Lung and Lobar Lung Transplant

POLICY NUMBER

A.7.03.07

DESCRIPTION

A lung transplant consists of replacing all or part of diseased lungs with healthy lung(s) or lobes. Transplantation is an option for patients with end-stage lung disease.

Solid organ transplantation offers a treatment option for patients with different types of endstage organ failure that can be lifesaving or provide significant improvements to a patient’s quality of life. Many advances have been made in the last several decades to reduce perioperative complications. Available data supports improvement in long-term survival as well as improved quality of life particularly for liver, kidney, pancreas, heart, and lung transplants. Allograft rejection remains a key early and late complication risk for any organ transplantation. Transplant recipients require life-long immunosuppression to prevent rejection. Patients are prioritized for transplant by mortality risk and severity of illness criteria developed by Organ Procurement and Transplantation Network and United Network of Organ Sharing.

Lung Transplant

In 2023, 46,630 transplants were performed in the United States procured from more than 16,000 deceased donors and 6,900 living donors. Lung transplants were the fourth most common procedure with 3,026 transplants performed from both deceased and living donors in 2023.

End-stage lung disease may derive from different etiologies. The most common indications for lung transplantation are chronic obstructive pulmonary disease, idiopathic pulmonary fibrosis, cystic fibrosis, alpha-1 antitrypsin deficiency, and idiopathic pulmonary arterial hypertension. Before consideration for transplant, patients should be receiving maximal medical therapy, including oxygen supplementation, or surgical options, such as lung-volume reduction surgery for chronic obstructive pulmonary disease. Lung or lobar lung transplantation is an option for patients with end-stage lung disease despite these measures.

A lung transplant refers to single-lung or double-lung replacement. In a single-lung transplant, only one lung from a deceased donor is provided to the recipient. In a double-lung transplant, both the recipient's lungs are removed and replaced by the donor's lungs. In a lobar transplant, a lobe of the donor’s lung is excised, sized appropriately for the recipient’s thoracic dimensions, and transplanted. Donors for lobar transplant have primarily been living-related donors, with one lobe obtained from each of 2 donors (generally friends or family members) in cases for which bilateral transplantation is required. There are also cases of cadaver lobe transplants.

Potential recipients who are 12 years of age and older are ranked according to the Lung Allocation Score. A score may range between 0 and 100 and incorporates predicted survival after transplantation and predicted survival on the waiting list; the Lung Allocation Score takes into consideration the patient’s disease and clinical parameters. The waiting list incorporates the Lung Allocation Score, geography, and blood type classifications. Children younger than 12 years old receive priority for lung allocation. Under this system, children younger than 12 years old with respiratory lung failure and/or pulmonary hypertension who meet criteria are considered “priority 1,” and all other candidates in the age group are considered “priority 2.” A lung review board has the authority to adjust scores on appeal for adults and children.

Potential Contraindications to Transplantation

Malignancy

Malignancies are common after lung transplantation, with 21% and 40% of patients reporting 1 or more malignancies at 5 and 10 years posttransplantation, respectively. Skin cancer occurred most frequently, and lymphoproliferative disorders were the malignancies most associated with morbidity posttransplantation.

Human Immunodeficiency Virus Infection

Current OPTN policy permits human immunodeficiency virus (HIV)-positive transplant candidates. The 2020 US Public Health Service guideline also allows for transplantations in HIV-positive recipients with proper screenings and effective regimens for HIV infections; it recommended that all transplant candidates receive HIV, hepatitis b virus (HBV), and hepatitis C virus (HCV) testing during hospital admission for transplant surgery. In 2022, the US Public Health Service published updated guidance for testing transplant candidates aged less than 12 years of age. They recommended that children less than 12 years of age who have received postnatal infectious disease testing are exempt from repeat pretransplant HIV, HBV, and HCV testing during hospital admission for transplant surgery.

The British HIV Association and the British Transplantation Society (2017) updated their guidelines on kidney transplantation in patients with HIV disease. These criteria for adding a patient to the waitlist may be extrapolated to other organs:

• Adherent with treatment, particularly antiretroviral therapy;

• Cluster of Differentiation 4 count greater than 100 cells/mL (ideally >200 cells/mL) for at least 3 months;

• Undetectable HIV viremia (<50 HIV-1 RNA copies/mL) for at least 6 months;

• No opportunistic infections for at least 6 months;

• No history of progressive multifocal leukoencephalopathy, chronic intestinal cryptosporidiosis, or lymphoma.

Other Infections

Infection with Burkholderia cenocepacia is associated with increased mortality in some transplant centers, a factor that may be considered when evaluating the overall risk of transplant survival. Two articles have evaluated the impact of infection with various species of Burkholderia on outcomes for lung transplantation for cystic fibrosis. In a study by Murray and colleagues, multivariate Cox survival models were applied to 1,026 lung transplant candidates and 528 transplant recipients. Of the transplant recipients, 88 were infected with Burkholderia. Among transplant recipients infected with B. cenocepacia, only those infected with nonepidemic strains (n=11) had significantly greater posttransplant mortality than uninfected patients (hazard ratio [HR], 2.52; 95% confidence interval [CI], 1.04 to 6.12; p=.04). Transplant recipients infected with Burkholderia gladioli (n=14) also had significantly greater posttransplant mortality than uninfected patients (HR, 2.23; 95% CI, 1.05 to 4.74; p=.04). When adjustments for specific species or strains were included, the Lung Allocation Scores of Burkholderia multivorans-infected transplant candidates were comparable with uninfected candidate scores, and scores for patients infected with nonepidemic B. cenocepacia or B. gladioli were lower. In a smaller study of 22 patients colonized with Burkholderia cepacia complex who underwent lung transplantation in 2 French centers, Boussaud and colleagues reported that the risk of death by univariate analysis was significantly higher for the 8 patients infected with B. cenocepacia than for the other 14 colonized patients (11 of whom had B. multivorans).

An analysis of international registry data by Yusen and colleagues found that non-cytomegalovirus (CMV) infection is a major cause of mortality within 30 days of a lung transplant in adults. A total of 655 (19%) of 3,424 deaths after transplants between 1990 and 2015 were due to non-CMV infection. Only 3 (0.1%) of the deaths were due to CMV infection.

Solid organ transplants are a surgical procedure and, as such, are not subject to regulation by the U.S. Food and Drug Administration.

The U.S. Food and Drug Administration regulates human cells and tissues intended for implantation, transplantation, or infusion through the Center for Biologics Evaluation and Research, under Code of Federal Regulation Title 21, parts 1270 and 1271. Solid organs used for transplantation are subject to these regulations.

POLICY

No benefits will be provided for a covered transplant procedure or a transplant evaluation unless the Member receives prior authorization through case management from Blue Cross & Blue Shield of Mississippi.

Lung transplantation may be consideredmedically necessary for carefully selected individuals with irreversible, progressively disabling, end-stage pulmonary disease unresponsive to maximum medical therapy including, but not limited to, one of the conditions listed below.

A lobar lung transplant from a living or deceased donor may be considered medically necessary for carefully selected individuals with end-stage pulmonary disease including, but not limited to, one of the conditions listed below:

• Bilateral bronchiectasis

• Alpha-1 antitrypsin deficiency

• Primary pulmonary hypertension

• Cystic fibrosis (both lungs to be transplanted)

• Bronchopulmonary dysplasia

• Postinflammatory pulmonary fibrosis

• Idiopathic/interstitial pulmonary fibrosis

• Sarcoidosis

• Scleroderma

• Lymphangiomyomatosis

• Emphysema

• Eosinophilic granuloma

• Bronchiolitis obliterans

• Recurrent pulmonary embolism

• Pulmonary hypertension due to cardiac disease

• Chronic obstructive pulmonary disease

• Eisenmenger's syndrome

Lung or lobar lung retransplantation after a failed lung or lobar lung transplant may be considered medically necessary in individuals who meet criteria for lung transplantation.

Lung or lobar lung transplantation is considered investigational in all other situations.

POLICY EXCEPTIONS

For Federal Employee Program (FEP) subscribers only, lung and lobar lung transplant may be considered medically necessary. (See FEP policy)

For State and School Employee subscribers, all transplants must be certified as medically necessary by the Plan’s Utilization Review Vendor. No benefits will be provided for any transplant procedure unless prior approval for the transplant is obtained. 

POLICY GUIDELINES

The coverage guidelines outlined in the Medical Policy Manual should not be used in lieu of the Member's specific benefit plan language.

Potential contraindications subject to the judgment of the transplant center:

• Known current malignancy, including metastatic cancer;

• Recent malignancy with high risk of recurrence;

• Untreated systemic infection making immunosuppression unsafe, including chronic infection;

• Other irreversible end-stage disease not attributed to lung disease;

• History of cancer with a moderate risk of recurrence;

• Systemic disease that could be exacerbated by immunosuppression;

• Psychosocial conditions or chemical dependency affecting ability to adhere to therapy.

Policy-specific

• Coronary artery disease (CAD) not amenable to percutaneous intervention or bypass grafting, or associated with significant impairment of left ventricular function*; or

• Colonization with highly resistant or highly virulent bacteria, fungi, or mycobacteria.

*Some patients may be candidates for combined heart and lung transplantation.

Individuals must meet United Network for Organ Sharing guidelines for a Lung Allocation Score greater than zero.

Lung-Specific GuidelinesBilateral lung transplantation is typically required when chronic lung infection and disease is present (i.e., associated with cystic fibrosis and bronchiectasis). Some, but not all, cases of pulmonary hypertension will require bilateral lung transplantation.

Bronchiolitis obliterans is associated with chronic lung transplant rejection, and thus may be the etiology of a request for lung retransplantation.

Medically Necessary is defined as those services, treatments, procedures, equipment, drugs, devices, items or supplies furnished by a covered Provider that are required to identify or treat a Member's illness, injury or Mental Health Disorders, and which Company determines are covered under this Benefit Plan based on the criteria as follows in A through D:

A.  consistent with the symptoms or diagnosis and treatment of the Member's condition, illness, or injury; and

B.  appropriate with regard to standards of good medical practice; and

C.  not solely for the convenience of the Member, his or her Provider; and

D.  the most appropriate supply or level of care which can safely be provided to Member. When applied to the care of an Inpatient, it further means that services for the Member's medical symptoms or conditions require that the services cannot be safely provided to the Member as an Outpatient.

For the definition of medical necessity, “standards of good medical practice” means standards that are based on credible scientific evidence published in peer-reviewed medical literature generally recognized by the relevant medical community, and physician specialty society recommendations, and the views of medical practitioners practicing in relevant clinical areas and any other relevant factors. BCBSMS makes no payment for services, treatments, procedures, equipment, drugs, devices, items or supplies which are not documented to be Medically Necessary. The fact that a Physician or other Provider has prescribed, ordered, recommended, or approved a service or supply does not in itself, make it Medically Necessary.

Investigative is defined as the use of any treatment procedure, facility, equipment, drug, device, or supply not yet recognized as a generally accepted standard of good medical practice for the treatment of the condition being treated and; therefore, is not considered medically necessary. For the definition of Investigative, “generally accepted standards of medical practice” means standards that are based on credible scientific evidence published in peer-reviewed medical literature generally recognized by the relevant medical community, and physician specialty society recommendations, and the views of medical practitioners practicing in relevant clinical areas and any other relevant factors. In order for equipment, devices, drugs or supplies [i.e, technologies], to be considered not investigative, the technology must have final approval from the appropriate governmental bodies, and scientific evidence must permit conclusions concerning the effect of the technology on health outcomes, and the technology must improve the net health outcome, and the technology must be as beneficial as any established alternative and the improvement must be attainable outside the testing/investigational setting.

POLICY HISTORY

8/1998: Approved by Medical Policy Advisory Committee (MPAC).

3/5/2002: Policy exception deleted.

5/1/2002: Type of Service and Place of Service deleted.

8/20/2003: ICD-9 procedure code range 33.50-33.52 listed separately, ICD-9 diagnosis code range 011.0-011.2, 011.4, 011.9, 277.00-277.01, 402.00-402.91, 415.11-415.19, 416.0, 416.8, 416.9, 491.20-491.21, 500-505 listed separately.

6/23/2004: Policy reviewed, Sources updated, Policy exception added.

07/21/2005: Policy reviewed by MPAC, "HIV positivity is not an absolute contraindication to transplant. Each individual transplant center will determine patient selection criteria for HIV positive patients."

10/17/2005: Code Reference table updated: CPT codes 32855, 32856 added; ICD-9 procedure code 00.91, 00.92, 00.93 added; HCPCS codes S2060, S2061 S2152 added; diagnosis code 518.3 added.

3/14/2006: Coding updated. CPT4 2006 revisions added to policy.

3/23/2006: Coding updated. CPT4 2006 revisions added to policy.

9/20/2007: Code Reference section updated. ICD-9 2007 revisions added to policy.

4/23/2009: Policy reviewed, no changes.

9/28/2009: Code reference section updated. New ICD-9 diagnosis code 416.2 added to covered table. ICD-9 procedure codes 32.3, 32.4, 32.5 deleted codes as of 9-30-2007 deleted from the covered table.

02/23/2011: Policy statement and guidelines updated to include specific contraindications for lung transplant.

02/24/2012: Deleted outdated references from the Sources section. Contraindications moved to the Policy Guidelines section, and the absolute and relative contraindications were combined. Deleted outdated references from the Sources section.

04/09/2013: Policy reviewed. In the lobar policy statement, "children and adolescents" was changed to "carefully selected patients."

09/16/2014: Policy reviewed; description updated. First medically necessary statement revised to state that lung transplantation may be considered medically necessary for carefully selected patients with irreversible, progressively disabling, end-stage pulmonary disease unresponsive to maximum medical therapy.Lobar policy statement revised to add that transplant may be from a living or deceased donor. Lobar policy statement list of conditions revised to remove pulmonary fibrosis and emphysematous bleb; added postinflammatory pulmonary fibrosis. Added the following policy statements: 1) Lung or lobar lung retransplantation after a failed lung or lobar lung transplant may be considered medically necessary in patients who meet criteria for lung transplantation. 2) Lung or lobar lung transplantation is considered investigational in all other situations.

02/16/2015: Policy description and statement unchanged. Policy guidelines updated to add "Policy-specific" to the list of potential contraindications subject to the judgment of the transplant center.

08/31/2015: Code Reference section updated for ICD-10. Corrected ICD-9 diagnosis code 415.2 to 415.12.

06/01/2016: Policy number A.7.03.07 added. Policy Guidelines updated to add medically necessary and investigative definitions.

09/30/2016: Code Reference section updated to revise code descriptions for ICD-10 diagnosis codes T82.817A and T82.818A.

09/19/2017: Policy description updated regarding FDA regulations. Policy statements unchanged.

09/29/2017: Code Reference section updated to add new ICD-10 diagnosis codes I27.20, I27.21, I27.22, I27.23, I27.24, and I27.29. Effective 10/01/2017.

12/22/2017: Code Reference section updated to add new 2018 CPT codes 0495T and 0496T.

08/31/2018: Policy description updated regarding FDA regulation. Policy statement unchanged.

09/12/2019: Policy reviewed. Policy statements unchanged. Code Reference section updated to remove deleted ICD-10 diagnosis code I27.2.

09/23/2019: Code Reference section updated to add new ICD-10 diagnosis codes I26.93 and I26.94, effective 10/01/2019.

09/17/2020: Policy description updated regarding solid organ transplantation and 2019 lung transplant data. Policy statements unchanged.

12/20/2021: Policy description updated regarding new data for transplants, potential contraindications, and other infections. Policy statements unchanged. Policy Guidelines updated to change "Nervous/Mental Conditions" to "Mental Health Disorders" and "Medically Necessary" to "medical necessity." Code Reference section updated to revise code description for ICD-10 diagnosis code M35.02 and to remove deleted ICD-10 diagnosis code J82 and ICD-9 code 518.3.

11/07/2022: Policy description updated regarding new data for transplants. Policy statements and Policy Guidelines updated to change "patients" to "individuals."

10/06/2023: Policy description updated regarding new data for transplants. Added information regarding testing transplant candidates. Policy statements unchanged.

10/01/2024: Code Reference section updated to add new ICD-10 diagnosis codes I26.03, I26.04, I26.95, and I26.96.

11/11/2024: Policy description updated regarding new data for transplants. Policy statements unchanged.

SOURCE(S)

Blue Cross Blue Shield Association policy # 7.03.07

CODE REFERENCE

This may not be a comprehensive list of procedure codes applicable to this policy.

The code(s) listed below are ONLY medically necessary if the procedure is performed according to the "Policy" section of this document.  

Covered Codes

CPT copyright American Medical Association. All rights reserved. CPT is a registered trademark of the American Medical Association.