted neurostimulator pulse generator system for central sleep apnea
  • 0435T Programming device evaluation of implanted neurostimulator pulse generator system for central sleep apnea; single session 
  • 0436T Programming device evaluation of implanted neurostimulator pulse generator system for central sleep apnea; during sleep study
  • 64575 Incision for implantation of neurostimulator electrode array; peripheral nerve (excludes sacral nerve)
  • 64580 Incision for implantation of neurostimulator electrode array; neuromuscular
  • 64585 of peripheral neurostimulator electrode array
  • 64590 Insertion or replacement of peripheral or gastric neurostimulator pulse generator or receiver, direct or inductive coupling
  • 64595 Revision or removal of peripheral or gastric neurostimulator pulse generator or receiver 
  • 95970 Electronic analysis of implanted neurostimulator pulse generator/transmitter (eg, contact group(s), interleaving, amplitude, pulse width, frequency [Hz], on/off cycling, burst, magnet mode, dose lockout, patient selectable parameters, responsive neurostimulation, detectopm algorithms, closed loop parameters, and passive parameters) by physician or other qualified health care professional; with brain, cranial nerve, spinal cord, peripheral nerve, or sacral nerve, neurostimulator pulse generator/transmitter, without programming  
  • 95971 Electronic analysis of implanted neurostimulator pulse generator/transmitter (eg, contact group(s), interleaving, amplitude, pulse width, frequency [Hz], on/off cycling, burst, magnet mode, dose lockout, patient selectable parameters, responsive neurostimulation, detectopm algorithms, closed loop parameters, and passive parameters) by physician or other qualified health care professional; with simple spinal cord or peripheral nerve (eg, sacral nerve) neurostimulator pulse generator/transmitter programming by physician or other qualified health care professional
  • 95972 Electronic analysis of implanted neurostimulator pulse generator/transmitter (eg, contact group(s), interleaving, amplitude, pulse width, frequency [Hz], on/off cycling, burst, magnet mode, dose lockout, patient selectable parameters, responsive neurostimulation, detectopm algorithms, closed loop parameters, and passive parameters) by physician or other qualified health care professional; with complex spinal cord or peripheral nerve (eg, sacral nerve) neurostimulator pulse generator/transmitter programming by physician or other qualified health care professional
  • C1767 Generator neurostimulator (implantable) non-rechargeable
  • C1778 Lead, neurostimulator (implantable)
  • C1787 Patient programmer, neurostimulator
  • C1816 Receiver and/or transmitter, neurostimulator (implantable)
  • C1820 Generator, neurostimulator (implantable), non high-frequency with rechargeable battery and charging system
  • C1822 Generator, neurostimulator (implantable), high frequency, with rechargeable battery and charging system
  • C1823 Generator, neurostimulator (implantable), non-rechargeable, with transvenous sensing and stimulation leads
  • C1897 Lead, neurostimulator test kit (implantable)
  • L8679 Implantable neurostimulator, pulse generator any type
  • L8680 Implantable neurostimulator electrode, each
  • L8681 Patient programmer (external) for use with implantable programmable neurostimulator pulse generator, replacement only
  • L8682 Implantable neurostimulator radiofrequency receiver
  • L8683 Radiofrequency transmitter (external) for use with implantable neurostimulator radiofrequency receiver
  • L8685 Implantable neurostimulator pulse generator, single array, rechargeable includes extension
  • L8686 Implantable neurostimulator pulse generator, single array, nonrechargeable, includes extension
  • L8687 Implantable neurostimulator pulse generator, dual array, rechargeable, includes extension
  • L8688 Implantable neurostimulator pulse generator, dual array, nonrechargeable, includes extension
  • L8689 External recharging system for battery (internal) for use with implantable neurostimulator, replacement only

 

Selected References:

  • 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. PMID 25770408
  • Ali A, Flageole H. Diaphragmatic pacing for the treatment of congenital central alveolar hypoventilation syndrome. J Pediatr Surg. 2008; 43(5):792-796. PMID 18485940
  • 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. PMID 19016542
  • Costanzo MR, Ponikowski P, Javaheri S, et al. Transvenous neurostimulation for central sleep apnoea: a randomised controlled trial. Lancet. 2016; 388(10048):974-982. PMID 27598679
  • DiMarco AF, Onders RP, Ignagni A, et al. Phrenic nerve pacing via intramuscular diaphragm electrodes in tetraplegic subjects. Chest. 2005; 127(2):671-678. PMID 15706014
  • 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. PMID 12137341
  • 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. PMID 22023158
  • 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. PMID 18475279
  • 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):851-855. PMID 23042087
  • 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. PMID 17874683
  • 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. PMID 19067067
  • 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. PMID 20797750
  • 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. PMID 21238643
  • 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. PMID 24458038
  • 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 PMID 22777487
  • Weese-Mayer D, Berry-Kravis E, Ceccherini I, et. al. American Thoracic Society (ATS) Clinical Policy Statement: Congenital Central Hypoventilation Syndrome: Genetic Basis, Diagnosis and Management. September 2009. Am J Respir Crit Care Med Vol 181 pp 626-644, 2010
  • Centers for Medicare and Medicaid Services. National Coverage Determination for Phrenic Nerve Stimulators. NCD 160.19. Longstanding coverage determination; Effective date not posted. 
  • 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.
  • 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, 2017. 
  • 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. 
  • 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. 
  • 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. 
  • U.S. Food and Drug Administration (FDA). Center for Devices and Radiological Health (CDRH). Premarket Notification Database. RemedÄ“ System (Respicardia, Inc., Minnetonka, MN). Summary of Safety and Effectiveness. No. P160039. Rockville, MD: FDA. October 6, 2017. 
  • National Institute for Health and Clinical Excellence (NICE) Intramuscular Diaphragm Stimulation of Ventilator Dependent Chronic Respiratory Failure Caused by High Spinal Cord Injuries. Interventional Procedure Guidance (IPG594). Published September 2017. 
  • National Institute for Health and Clinical Excellence (NICE) Intramuscular Diaphragm Stimulation for Ventilator-Dependent Chronic Respiratory Failure Caused by Motor Neuron Disease. Interventional Procedure Guidance (IPG593). Published September 2017
  • UpToDate. Central Sleep Apnea Treatment. M Safwan Badr M.D., Topic last updated June 5, 2018. 
  • UpToDate. Pacing the Diaphragm: Patient Selection, Evaluation, Implantation and Complications. Donald W. Marion M.D., Topic last updated April 30, 2018. 
  • UpToDate. Congenital Central Hypoventilation Syndrome and Other Causes of Sleep-Related Hypoventilation in Childre. Robert T. Brouillette M.D., Topic Last Updated June 14, 2018. 
  • Bhimji S, Mosenifar Z et. al. Diaphragm Pacing. Medscape. 
  • Yancy C, Jessup M, Bozkurt B, et. al. 2017 ACC/AHA/HFSA Focused Update of the 2013 ACCF/AHA Guideline for the Management of Heart Failure. Circulation 2014;136:e137-e161
  • Yancy C, Jessup M, Bozkurt B, et. al. 2013 ACCF/AHA Guideline for the Management of Heart Failure. Journal of the American College of Cardiology Vol. 62, No. 16, 2013
  • Aurora RN, Bista SR, Casey KR, et. al. Updated Adaptive Servo-Ventilation Recommendations for the 2012 AASM Guideline: The Treatment of Central Sleep Apnea Syndromes in Adults: Practice Parameters with an Evidence Based Literature Review and Meta-Analyses. Journal of Clinical Sleep Medicine Vol. 12, No. 5, 2016
  • Costanzo M, Khayat R, Ponikowski P, et. al. Mechanisms and clinical consequences of untreated central sleep apnea in heart failure. Journal of the American College of Cardiology Vol. 65, No. 1, 2015
  • Costanzo M, Ponikowski P, Javaheri S, et. al. Transvenous neurostimulation for central sleep apnea: a randomized controlled trial. Lancet 2016;388:974-82
  • Costanzo M, Ponikowski P, Javaheri S, et. al. Sustained 12 month benefit of phrenic nerve stimulation for central sleep apnea. 
  • Jakielski D, Ponikowski P, Augostini R, et. al. Tranvenous stimulation of the phrenic nerve for the treatment of central sleep apnea: 12 months experience with remede system. European Journal of Heart Failure 2016 18, 1386-1393
  • Ding N and Zhang X. Transvenous phrenic nerve stimulation a novel therapeutic approach for central sleep apnea. J Thorac Dis 2018;10(3) 
  • Cowie M, Woehrle H, Wegscheider K, et. al. Adaptive servo-ventilation for central sleep apnea in systolic heart failure. The New England Journal of Medicine September 17, 2015 Vol. 373 No. 12 1095-1105
  • ECRI Institute. Hotline Response. Neurostimulation Therapy for Central Sleep Apnea. March 2016.

 

Policy History:

  • October 2018, New Policy

Wellmark medical policies address the complex issue of technology assessment of new and emerging treatments, devices, drugs, etc.   They are developed to assist in administering plan benefits and constitute neither offers of coverage nor medical advice. Wellmark medical policies contain only a partial, general description of plan or program benefits and do not constitute a contract. Wellmark does not provide health care services and, therefore, cannot guarantee any results or outcomes. Participating providers are independent contractors in private practice and are neither employees nor agents of Wellmark or its affiliates. Treating providers are solely responsible for medical advice and treatment of members. Our medical policies may be updated and therefore are subject to change without notice.

 

*CPT® is a registered trademark of the American Medical Association.