Transplantation of Lung Or Lobar Lung Product Applicability

bmchp.org | 888-566-0008 wellsense.org | 877-957-1300

Medical Policy

Transplantation of Lung or Lobar Lung

Policy Number: OCA 3.24 Version Number: 15 Version Effective Date: 04/01/18

Product Applicability All Plan+ Products

Well Sense Health Plan Boston Medical Center HealthNet Plan New Hampshire Medicaid MassHealth ACO NH Health Protection Program MassHealth MCO Qualified Health Plans/ConnectorCare/Employer Choice Direct

Senior Care Options ◊

Notes: + Disclaimer and audit information is located at the end of this document. ◊ The guidelines included in this Plan policy are applicable to members enrolled in Senior Care Options only if there are no criteria established for the specified service in a Centers for Medicare & Medicaid Services (CMS) national coverage determination (NCD) or local coverage determination (LCD) on the date of the prior authorization request. Review the member’s product-specific benefit documents at www.SeniorsGetMore.org to determine coverage guidelines for Senior Care Options.

Policy Summary The Plan considers lung transplantation for end-stage pulmonary disease for adults and children to be medically necessary when applicable Plan medical criteria are met, including lobar lung transplant and lung transplantation (single-lung or double-lung replacement). All transplant-related consults, evaluations, procedures, and post-transplant follow-up services should be managed within the Plan’s provider network or at the most appropriate preferred transplant facility, depending upon the type of transplant. Prior authorization is required for ALL transplantation services. It will be determined during the Plan’s prior authorization process if the specific transplant service is considered medically

Transplantation of Lung or Lobar Lung

+ Plan refers to Boston Medical Center Health Plan, Inc. and its affiliates and subsidiaries offering health coverage plans to enrolled members. The Plan operates in Massachusetts under the trade name Boston Medical Center HealthNet Plan and in other states under the trade name Well Sense Health Plan. 1 of 32

necessary for the requested indication within the Plan’s provider network, as appropriate. Heart-lung transplantation requires Plan Medical Director review.

Prior authorization requests for transplantation services for Plan members are evaluated utilizing medical necessity criteria in the applicable Plan medical policy. If there is no Plan medical policy for the requested type of transplantation (e.g., simultaneous pancreas and lung transplantation), the Plan uses InterQual® criteria to determine the medical necessity of the requested transplantation services. The Plan conducts an individual evaluation of the member’s medical condition based on the guidelines outlined in the Plan’s Clinical Review Criteria administrative policy, policy number OCA 3.201, when there is no applicable Plan medical policy and InterQual® criteria are not established for the requested type of transplantation. When a member is deemed to be an appropriate candidate for transplantation services based on the Plan’s applicable medical necessity criteria and the evaluation conducted by the treating provider, final approval is required by a Plan Medical Director for the member’s transplantation. In addition, Plan Medical Director review is required when the Plan’s applicable medical necessity criteria are not met for requested transplantation services.

The Plan’s Clinical Technology Evaluation administrative policy, policy number OCA 3.13, outlines the Plan’s process for evaluating new technology and the new application of existing technology. See the Plan’s Medically Necessary medical policy, policy number OCA 3.14, for the product-specific definitions of medically necessary treatment. Review the Plan’s Experimental and Investigational Treatment medical policy, policy number OCA 3.12, for the product-specific definitions of experimental or investigational treatment.

The Plan member must meet the eligibility criteria from the transplanting institution. The eligibility criteria of the transplanting institution must follow the applicable United Network for Organ Sharing (UNOS) guidelines. The hospital in which the organ transplants are performed must be a member of the Organ Procurement and Transplantation Network (OPTN) in accordance with the Public Health Service Act, comply with applicable OPTN organ allocation and procurement guidelines, and follow the Centers for Medicare & Medicaid Services (CMS) applicable conditions of participation for the specified organ to be transplanted (including but not limited to the following Code of Federal Regulations: 42 CFR Parts 405, 482, 488, and 498). The transplant program (including affiliated transplant facility, transplant surgeons, transplant physicians, and staff) must follow the designated UNOS/OPTN transplant program criteria for the applicable transplant service and comply with all applicable UNOS/OPTN professional standards. Senior Care Options members will have access to transplant services according all applicable CMS guidelines, including but not limited to the provisions specified in the Medicare Managed Care Manual, Chapter 4 – Benefits and Beneficiary Protections, 10.11 Transplant Services.

See the member’s applicable benefit documents available at www.bmchp.org for benefit coverage and associated transplant guidelines for a BMC HealthNet Plan member, at www.SeniorsGetMore.org for a Senior Care Options member, and at www.wellsense.org for a Well Sense Health Plan member. If a transplant is requested for a Well Sense Health Plan member and authorized by the Plan as medically necessary, a Plan-approved transplant center will review the case to determine the member’s status as Transplantation of Lung or Lobar Lung

a candidate for a transplant at that facility (based on the clinical guidelines utilized by the transplant program).

Description of Item or Service Lung Transplantation: The surgical replacement of lung from a deceased donor into a recipient. A lung transplant consists of replacing all or part of diseased lung(s) with healthy lung(s). A lung transplant refers to single-lung or double-lung replacement. In a single-lung transplant, only one (1) lung from a deceased donor is provided to the recipient. In a double-lung transplant, both of the recipient's lungs are removed and replaced by the donor's lungs.

Lobar Lung Transplantation: The surgical replacement of one (1) or both lungs in a recipient after removal of the right or left lower lung lobe from one (1) or two (2) donors (living donor or deceased donor). Lobar lung transplantation is usually reserved for pediatric patients who are not expected to survive the waiting time for a deceased donor transplant. 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 (1) lobe obtained from each of two (2) donors when bilateral transplantation is required. The living donor is left with four lung lobes, and the patient receives one of the donor’s lower lobes. When medically necessary, the recipient has both of the individual’s diseased lungs removed and receives two new lung lobes, one from each of two donors. There are also cases of cadaver lobe transplants.

Medical Policy Statement When a member is deemed an appropriate candidate for transplantation services based on the Plan’s applicable medical necessity criteria and the evaluation conducted by the treating provider, final approval is required by a Plan Medical Director for the member’s transplantation. In addition, Plan Medical Director review is required when the Plan’s applicable medical necessity criteria are not met for requested transplantation services. An evaluation for transplantation services conducted by the treating provider is defined as a consultation and diagnostic testing or other testing required to assess a member’s appropriateness and readiness for transplantation; an evaluation does not include care required as part of the course of treatment for the underlying medical condition. See the Policy Statement section of the Plan’s Transplant Administration policy, administrative policy number OCA 3.10, for guidelines on how the Plan processes requests for evaluations by participating providers and non-participating providers.

Lobar lung (from one [1] or two [2] donors) or lung transplantation (single-lung or double-lung replacement) is considered medically necessary when the medical record documentation supports that the member, as assessed by the transplant surgeon or a designee of the multidisciplinary transplant team, meets ALL of the following applicable criteria for transplant clearance, as specified below in item A (Initial Transplantation and Retransplantation Criteria for Adult and Pediatric Members) and item B (Disease-Specific Criteria for Adult and Pediatric Members):

Transplantation of Lung or Lobar Lung

A. Initial Transplantation and Retransplantation Criteria for Adult and Pediatric Members:

See applicable criteria below, EITHER item 1 for criteria for an initial transplantation or item 2 for criteria for retransplantation.

1. Initial Transplantation Criteria:

ALL of the following criteria must be met for an initial transplant for each adult member and pediatric member, as specified below in items a through m:

a. Diagnosis of totally irreversible chronic pulmonary disease; AND

b. Limited life expectancy of no more than two (2) years (based on the treating provider’s evaluation and clinical judgment); AND

c. Oxygen dependence; AND

d. Substantial limitation of daily activities; AND

e. Evaluation demonstrating absence of potential complications that could diminish the success of transplantation; AND

f. Acceptable nutritional status; AND

g. Good rehabilitation potential; AND

h. Compliance with medical management; AND

i. Satisfactory psychosocial profile and emotional support system; AND

j. Abstinence from smoking for at least six (6) months when history includes smoking; AND

k. Pre-surgical clearance by a cardiologist for the transplantation; AND

l. All the transplanting institution’s eligibility criteria are met; AND

m. The transplant meets ONE (1) of the following criteria, as specified below as item (1) or item (2):

(1) A deceased donor will be used for the lung transplant (single-lung or double-lung replacement) or lobar lung transplant; OR

Transplantation of Lung or Lobar Lung

(2) A living donor will be used rather than a deceased donor for lobar lung transplantation when the transplant team has determined that the member is a suitable candidate for a living donor transplant and at least ONE (1) of the following criteria are met, as specified below as item (a), item (b), or item (c):

(a) A deceased donor is unavailable; OR

(b) Member is deteriorating clinically to the point of transplant ineligibility while waiting for deceased donor organ donation; OR

2. Retransplantation Criteria:

Retransplantation is covered when BOTH of the following criteria are met, as specified below in item a and item b:

a. ALL criteria are met for the initial transplantation (as specified in item A1 above, Initial Transplantation Criteria); AND

b. The member has at least ONE (1) of the following indications, as specified below in item (1), item (2), or item (3):

(1) Graft failure of an initial lung or lobular lung transplant due to EITHER of the following, as specified below as item (a) or item (b):

(a) Technical reason, excluding serious reportable event and/or provider- preventable condition; † OR

† Note: See the Plan’s Provider Preventable Conditions and Serious Reportable Events reimbursement policy (policy number 4.610 for BMC HealthNet Plan members, policy number SCO 4.610 for Senior Care Options members, and policy number WS 4.29 for Well Sense Health Plan members) for definitions of serious reportable events and provider-preventable conditions.

(b) Hyperacute rejection (see Definitions section); OR

(2) Chronic rejection (see Definitions section); OR

(3) Recurrent disease; AND

Transplantation of Lung or Lobar Lung

B. Disease-Specific Criteria for Adult and Pediatric Members:

1. ALL applicable transplant criteria are met for EITHER an initial transplantation (as specified above in item A1) or retransplantation (as specified above in item A2); AND

2. The member has at least ONE (1) of the following diseases and ALL applicable criteria are met for that disease, as specified below in items a through e:

a. Bronchiectatic Disease:

Includes but is not limited to cystic fibrosis, acquired bronchiectasis, or congenital bronchiectasis with ALL of the following clinical indications, as specified below in items (1) through (4):

(1) Forced expiratory volume in 1 second (FEV1) ≤ 30% of predicted value or rapid respiratory deterioration with FEV1 >30%; AND

(2) paCO2 > 50 mm Hg; AND

(3) paO2 < 55 mm Hg; AND

(4) Increasing frequency and severity of exacerbations; OR

b. Nonbronchiectatic Disease:

Includes but is not limited to COPD, emphysema, alpha-1 antitrypsin disease, bronchiolitis obliterans syndrome (BOS), or chronic bronchitis with ALL of the following clinical indications, as specified below in items (1) through (5):

(1) FEV1 < 25% of the predicted value; AND

(2) paCO2 ≥ 55 mm Hg; AND

(3) paO2 < 55-60 mm Hg; AND

(4) Elevated pulmonary artery pressures (secondary pulmonary hypertension); AND

(5) Clinical course - rapid rate of decline in FEV1 or life-threatening exacerbations; OR

Transplantation of Lung or Lobar Lung

c. Interstitial Lung Disease:

Includes but is not limited to idiopathic pulmonary fibrosis (IPF), interstitial pulmonary fibrosis, sarcoidosis, scleroderma, lymphangiomyomatosis, eosinophilic granuloma, pneumoconiosis or other lung disease due to external agents such as asbestos, crystalline silica, organic coal dust, or histiocytosis X with ALL of the following clinical indications, as specified below in items (1) through (5):

(1) Symptomatic, progressive disease with failure to respond to optimal medical treatment; AND

(2) Vital capacity < 60% to 65% of predicted value; AND

(3) Diffusing capacity of the lung for carbon monoxide (DLCO) < 50% to 60% of predicted value; AND

(4) Resting hypoxemia with PaO2 < 55 mm Hg; AND

(5) Rapid progression of IPF warrants early referral; OR

d. Pulmonary Hypertension:

Includes but is not limited to primary or secondary due to cardiac disease, idiopathic pulmonary hypertension, pulmonary emboli, or Eisenmenger’s syndrome with ALL of the following clinical indications, as specified below in items (1) through (5):

(1) New York Heart Association functional class III or IV; AND

(2) Mean right atrial pressure of greater than 10 mm Hg; AND

(3) Mean pulmonary arterial pressure of greater than 50 mm Hg; AND

(4) Cardiac index of less than 2.5 L/min/m2; AND

(5) Failure of therapy with long-term prostacyclin infusion; OR

e. Untreatable End-Stage Pulmonary Disease of Any Etiology

Transplantation of Lung or Lobar Lung

Limitations All transplant-related consultations, evaluations, procedures, and post-transplant follow-up services should be managed within the Plan’s provider network or at the most appropriate preferred transplant facility (depending upon the type of transplant) and according to the administrative guidelines specified in the Plan’s Transplant Administration policy, administrative policy number OCA 3.10. Prior authorization is required for ALL transplantation services.

1. Heart-Lung Transplantation:

Plan Medical Director review is required for each prior authorization request for a heart-lung transplantation. For the Plan Medical Director to determine the medical necessity of the procedure, the treating provider must submit medical record documentation that supports the member’s diagnosis, past medical history (including previous medical, surgical, and pharmacological interventions and corresponding clinical outcomes), diagnostic test results, clinical indications for a heart-lung transplant, and documentation that the member has been assessed by the transplant surgeon or a designee of the multidisciplinary transplant and is considered an appropriate candidate for a heart-lung transplant, and documentation that the member has met the applicable criteria at the transplant center where the procedure is expected to be performed. Candidates for heart-lung transplantation are individuals with end- stage cardiopulmonary failure not amenable to conventional medical therapy or surgical repair; causes of end-stage cardiopulmonary failure may include irreparable congenital cardiac anomalies with pulmonary hypertension (Eisenmenger complex), primary pulmonary hypertension with irreversible right-heart failure, or sarcoidosis involving only the heart and lungs. The age of 60 years is the conventional upper limit for most candidates; however, transplant centers with more experience may evaluate patients older than 60 years on an individual basis.

Transplantation of Lung or Lobar Lung

2. The Plan considers ANY of the following services experimental and investigational, as specified below in items a through c:

a. Lung transplantation for an individual with coronary artery disease not amenable to percutaneous intervention or bypass grafting, or an individual with coronary artery disease associated with significant impairment of left ventricular function is considered experimental and investigational.

b. Lung xenotransplantation (e.g., porcine xenografts) is considered experimental and investigational for any indication.

c. Prophylactic anti-reflux surgery to improve lung function and survival in lung transplant recipient without gastroesophageal reflux disease is considered experimental and investigational.

See the Plan’s Experimental and Investigational Treatment medical policy, policy number OCA 3.12, for the product-specific definitions of experimental or investigational treatment.

3. Contraindications to lung transplantation include but are not limited to ANY of the following, as specified below in item a. and/or item b:

a. Absolute contraindications, where there is no reasonable circumstance for undertaking transplant surgery, include at least ONE (1) of the following, as specified below in item 1 or item 2:

(1) Immunosuppressed or potentially exacerbated by immunosuppression with at least ONE (1) of the following conditions, as specified below in items (a) through (h):

(a) Known active malignancy, including metastatic cancer, other than non- melanomatous skin cancer; OR

(b) Recently treated malignancy within two (2) years of curative treatment without evidence of recurrence (within five [5] years for breast cancer, colorectal cancer, or melanoma); this absolute contraindication does NOT include an early stage cancer in which the cancerous growth or tumor is still confined to the site from which it started, and has not spread to surrounding tissue or other organs in the body (i.e., carcinoma in situ, cancer in situ, preinvasive carcinoma, in situ lesions);** OR

** Note: The assessment of risk recurrence must be determined by the transplant team; the transplant team must then submit a written statement Transplantation of Lung or Lobar Lung

to the Plan explaining why the member with a recently treated malignancy or a member with a moderate or high risk of recurrence is an appropriate candidate for transplant surgery.

(d) AIDS (diagnosis based on CDC definition of CD4 count, 200cells/mm3) unless ALL of the following are noted in the member’s medical record, as specified below in items i through iv:

i. CD4 count >200cells/mm3 for more than 6 months; AND

ii. HIV-1 RNA undetectable; AND

iii. On stable anti-retroviral therapy more than 3 months; AND

iv. No other complications from AIDS (e.g., opportunistic infection, including aspergillus, tuberculosis, coccidioide-mycosis, resistant fungal infections, Kaposi's sarcoma or other neoplasm); OR

(e) Complicated or uncontrolled diabetes mellitus; OR

(f) Hepatitis B virus antigen positive (surface, core or both) based on criteria established by the transplantation center; OR

(g) Cirrhosis; OR

(h) Significant infection present outside the lungs and outside the upper respiratory tract; OR

(2) The member has at least ONE (1) of the following other conditions specified below in items (a) through (q):

(a) Active substance abuse or active smoking within the last six (6) months (including drug, alcohol, or tobacco); OR

(b) Inability to adhere to the therapeutic regimen necessary to preserve the transplant, including but not limited to compliance with the prescribed medication regimen, monitoring for signs and symptoms of complications, avoidance of risk factors that may result in adverse clinical outcomes, and/or attendance at regular clinical checkups; OR

Transplantation of Lung or Lobar Lung

(d) Active/symptomatic coronary artery disease with any ONE (1) of the following, as specified below in items i through iii:

i. Not amendable to percutaneous intervention or bypass grafting; OR

ii. Associated with significant impairment of left ventricular function; OR

iii. Without cardiac clearance for transplantation; OR

(e) Active systemic collagen vascular (connective tissue) disease; OR

(f) Body mass index greater than 35kg/m2; OR

(g) Cardiac insufficiency - right or left ventricular ejection fraction less than 20 percent; OR

(h) Demonstrated patient noncompliance which would place the organ at risk by not adhering to medical recommendations; OR

(i) Non-rehabilitative pulmonary disability; OR

(j) Donor recipient incompatible as proven by positive cross match testing; OR

(k) Member lack of acceptance of potential complications from immunosuppressive medications; OR

(l) Significantly impaired hepatic function characterized by persistent and marked elevation of international normalized ratio (INR) or severe liver dysfunction caused by antituberculous therapy; OR

(m) Surgically remediable chronic thromboembolic disease; OR

(n) For a pediatric member (less than age 18 on the date of service), dysfunction of major organs other than the lung, particularly renal dysfunction with creatinine clearance of < 50, because of the impact of immunosuppressive drugs on renal function (and multiple-organ transplant may be considered as an alternative, when clinically appropriate); OR

(o) For an adult member (age 18 or older on date of service), extrapulmonary end- stage organ disease (and multiple-organ transplant may be considered as an alternative, when clinically appropriate); OR

(p) Active mycobacterium tuberculosis; OR Transplantation of Lung or Lobar Lung

(q) Member is receiving high dose steroid therapy (more than 40 mg daily) that cannot be tapered or discontinued.

b. Relative contraindications are listed below items (1) through (8). Any ONE (1) of these contraindications puts the member at a higher risk of complications; this risk may be outweighed by other medical considerations and therefore transplant surgery may be considered after Medical Director review and approval if other Plan criteria are met:

(1) Body mass index (BMI) less than 17 kg/m2 or BMI 30 kg/m2 or higher;† OR

† Note: This Plan policy also includes an absolute contraindication of BMI greater than 35 kg/m2

(2) Dependence on mechanical ventilation with clinically unstable pulmonary function tests; OR

(3) Age greater than 65 years old on the date of service for lobar lung transplant, single lung transplant, or double lung transplant; OR

(4) Significant chest wall or spinal deformity; OR

(5) Previous pleurodesis, pleurectomy, or complicated cardiothoracic surgery (excluding simple pneumothorax treated with closed tube thoracostomy, open lung biopsy, or uncomplicated lobectomy) which increases the technical difficulty of extracting the native lung and the operative risk of lung transplantation; OR

(6) Presence of significant esophageal dysfunction with ineffective esophageal motility that is likely to cause chronic rejection manifested as bronchiolitis obliterans syndrome, as determined by the treating provider or transplant surgeon; OR

(7) For a pediatric member (less than age 18 on the date of service), severe musculoskeletal disease affecting the thorax (e.g., kyphoscoliosis) and progressive neuromuscular disease; OR

(8) Member with another medical condition that may cause end-organ damage (such has systemic hypertensions, epilepsy, central venous obstruction, peptic ulcer disease, or gastroesophageal reflux) when the condition is not optimally treated before transplantation.

Transplantation of Lung or Lobar Lung

Definitions Carcinoma In Situ: A group of abnormal cells that remain in the place where they first formed and have not spread. These abnormal cells may become cancer and spread into nearby normal tissue and is also called stage 0 disease.

Cardiac Index: A cardiodynamic measure based on the cardiac output, which is the amount of blood the left ventricle ejects into the systemic circulation in one minute, measured in liters per minute (l/min). Cardiac output is indexed to a patient's body size by dividing by the body surface area to yield the cardiac index.

Collagen Vascular Disease (CVD): A diverse group of autoimmune disorders (i.e., the body is allergic to itself) including: systemic lupus erythematosus, rheumatoid arthritis, progressive systemic sclerosis, dermatomyositis /polymyositis, ankylosing spondylitis, Sjögren syndrome, or mixed connective tissue diseases. Many CVDs involve the lungs either directly or as a complication of treatment of the CVD. Many parts of the respiratory system may be involved, including the airways, blood vessels, lung tissue, the tissue lining around the lungs and chest cavity, and/or respiratory muscles.

Double Lung Transplant (Bilateral): The surgical replacement of both lungs in the recipient by both lungs from a deceased donor. In general, this procedure may be appropriate for any ONE (1) of the following conditions, as specified below in items 1 through 3:

1. End-stage chronic obstructive pulmonary disease: Emphysema

2. Infectious lung disease:

a. Bronchiectasis

b. Cystic fibrosis

3. Pulmonary vascular disease:

a. Eisenmenger’s syndrome with cardiac repair

b. Primary pulmonary hypertension

Lung Allocation Score (LAS): The United Network for Organ Sharing (UNOS) has developed a system for prioritizing patients for lung transplants called the Lung Allocation Score System or LAS. The LAS system prioritizes lung transplant candidates age 12 and older for donor lung offers by assigning them a score from 0 to 100. The score is based on each patient's individual medical information that reflects both the seriousness of each patient's medical condition before transplant and the likelihood of success after a transplant; the score includes patient diagnosis, comorbidities, laboratory values, test results, and other clinical measures (including forced vital capacity, pulmonary artery and capillary

Transplantation of Lung or Lobar Lung

wedge pressures, oxygen at rest, age, body mass index, functional status, six minute walk, serum creatinine, and if assisted ventilation is required). The candidate with the highest LAS in a particular age group will receive first priority for a donor lung offer.

New York Heart Association (NYHA) Functional Class: Functional classification system of heart failure that categorizes a patient’s condition and cardiovascular disability based on severity of symptoms and limitations during physical activity, as specified below in items 1 through 4:

1. NYHA Functional Class I (Mild): Patient with cardiac disease without limitation of physical activity. Ordinary physical activity does not cause undue fatigue, palpitation, or dyspnea.

2. NYHA Functional Class II (Mild): Patient with cardiac disease with slight limitation of physical activity. Comfortable at rest, but ordinary physical activity results in fatigue, palpitation, or dyspnea.

3. NYHA Functional Class III (Moderate): Patient with cardiac disease producing marked limitation of physical activity. Comfortable at rest; less than ordinary physical activity causes fatigue, palpitation, dyspnea, or anginal pain.

4. NYHA Functional Class IV (Severe): Patient with cardiac disease resulting in inability to carry out any physical activity without discomfort. Symptoms of cardiac insufficiency or angina are present at rest; symptoms are increased with physical activity.

Single Lung Transplant (Unilateral): The surgical removal of a single lung from a deceased donor or living donor to replace one (1) lung in the recipient. In general, single lung transplantation may be appropriate for at least ONE (1) of the following conditions, as specified below in items 1 through 3:

1. Chronic obstructive pulmonary disease:

Emphysema with or without alpha1-antitrypsin deficiency; OR

2. Interstitial lung disease:

a. Eosinophilic granuloma; OR

b. Idiopathic interstitial pulmonary fibrosis; OR

c. Sarcoidosis; OR

d. Lymphangioleiomyomatosis; OR

Transplantation of Lung or Lobar Lung

3. Pulmonary vascular disease:

a. Eisenmenger's syndrome with cardiac repair; OR

b. Primary pulmonary hypertension

Transplant Rejection: A process in which a transplant recipient's immune system attacks the transplanted organ or tissue. There are three (3) clinicopathologic stages of rejection:

1. Hyperacute Rejection: A recipient’s immune reaction that occurs within a few minutes after the transplant when the antigens are completely unmatched, resulting in organ failure within the first hours after transplantation. The tissue must be removed right away so the recipient does not die.

2. Acute Rejection: A recipient’s immune reaction that occurs any time from the first week after the transplant (during which the immune response increases in intensity) and generally up to 60 to 90 days after organ transplantation. It may be Grade I (mild), Grade II (moderate) or Grade III (severe). All recipients have some amount of acute rejection.

3. Chronic Rejection: A recipient’s immune reaction that occurs more than 60 days after transplantation and can take place over many years. This is the body's constant immune response against the new organ that slowly damages the transplanted tissues or organ.

Xenotransplantation: According to the U.S. Public Health Service, xenotransplantation is defined as any procedure that involves the transplantation, implantation, or infusion into a human recipient of either of the following, as specified below in item 1 or item 2:

1. Live cells, tissues, or organs from a non-human animal source; OR

2. Human body fluids, cells, tissues or organs that have had ex vivo contact with live non-human animal cells, tissues, or organs. (See this policy’s Limitations section.)

Applicable Coding The Plan uses and adopts up-to-date Current Procedural Terminology (CPT) codes from the American Medical Association (AMA), International Statistical Classification of Diseases and Related Health Problems, 10th revision (ICD-10) diagnosis codes developed by the World Health Organization and adapted in the United Stated by the National Center for Health Statistics (NCHS) of the Centers for Disease Control under the U.S. Department of Health and Human Services, and the Health Care Common Procedure Coding System (HCPCS) established and maintained by the Centers for Medicare & Medicaid Services (CMS). Because the AMA, NCHS, and CMS may update codes more frequently or at different intervals than Plan policy updates, the list of applicable codes included in this Plan policy is for informational purposes only, may not be all inclusive, and is subject to change without prior Transplantation of Lung or Lobar Lung

notification. Whether a code is listed in the Applicable Coding section of this Plan policy does not constitute or imply member coverage or provider reimbursement. Providers are responsible for reporting all services using the most up-to-date industry-standard procedure and diagnosis codes as published by the AMA, NCHS, and CMS at the time of the service.

Providers are responsible for obtaining prior authorization for the services specified in the Medical Policy Statement section and Limitation section of this Plan policy, even if an applicable code appropriately describing the service that is the subject of this Plan policy is not included in the Applicable Coding section of this Plan policy. Coverage for services is subject to benefit eligibility under the member’s benefit plan. Please refer to the member’s benefits document in effect at the time of the service to determine coverage or non-coverage as it applies to an individual member. See Plan reimbursement policies for Plan billing guidelines.

CPT Codes Description: Codes Covered When Medically Necessary 32851 Lung transplant, single; without cardiopulmonary bypass 32852 Lung transplant, single; with cardiopulmonary bypass 32853 Lung transplant, double (bilateral sequential or en bloc); without cardiopulmonary bypass 32854 Lung transplant, double (bilateral sequential or en bloc); with cardiopulmonary bypass HCPCS Codes Description: Codes Covered When Medically Necessary S2060 Lobar lung transplantation

Plan note: This code is not payable for the Senior Care Options product. S2061 Donor lobectomy (lung) for transplantation, living donor

Plan note: This code is not payable for the Senior Care Options product.

Clinical Background Information Lung transplantation may be appropriate for patients with advanced lung disease whose clinical status has progressively declined despite maximal medical or surgical therapy with a limited life expectancy over the next two (2) years. Candidates are usually symptomatic during activities of daily living and ideally should be free of any other organ dysfunction or medical problem that would substantially jeopardize the outcome of transplantation. (Source: Hachem RR. UpToDate®). Lung transplantation has become a viable treatment option for selected patients with end-stage lung disease due to a wide variety of underlying disorders. Single, double, and lobar-lung transplantation have all been performed successfully. The type of lung transplantation procedure used (i.e., lobar, single, or double) and donor type (i.e., deceased or living) are based upon the candidate's condition and indication for transplantation, and the availability of donor organs. As donor organs are scarce relative to the number of candidates needing transplantation, conservation of acceptable donor organs is also taken into consideration. Deceased donor lung transplantation, also known as cadaveric donor lung

Transplantation of Lung or Lobar Lung

transplantation, is most commonly performed. Individuals older than age 12 are allocated donated deceased donor lungs using the Lung Allocation Score based on survival benefit and medical condition. Organ allocation to children under age 12 is based solely on waiting time.

Living donor lobar lung transplantation has shown success and addresses the shortage of deceased donor organs. Living donor lobar lung transplantation is less commonly performed. A transplant from living donors usually involves three operations, one on each of two donors and one on the recipient. The lower lobe of the right lung is removed from one donor and the lower lobe of the left lung is removed from the other donor. Both lungs are then removed from the recipient and are replaced by the lung implants from the donors in a single operation. Although deceased donor lung transplantation is preferred to avoid risk to two healthy donors, living donor lobar lung transplant may be an acceptable alternative when the recipient (usually a child or adolescent) is not likely to survive long enough to receive deceased donor lungs.

The goal of lung transplantation is to improve quality of life and long-term survival in patients with end-stage pulmonary disease. Advances in donor and recipient selection, new immunosuppressive medications, new and improved surgical techniques, and increased medical management of infections have improved the overall survival in patients after lung transplantation. Consideration for lung transplantation is based on an evaluation for potential complications that could diminish its success.

Lung transplantation is an effective treatment for end stage lung disease. However, long-term survival is limited by the development of chronic rejection manifested as bronchiolitis obliterans syndrome (BOS). GER and altered motility has long been associated with lung disease and are common among patients and the post lung transplant population. The evidence collected to date strongly supports a role for the aspiration of gastric contents as a causative or additive etiology to developing BOS. Common in advanced lung disease, changes in diaphragmatic position, decreased lower esophageal sphincter pressure, and changes in intrathoracic pressure are proposed mechanisms favoring reflux. Ineffective esophageal motility (IEM) is the most common motility disorder in patients with GER- associated respiratory symptoms. Aspiration secondary to GER and altered foregut motility have been identified as potential contributors to lung allograft dysfunction. Lung transplant recipients appear to be at increased risk of GER and aspiration through the following mechanisms: (1) The cough reflexes and mucociliary clearance which are the normal defense mechanisms against aspiration are dramatically impaired; (2) mucociliary clearance is less than normal in transplanted lungs (measured to be less than 15% of normal); and (3) It is hypothesized that even small amounts of aspiration can lead to significant injury, particularly with multiple repeated episodes over time (with a causative or additive etiology to develop BOS).

The U.S. Department of Health and Human Services (DHHS) has oversight responsibility for the organ allocation system in the United States. Congress established the Organ Procurement and Transplantation Network (OPTN) when it enacted the National Organ Transplant Act (NOTA) of 1984. The Act called for a unified transplant network to be operated by a private, nonprofit organization

Transplantation of Lung or Lobar Lung

under federal contract. United Network for Organ Sharing (UNOS) was awarded the initial OPTN contract in 1986 and continues to administer the OPTN.

At the time of the Plan’s most recent policy review, no national coverage determination (NCD) or local coverage determination (LCD) was found from the Centers for Medicare & Medicaid Services (CMS) for single, double, and/or lobar lung transplantation. CMS requires that services be performed at a Medicare-approved facility for lung transplant services (as specified in 42 CFR Parts 405, 482, 488, and 498 Medicare Program, Hospital Conditions of Participation: Requirements for Approval and Re- Approval of Transplant Centers to Perform Organ Transplants, Final Rule, March 30, 2007). CMS evaluates detailed criteria for facility participation that include but are not limited to the following: Clinical experience, patient selection of suitable candidates, patient management with good patient outcomes, experience and survival rates, maintenance of data, organ procurement, laboratory services, and billing guidelines. Senior Care Options members will have access to transplant services according all applicable CMS guidelines, including but not limited to the provisions specified in the Medicare Managed Care Manual, Chapter 4 – Benefits and Beneficiary Protections, 10.11 Transplant Services. Verify applicable CMS criteria are in effect for lung transplant services in an NCD or LCD on the date of the prior authorization request for a Senior Care Options member.

References Ahmad U, Wang Z, Bryant AS, Kim AW, Kukreja J, Mason DP, Bermudez CA, Detterbeck FC, Boffa DJ. Outcomes for lung transplantation for lung cancer in the United Network for Organ Sharing Registry. Ann Thorac Surg. 2012 Sep;94(3):935-40; discussion 940-1. doi: 10.1016/j.athoracsur.2012.04.069. Epub 2012 Jul 25. PMID: 22835555.

Allen JG, Arnaoutakis GJ, Weiss ES, Merlo CA, Conte JV, Shah AS. The impact of recipient body mass index on survival after lung transplantation. J Heart Lung Transplant. 2010 Sep;29(9):1026-33. doi: 10.1016/j.healun.2010.05.005. Epub 2010 Jun 16. PMID: 20558085.

Allen JG, Weiss ES, Merlo CA, Baumgartner WA, Conte JV, Shah AS. Impact of donor-recipient race matching on survival after lung transplantation: analysis of over 11,000 patients. J Heart Lung Transplant. 2009 Oct;28(10):1063-71. doi: 10.1016/j.healun.2009.06.012. PMID: 19782288.

American College of Cardiology (ACC), American Heart Association (AHA), Heart Failure Society of America (HFSA). Yancy CW, Jessup M, Bozkurt B, Butler J, Casey DE Jr, Colvin MM, Drazner MH, Filippatos GS, Fonarow GC, Givertz MM, Hollenberg SM, Lindenfeld J, Masoudi FA, McBride PE, Peterson PN, Stevenson LW, Westlake C. 2017 ACC/AHA/HFSA Focused Update of the 2013 ACCF/AHA Guideline for the Management of Heart Failure: A Report of the ACC/AHA Task Force on Clinical Practice Guidelines and the HFSA. J Am Coll Cardiol. 2017;Apr 28;70(6):776-803. doi: 10.1016/j.jacc.2017.04.025. Accessed at: http://www.onlinejacc.org/content/70/6/776?_ga=2.140394931.1068379667.1520950858- 108633920.1512161961

Transplantation of Lung or Lobar Lung

American Society of Transplantation (AST). Guidelines and Opinions. Heart and Lung. Accessed at: https://www.myast.org/education/guidelines-and-opinions#

American Society of Transplantation (AST). Guidelines and Opinions. Infectious Disease. Accessed at: https://www.myast.org/education/guidelines-and-opinions#

American Society of Transplantation (AST). Key Position Statements. Accessed at: https://www.myast.org/public-policy/key-position-statements/key-position-statements

Beaty CA, George TJ, Kilic A, Conte JV, Shah AS. Pre-Transplant Malignancy: An Analysis of Outcomes after Thoracic organ Transplantation. J Heart Lung Transplant. 2013 Feb;32(2):202-11. Published online 2012 Dec 21. doi: 10.1016/j.healun.2012.11.003. PMCID: PMC412890e.

Belli EV, Landolfo K, Keller C, Thomas M, Odell J. Lung cancer following lung transplant: single institution 10 year experience. Lung Cancer. 2013 Sep;81(3):451-4. doi: 10.1016/j.lungcan.2013.05.018. Epub 2013 Jul 5. PMID: 23831244.

Benden C. Pediatric lung transplantation. J Thorac Dis. 2017 Aug;9(8):2675–83. doi: 10.21037/jtd.2017.07.84. PMCID: PMC5594176.

Benden C, Ridout DA, Edwards LB, Boehler A, Christie JD, Sweet SC. Body mass index and its effect on outcome in children after lung transplantation. J Heart Lung Transplant. 2013 Feb;32(2):196-201. doi: 10.1016/j.healun.2012.11.002. Epub 2012 Nov 28. PMID: 23200225.

Bosanquet JP, Witt CA, Bemiss BC, Byers DE, Yusen RD, Patterson AG, Kreisel D, Mohanakumar T, Trulock EP, Hachem RR. The impact of pre-transplant allosensitization on outcomes after lung transplantation. J Heart Lung Transplant. 2015 Nov;34(11):1415-22. doi: 10.1016/j.healun.2015.06.003. Epub 2015 Jun 10. PMID: 26169666.

Brennan DC, Rodeheffer RJ, Ambinder RF. Development of malignancy following solid organ transplantation. UpToDate. 2016 May 9. Accessed at: https://www.uptodate.com/contents/development-of-malignancy-following-solid-organ- transplantation

Canadian Society of Transplantation (CST). Reference Documents. Accessed at: http://www.cst- transplant.ca/resources-for-health-professionals.html

Castleberry A, Mulvihill MS, Yerokun BA, Gulack BC, Englum B, Snyder L, Worni M, Osho A, Palmer S, Davis RD, Hartwig MG. The utility of 6-minute walk distance in predicting waitlist mortality for lung transplant candidates. J Heart Lung Transplant. 2017 Jul;36(7):780–6. Published online 2016 Dec 30. doi: 10.1016/j.healun.2016.12.015. PMCID: PMC5495471.

Transplantation of Lung or Lobar Lung

Castleberry AW, Martin JT, Osho AA, Hartwig MG, Hashmi ZA, Zanotti G, Shaw LK, Williams JB, Lin SS, Davis RD. Coronary revascularization in lung transplant recipients with concomitant coronary artery disease. Am J Transplant. 2013 Nov;13(11):2978-88. doi: 10.1111/ajt.12435. Epub 2013 Sep 18. PMID: 24102830.

Castor JM, Wood RK, Muir AJ, Palmer SM, Shimpi RA. Gastroesophageal Reflux and Altered Motility in Lung Transplant Rejection. Neurogastroenterol Motil. 2010 Aug;22(8):841–850. Published online 2010 May 26. doi: 10.1111/j.1365-2982.2010.01522.x. PMCID: PMC2911519.

Cedars-Sinai. Inclusion and Exclusion Criteria. Lung Transplantation. Accessed at: https://www.cedars- sinai.edu/Patients/Programs-and-Services/Lung-Transplant-Center/For-Physicians/When-to- Refer/Inclusion-and-Exclusion-Criteria.aspx

Centers for Disease Control and Prevention (CDC). Transplant Safety. Guidelines. Accessed at: http://www.cdc.gov/transplantsafety/hc-providers/guidelines.html

Centers for Medicare & Medicaid Services (CMS). Conditions for Coverage (CfCs) & Conditions of Participations (CoPs). Transplant Centers. Accessed at: https://www.cms.gov/Regulations-and- Guidance/Legislation/CFCsAndCoPs/transplantcenters.html

Centers for Medicare & Medicaid Services (CMS). Medicare Approved Transplant Programs. Program Types: Approved Heart-Only Programs, Approved Intestinal and/or Multivisceral Programs, Approved Kidney-Only Programs, Approved Liver Programs, Approved Lung-Only Programs, and Approved Pancreas (Pancreas-Only and Combined Kidney/Pancreas) Programs. 2018 Jan 16. Accessed at: https://www.cms.gov/Medicare/Provider-Enrollment-and- Certification/CertificationandComplianc/Downloads/ApprovedTransplantPrograms.pdf

Centers for Medicare & Medicaid Services (CMS). Medicare Managed Care Manual. Chapter 4 – Benefits and Beneficiary Protections. 10.11 Transplant Services. Rev 121. Issued 2016 Apr 22. Accessed at: https://www.cms.gov/Regulations-and-Guidance/Guidance/Manuals/downloads/mc86c04.pdf

Centers for Medicare & Medicaid Services (CMS). Quality, Safety & Oversight – Certification & Compliance. Transplant. Accessed at: https://www.cms.gov/Medicare/Provider-Enrollment-and- Certification/CertificationandComplianc/Transplant.html

Centers for Medicare & Medicaid Services (CMS). Welcome to the Medicare Coverage Database. Accessed at: https://www.cms.gov/medicare-coverage-database/overview-and-quick-search.aspx

Chandrashekaran S, Keller CA, Kremers WK, Peters SG, Hathcock MA, Kennedy CC. Weight Loss Prior to Lung Transplantation is Associated with Improved Survival. J Heart Lung Transplant. 2015 May;34(5):651–7. Published online 2014 Nov 17. doi: 10.1016/j.healun.2014.11.018. PMCID: PMC4417392.

Transplantation of Lung or Lobar Lung

Chan EY, Goodarzi A, Sinha N, Nguyen DT, Youssef JG, Suarez EE, Kaleekal T, Graviss EA, Bruckner BA, MacGillivray TE, Scheinin SA. Long-Term Survival in Bilateral Lung Transplantation for Scleroderma- Related Lung Disease. Ann Thorac Surg. 2018 Mar;105(3):893-900. doi: 10.1016/j.athoracsur.2017.09.038. Epub 2018 Feb 1. PMID: 29394994.

Chapman JR, Webster AC, Wong G. Cancer in the Transplant Recipient. Cold Spring Harb Perspect Med. 2013 Jul;3(7):a015677. doi: 10.1101/cshperspect.a015677. PMCID: PMC3685882.

Commonwealth of Massachusetts. MassHealth Guidelines for Medical Necessity Determination. Organ Transplant Procedures. Executive Office of the Health and Human Services. Accessed at: https://www.mass.gov/guides/masshealth-guidelines-for-medical-necessity-determination-for-organ- transplant-procedures

Cooper DKC. The Case for Xenotransplantation. Clin Transplant. 2015 Apr; 29(4): 288–293. Published online 2015 Feb 28. doi: 10.1111/ctr.12522. PMCID: PMC4402124.

Date H, Sato M, Aoyama A, Yamada T, Mizota T, Kinoshita H, Handa T, Tanizawa K, Chin K, Minakata K, Chen F. Living-donor lobar lung transplantation provides similar survival to cadaveric lung transplantation even for very ill patients. Eur J Cardiothorac Surg. 2015 Jun;47(6):967-72; discussion 972-3. doi: 10.1093/ejcts/ezu350. Epub 2014 Sep 16. PMID: 25228745.

Davis CS, Shankaran V, Kovacs EJ, Gagermeier J, Dilling D, Alex CG, Love RB, Sinacore J, Fisichella PM. Gastroesophageal reflux disease after lung transplantation: pathophysiology and implications for treatment. Surgery. 2010 Oct;148(4):737-44; discussion 744-5. doi: 10.1016/j.surg.2010.07.011. Epub 2010 Aug 21. PMID: 20727564.

Eberlein M, Reed RM, Chahla M, Bolukbas S, Blevins A, Van Raemdonck D, Stanzi A, Inci I, MarascoS, Shigemura N, Aigner C, Deuse T. Lobar lung transplantation from deceased donors: A systematic review. World J Transplant. 2017 Feb 24;7(1):70–80. Published online 2017 Feb 24. doi: 10.5500/wjt.v7.i1.70. PMCID: PMC5324031.

Egan TM, Edwards LB. Effect of the lung allocation score on lung transplantation in the United States. J Heart Lung Transplant. 2016 Apr;35(4):433-9. doi: 10.1016/j.healun.2016.01.010. Epub 2016 Jan 15. PMID: 26922274.

European Society of Cardiology (ESC), European Respiratory Society (ERS), International Society of Heart and Lung transplantation (ISHLT). Galiè N, Hoeper MM, Humbert M, Torbicki A, Vachiery JL, Barbera JA, Beghetti M, Corris P, Gaine S, Gibbs JS, Gomez-Sanchez MA, Jondeau G, Klepetko W, Opitz C, Peacock A, Rubin L, Zellweger M, Simonneau G; ESC Committee for Practice Guidelines (CPG). Guidelines for the diagnosis and treatment of pulmonary hypertension: The task force for the diagnosis and treatment of pulmonary hypertension of the ESC and the ERS, endorsed by the ISHLT.Eur Heart J. 2009 Oct;30(20):2493-537. doi: 10.1093/eurheartj/ehp297. Epub 2009 Aug 27. PMID: 19713419.

Transplantation of Lung or Lobar Lung

Hadjiliadis D, Steele MP, Chaparro C, Singer LG, Waddell TK, Hutcheon MA, Davis RD, Tullis DE, Palmer SM, Keshavjee S. Survival of lung transplant patients with cystic fibrosis harboring panresistant bacteria other than Burkholderia cepacia, compared with patients harboring sensitive bacteria. J Heart Lung Transplant. 2007 Aug;26(8):834-8. Epub 2007 Jul 12. PMID: 17692788.

Hachem R. Lung transplantation: General guidelines for recipient selection. UpToDate. 2018 Jan 25. Accessed at: https://www.uptodate.com/contents/lung-transplantation-general-guidelines-for- recipient-selection

Hathorn KE, Chan WW, Lo WK. Role of gastroesophageal reflux disease in lung transplantation. World J Transplant. 2017 Apr 24;7(2):103-116. doi: 10.5500/wjt.v7.i2.103. PMID: 28507913.

Hayanga AJ, Aboagye J, Esper S, Shigemura N, Bermudez CA, D'Cunha J, Bhama JK. Extracorporeal membrane oxygenation as a bridge to lung transplantation in the United States: an evolving strategy in the management of rapidly advancing pulmonary disease. J Thorac Cardiovasc Surg. 2015 Jan;149(1):291-6. doi: 10.1016/j.jtcvs.2014.08.072. Epub 2014 Sep 17. PMID: 25524684

Hayanga JA, Lira A, Vlahu T, Yang J, Aboagye JK, Hayanga HK, Luketich JD, D’Cunha J. Lung Transplantation in Patients with High Lung Allocation Scores in the US: Evidence for the Need to Evaluate Score Specific Outcomes. J Transplant. 2015;2015: 836751. Published online 2015 Dec 21. doi: 10.1155/2015/836751. PMCID: PMC4698782.

Hayes Prognosis Overview. Organ Care System (OCS) Lung. Winifred Hayes, Inc. 2017 May.

Hook JL, Lederer DJ. Selecting lung transplant candidates: where do current guidelines fall short? Expert Rev Respir Med. 2012 Feb;6(1):51–61. doi: 10.1586/ers.11.83. PMCID: PMC3286653.

Huddleston CB. Lung transplantation for pulmonary hypertension in children. Pediatr Crit Care Med. 2010 Mar;11(2 Suppl):S53-6. doi: 10.1097/PCC.0b013e3181c8b697. PMID: 20216165.

Hryhorowicz M, Zeyland J, Słomski R, Lipiński D. Genetically Modified Pigs as Organ Donors for Xenotransplantation. Mol Biotechnol. 2017 Oct;59(9-10):435-444. doi: 10.1007/s12033-017-0024-9. PMID: 28698981.

The International Society for Heart & Lung Transplantation (ISHLT). International Thoracic Organ Transplant (TTX) Registry. Accessed at: https://www.ishlt.org/registries/heartLungRegistry.asp

The International Society for Heart & Lung Transplantation (ISHLT). ISHLT Standards Statements, Guidelines, and Consensus Documents. Accessed at: https://www.ishlt.org/guidelines/standardsStatements.asp

Transplantation of Lung or Lobar Lung

The International Society for Heart and Lung Transplantation (ISHLT). Lund LH, Khush KK, Cherikh WS, Goldfarb S, Kucheryavaya AY, Levvey BJ, Meiser B, Rossano JW, Chambers DC, Yusen RD, Stehlik J. The Registry of the International Society for Heart and Lung Transplantation: Thirty-fourth Adult Heart Transplantation Report—2017; Focus Theme: Allograft ischemic time. J Heart Lung Transplant. 2017 Oct;36(10):1037-46.

The International Society for Heart & Lung Transplantation (ISHLT). Registries – Heart/Lung Registries. Quarterly Data Report. Accessed at: https://www.ishlt.org/registries/quarterlyDataReport.asp

The International Society for Heart and Lung Transplantation (ISHLT). Yusen RD, Christie JD, Edwards LB, Kucheryavaya AY, Benden C, Dipchand AI, Dobbels F, Kirk R, Lund LH, Rahmel AO, Stehlik J; ISHLT. The Registry of the International Society for Heart and Lung Transplantation: Thirtieth Adult Lung and Heart-Lung Transplant Report--2013; focus theme: age. J Heart Lung Transplant. 2013 Oct;32(10):965- 78. doi: 10.1016/j.healun.2013.08.007. PMID: 24054805.

The International Society for Heart and Lung Transplantation (ISHLT). Yusen RD, Edwards LB, Dipchand AI, Goldfarb SB, Kucheryavaya AY, Levvey BJ, Lund LH, Meiser B, Rossano JW, Stehlik J. The Registry of the International Society for Heart and Lung Transplantation: Thirty-third Adult Lung and Heart–Lung Transplant Report—2016; Focus Theme: Primary Diagnostic Indications for Transplant. J Heart Lung Transplant. 2016 Oct;35(10):1170-84.

Kachala SS, Murthy SC. Lung transplantation for multifocal lung adenocarcinoma (multifocal lung carcinoma). Thorac Surg Clin. 2014 Nov;24(4):485-91. doi: 10.1016/j.thorsurg.2014.07.011. Epub 2014 Oct 23. PMID: 25441143.

Khan MS, Zhang W, Taylor RA, Dean McKenzie E, Mallory GB, Schecter MG, Morales DL, Heinle JS, Adachi I. Survival in pediatric lung transplantation: The effect of center volume and expertise. J Heart Lung Transplant. 2015 Aug;34(8):1073-81. doi: 10.1016/j.healun.2015.03.008. Epub 2015 Mar 25. PMID: 26023035.

Kilic A, Merlo CA, Conte JV, Shah AS. Lung transplantation in patients 70 years old or older: have outcomes changed after implementation of the lung allocation score? J Thorac Cardiovasc Surg. 2012 Nov;144(5):1133-8. doi: 10.1016/j.jtcvs.2012.07.080. Epub 2012 Aug 31. PMID: 22944081.

Kirkby S, Hayes D Jr. Pediatric lung transplantation: indications and outcomes. J Thorac Dis. 2014 Aug;6(8):1024–31. doi: 10.3978/j.issn.2072-1439.2014.04.27. PMCID: PMC4133536.

Kistler KD, Nalysnyk L, Rotella P, Esser D. Lung transplantation in idiopathic pulmonary fibrosis: a systematic review of the literature. BMC Pulm Med. 2014;14:139. Published online 2014 Aug 16. doi: 10.1186/1471-2466-14-139. PMCID: PMC4151866.

Transplantation of Lung or Lobar Lung

Lane CR, Tonelli AR. Lung transplantation in chronic obstructive pulmonary disease: patient selection and special considerations. Int J Chron Obstruct Pulmon Dis. 2015;10:2137–46. Published online 2015 Oct 9. doi: 10.2147/COPD.S78677. PMCID: PMC4608618.

Lederer DJ, Kawut SM, Wickersham N, Winterbottom C, Bhorade S, Palmer SM, Lee J, Diamond JM, Wille KM, Weinacker A, Lama VN, Crespo M, Orens JB, Sonett JR, Arcasoy SM, Ware LB, Christie JD; Lung Tansplant Oucomes Group. Obesity and Primary Graft Dysfunction after Lung Transplantation: the Lung Transplant Outcomes Group Obesity Study. Am J Respir Crit Care Med. 2011 Nov 1; 184(9):1055-61. Published online 2011 Nov 1. doi: 10.1164/rccm.201104-0728OC. PMCID: PMC3208644.

Lehr CJ, Zaas DW, Cheifetz IM, Turner DA. Ambulatory extracorporeal membrane oxygenation as a bridge to lung transplantation: walking while waiting. Chest. 2015 May;147(5):1213-8. doi: 10.1378/chest.14-2188. PMID: 25940249.

Mancini MC. Heart-Lung Transplantation. Medscape. 2017 Jun 7. Accessed at: https://emedicine.medscape.com/article/429188-overview

MedicineNet.com. Definition of Cardiac Index. 2017 Jan 1. Accessed at: http://www.medterms.com/script/main/art.asp?articlekey=39852

Medline Plus. Lung Transplantation. 2018 Mar 7. Accessed at: https://medlineplus.gov/lungtransplantation.html

MedlinePlus. Transplant Rejection. 2018 Mar 5. Accessed at: https://medlineplus.gov/ency/article/000815.htm

National Cancer Institute (NCI). National Institutes of Health. NCI Dictonary of Cancer Terms. Carcinoma In Situ. Accessed at: https://www.cancer.gov/publications/dictionaries/cancer- terms/def/carcinoma-in-situ

National Institute for Health and Clinical Excellence (NICE). Living-donor lung transplantation for end- stage lung disease. Interventional Procedure Guidance (IPG) 170. 2006 May. Accessed at: https://www.nice.org.uk/guidance/ipg170

Organ Procurement and Transplantation Network (OPTN). Allocation Calculators. Calculated Panel Reactive Antibody (CPRA) calculator to evaluate candidates for kidney, pancreas, and kidney/pancreas transplants; Estimated Post Transplant Survival (EPTS) score for adult candidates for kidney allocation system; Kidney Donor Profile Index (KDPI) calculator summarizes risk of graft failure after kidney transplant; Lung Allocation Score (LAS) to allocate lungs to candidates age 12 and older; Model for End- Stage Liver Disease (MELD) and Pediatric End-Stage Liver Disease (PELD) calculators used for liver allocation. Accessed at: https://optn.transplant.hrsa.gov/resources/allocation-calculators/

Transplantation of Lung or Lobar Lung

Organ Procurement and Transplantation Network (OPTN). Organ Procurement and Transplantation Network. Accessed at: https://optn.transplant.hrsa.gov/

Organ Procurement and Transplantation Network (OPTN). Policies. 2018 Mar 1. Accessed at: https://optn.transplant.hrsa.gov/governance/policies/

Organ Procurement and Transplantation Network (OPTN) and United Network for Organ Sharing (UNOS). OPTN/UNOS Ethics Committee. Ethical Principles in the Allocation of Human Organs. 2015 Jun 2. Accessed at: https://optn.transplant.hrsa.gov/resources/ethics/ethical-principles-in-the-allocation-of-human- organs/

Organ Procurement and Transplantation Network (OPTN) and United Network for Organ Sharing (UNOS). OPTN/UNOS Membership and Professional Standards Committee. OPTN/UNOS Public Comment Proposal. Consider Primary Transplant Surgeon Requirement- Primary or First Assistant on Transplant Cases. 2016. Accessed at: https://optn.transplant.hrsa.gov/media/1927/mpsc_primary_surgeon_txcases_20160815.pdf

Organ Procurement and Transplantation Network (OPTN) and United Network for Organ Sharing (UNOS). OPTN/UNOS Membership and Professional Standards Committee. OPTN/UNOS Public Comment Proposal. Updating Primary Kidney Transplant Physician Requirements. 2016. Accessed at: https://optn.transplant.hrsa.gov/media/1931/mpsc_update_kiphys_reqs_20160815.pdf

Organ Procurement and Transplantation Network (OPTN). View Data Reports. Accessed at: https://optn.transplant.hrsa.gov/data/view-data-reports/

Patel N, DeCamp M, Criner GJ. Lung transplantation and lung volume reduction surgery versus transplantation in chronic obstructive pulmonary disease. Proc Am Thorac Soc. 2008 May 1;5(4):447- 53. doi: 10.1513/pats.200707-107ET. PMCID: PMC2645318.

Pedraza I. Interstitial Lung Disease Associated With Collagen-Vascular Disease. Medscape. 2016 Aug 12. Accessed at: http://emedicine.medscape.com/article/1343513-overview

Plantier L, Skhiri N, Biondi G, Jebrak G, Himbert D, Castier Y, Lesèche G, Mal H, Thabut G, Fournier M. Impact of previous cardiovascular disease on the outcome of lung transplantation. J Heart Lung Transplant. 2010 Nov;29(11):1270-6. doi: 10.1016/j.healun.2010.05.010. Epub 2010 Jul 1. PMID: 20580260.

Schaffer JM, Singh SK, Reitz BA, Zamanian RT, Mallidi HR. Single- vs double-lung transplantation in patients with chronic obstructive pulmonary disease and idiopathic pulmonary fibrosis since the implementation of lung allocation based on medical need. JAMA. 2015 Mar 3;313(9):936-48. doi: 10.1001/jama.2015.1175. PMID: 25734735.

Transplantation of Lung or Lobar Lung

Scientific Registry of Transplant Recipients (SRTR) and Organ Procurement and Transplantation Network (OPTN). The SRTR/OPTN Annual Data Report. Statistics on Donation and Transplantation in the United States. Current Report and Archived Reports. Accessed at: https://www.srtr.org/reports- tools/srtroptn-annual-data-report/

Shah P, Orens JB. Impact of nutritional state on lung transplant outcomes: The weight of the evidence. J Heart Lung Transplant. 2013 Aug;32(8):755-6. doi: 10.1016/j.healun.2013.06.001. PMID: 23856213.

Singer JP, Chen H, Phelan T, Kukreja J, Golden JA, Blanc PD. Survival following lung transplantation for silicosis and other occupational lung diseases. Occup Med (Lond). 2012 Mar;62(2):134–7. Published online 2011 Nov 9. doi: 10.1093/occmed/kqr171. PMCID: PMC3283165.

Thabut G, Mal H. Outcomes after lung transplantation. J Thorac Dis. 2017 Aug;9(8):2684-91. doi: 10.21037/jtd.2017.07.85. PMID: 28932576.

Tomaszek SC, Fibla JJ, Dierkhising RA, Scott JP, Shen KR, Wigle DA, Cassivi SD. Outcome of lung transplantation in elderly recipients. Eur J Cardiothorac Surg. 2011 May;39(5):726-31. doi: 10.1016/j.ejcts.2010.08.034. Epub 2010 Nov 16. PMID: 21084198.

Toyoda Y, Toyoda Y. Heart-lung transplantation: adult indications and outcomes. J Thorac Dis. 2014 Aug;6(8):1138–42. doi: 10.3978/j.issn.2072-1439.2014.06.01. PMCID: PMC4133545.

United Network for Organ Sharing (UNOS). Bylaws. Policies and Membership Requirements for Transplant Programs and Personnel. 2015 Feb 1. Accessed at: https://www.unos.org/wp- content/uploads/unos/UNOS_Bylaws.pdf

United Network for Organ Sharing (UNOS). Data. Accessed at: https://unos.org/data/

United Network for Organ Sharing (UNOS). Facts and Figures. 2017 May 19. Accessed at: https://www.unos.org/wp-content/uploads/unos/UNOS_FactsFigures.pdf

U.S. Food and Drug Administration. Vaccines, Blood & Biologics. Xenotransplantation. 2018 Feb 5. Accessed at: https://www.fda.gov/BiologicsBloodVaccines/Xenotransplantation/

U.S. Food and Drug Administration. Vaccines, Blood & Biologics. Xenotransplantation. Information and Recommendations for Physicians Involved in the Co-Culture of Human Embryos with Non-Human Animal Cells. 2017 May 16. Accessed at: http://www.fda.gov/BiologicsBloodVaccines/Xenotransplantation/ucm136532.htm

Valour F, Brault C, Abbas-Chorfa F, Martin C, Kessler L, Kanaan R, Mosnier-Pudar H, Coltey B, Nove- Josserand R, Durupt S, Colin C, Durieu I. Outcome of cystic fibrosis-related diabetes two years after lung transplantation. Respiration. 2013;86(1):32-8. doi: 10.1159/000339928. Epub 2012 Aug 25. PMID: 22922226. Transplantation of Lung or Lobar Lung

Van Raemdonck D, Vos R, Yserbyt J, Decaluwe H, De Leyn P, Verleden GM. Lung cancer: a rare indication for, but frequent complication after lung transplantation. J Thorac Dis. 2016 Nov; 8(Suppl 11): S915–24. doi: 10.21037/jtd.2016.11.05. PMCID: PMC5124593.

Vermeijden JW, Zijlstra JG, Erasmus ME, van der Bij W, Verschuuren EA. Lung transplantation for ventilator-dependent respiratory failure. J Heart Lung Transplant. 2009 Apr;28(4):347-51. doi: 10.1016/j.healun.2009.01.012. PMID: 19332261.

Whitson BA, Hayes D Jr. Indications and outcomes in adult lung transplantation. J Thorac Dis. 2014 Aug;6(8):1018-23. doi: 10.3978/j.issn.2072-1439.2014.07.04. PMCID: PMC4133539.

Wigfield CH, Buie V, Onsager D. “Age” in lung transplantation: factors related to outcomes and other considerations. Curr Pulmonol Rep. 2016;5:152–8. Published online 2016 Aug 13. doi: 10.1007/s13665- 016-0151-y. PMCID: PMC4992499.

Yang SM, Huang SC, Kuo SW, Huang PM, Pan SC, Lee JM, Lai HS, Hsu HH. Long-term outcome after bilateral lung transplantation - a retrospective study from a low-volume center experience. BMC Surg. 2015 Mar 18;15:28. doi: 10.1186/s12893-015-0010-8. PMID: 25880739.

Yusen RD, Shearon TH, Qian Y, Kotloff R, Barr ML, Sweet S, Dyke DB, Murray S. Lung transplantation in the United States, 1999-2008. Am J Transplant. 2010 Apr;10(4 Pt 2):1047-68. doi: 10.1111/j.1600- 6143.2010.03055.x. PMID: 20420652.

Original Effective Original Approval Original Policy Date* and Version Policy Owner Date Approved by Number Regulatory Approval: N/A 10/02/05 Medical Policy Manager Quality and Clinical Version 1 as Chair of Medical Policy, Management Committee Internal Approval: Criteria, and Technology (Q&CMC) 08/02/05 Assessment Committee (MPCTAC) *Effective Date for the BMC HealthNet Plan Commercial Product(s): 01/01/12 *Effective Date of the Well Sense Health Plan New Hampshire Medicaid Product(s): 01/01/13 *Effective Date for Senior Care Options Product(s): 01/01/16

Policy formerly titled Lung Transplant until 07/31/13.

Transplantation of Lung or Lobar Lung

Policy Revisions History Revision Effective Date Review Date Summary of Revisions Approved by and Version Number 02/06/07 Updated template and references. Version 2 02/06/07: Q&CMC

02/19/08 Revised clinical criteria. Version 3 02/19/08: MPCTAC 02/26/08: Utilization Management Committee (UMC) 03/12/08: QIC 02/24/09 Updated clinical criteria for HIV. Version 4 02/24/09: MPCTAC Updated coding and references. 02/24/09: UMC 03/25/09: QIC 02/01/10 Updated references. Version 5 02/22/10: MPCTAC 03/24/10: QIC 03/01/11 Updated references. Updated the Version 6 03/16/11: MPCTAC clinical guideline statement and the 04/27/11: QIC medically appropriate indications with additional criteria. Updated the contraindications. 03/12/12 Updated references and clinical Version 7 03/21/12: MPCTAC guideline statement. Updated and 04/25/12: QIC clarified contraindications and added relative contraindications. 08/01/12 Off cycle review for Well Sense Health Version 8 08/13/12: MPCTAC Plan. Reformatted Medical Policy 09/06/12: QIC Statement, revised Applicable Coding introduction, updated code list, and revised Limitations section. 04/01/13 Review for effective date 08/01/13. 08/01/13 04/17/13: MPCTAC Revised title, removed redundant text Version 9 05/16/13: QIC in Clinical Background Information section, revised Summary and Description of Item or Service sections, added clinical criteria and limitations, revised and added definitions, updated language in Applicable Coding section and revised applicable code list, added text to Clinical Background Information section, and added and updated references. Referenced Medically Necessary policy, Experimental and Investigational Treatment policy, and Transplantation of Lung or Lobar Lung

Policy Revisions History Reimbursement Guidelines: Serious Reportable Event/Provider Preventable Condition. 03/01/14 Review for effective date 07/01/14. 07/01/14 03/19/14: MPCTAC Updated Summary and References Version 10 04/16/14: QIC sections. Revised criteria in the Medical Policy Statement section and the Limitations section. 03/01/15 Review for effective 05/01/15. 05/01/15 03/18/15: MPCTAC Updated Summary and References Version 11 04/08/15: QIC sections. Removed Commonwealth Care, Commonwealth Choice, and Employer Choice from the list of applicable products because the products are no longer available. 11/25/15 Review for effective date 01/01/16. 01/01/16 11/18/15: MPCTAC Updated template with list of Version 12 11/25/15: MPCTAC applicable products and notes. (electronic vote) Revised language in the Applicable 12/09/15: QIC Coding section. 03/01/16 Review for effective date 07/01/16. 07/01/16 03/16/16: MPCTAC Updated Summary, Definitions, Clinical Version 13 04/13/16: QIC Background Information, References, and References to Applicable Laws and Regulations sections. Administrative changes made to the Medical Policy Statement section. Revised criteria in the Limitations section. 03/01/17 Review for effective date 06/07/17. 06/07/17 03/15/17: MPCTAC Updated Summary, Definitions, Clinical Version 14 Background Information, and References sections. Revised criteria in the Medical Policy Statement and Limitations sections. Plan notes added to the Applicable Coding section. 03/01/18 Review for effective date 04/01/18. 04/01/18 03/21/18: MPCTAC Administrative changes made to the Version 15 Medical Policy Statement and Limitations sections. Updated the Policy Summary, References, and Other Applicable Policies sections.

Transplantation of Lung or Lobar Lung

Last Review Date 03/01/18

Next Review Date 03/01/19

Authorizing Entity MPCTAC

Other Applicable Policies Administrative Policy - Clinical Review Criteria, policy number OCA 3.201 Administrative Policy - Clinical Technology Evaluation, policy number OCA 3.13 Administrative Policy - Transplantation Administration, policy number OCA 3.10 Medical Policy - Experimental and Investigational Treatment, policy number OCA 3.12 Medical Policy - Medical Nutrition Therapy in the Outpatient or Office Setting, policy number OCA 3.66 Medical Policy - Medically Necessary, policy number OCA 3.14 Medical Policy - Transplantation of Pancreas or Pancreas-Kidney, policy number OCA 3.25 Medical Policy - Transplantation of Small Bowel, Small Bowel-Liver, or Multivisceral Organs, policy number OCA 3.26 Reimbursement Policy - Anesthesia, policy number 4.103 Reimbursement Policy - Anesthesia, policy number SCO 4.103 Reimbursement Policy - Anesthesia, policy number WS 4.11 Reimbursement Policy - Bilateral and Multiple Procedure Reductions, policy number 4.607 Reimbursement Policy - Bilateral and Multiple Procedure Reductions, policy number SCO 4.607 Reimbursement Policy - Diabetes Self-Management Training (DSMT)/Medical Nutrition Therapy (MNT), policy number 4.32 Reimbursement Policy - Diabetes Self-Management Training (DSMT)/Medical Nutrition Therapy (MNT), policy number WS 4.32 Reimbursement Policy - General Billing and Coding Guidelines, policy number 4.31 Reimbursement Policy - General Billing and Coding Guidelines, policy number SCO 4.114 Reimbursement Policy - General Billing and Coding Guidelines, policy number WS 4.17 Reimbursement Policy - General Clinical Editing and Payment Accuracy Review Guidelines, policy number 4.108 Reimbursement Policy - General Clinical Editing and Payment Accuracy Review Guidelines, policy SCO 4.31 Reimbursement Policy - General Clinical Editing and Payment Accuracy Review Guidelines, policy number WS 4.18 Reimbursement Policy - Hospital, policy number WS 4.21 Reimbursement Policy - Inpatient Hospital, policy number 4.110 Reimbursement Policy - Inpatient Hospital, policy number SCO 4.110 Transplantation of Lung or Lobar Lung

Reimbursement Policy - Outpatient Hospital, policy number 4.17 Reimbursement Policy - Outpatient Hospital, policy number SCO 4.17 Reimbursement Policy - Physician and Non-Physician Practitioner Services, policy number 4.608 Reimbursement Policy - Physician and Non-Physician Practitioner Services, policy number SCO 4.608 Reimbursement Policy - Physician and Non-Physician Practitioner Services, policy number WS 4.28 Reimbursement Policy - Professional Bilateral and Multiple Procedure Reductions, policy number WS 4.24 Reimbursement Policy - Provider Preventable Conditions and Serious Reportable Events, policy number 4.610 Reimbursement Policy - Provider Preventable Conditions and Serious Reportable Events, policy number SCO 4.610 Reimbursement Policy - Provider Preventable Conditions and Serious Reportable Events, policy number WS 4.29

Reference to Applicable Laws and Regulations 42 CFR Parts 405, 482, 488, and 498. Code of Federal Regulations. Centers for Medicare & Medicaid Services (CMS). Medicare Program. Hospital Conditions of Participation: Requirements for Approval and Re-Approval of Transplant Centers to Perform Organ Transplants. Final Rule. March 30, 2007. Accessed at: https://www.cms.gov/Medicare/Provider-Enrollment-and- Certification/GuidanceforLawsAndRegulations/Downloads/TransplantFinalLawandReg.pdf

42 U.S. Code § 274. United States Code. Public Health Service (PHS) Act. Organ Procurement and Transplantation Network. Accessed at: https://www.law.cornell.edu/uscode/text/42/274

78 FR 48164-69. Federal Register. Centers for Medicare & Medicaid Services (CMS). Medicare Program. Revised Process for Making National Coverage Determinations. August 7, 2013. Accessed at: https://www.cms.gov/Medicare/Coverage/DeterminationProcess/Downloads/FR08072013.pdf

130 CMR 415.400. Commonwealth of Massachusetts. Code of Massachusetts Regulations. Division of Medical Assistance. MassHealth Provider Manual Series. Acute Inpatient Hospital Manual. Transmittal Letter AIH-52. January 2, 2015. Accessed at: http://www.mass.gov/eohhs/docs/masshealth/regs- provider/regs-acuteinpathosp.pdf

130 CMR 433.000. Commonwealth of Massachusetts. Code of Massachusetts Regulations. Division of Medical Assistance. MassHealth Provider Manual Series. Physician Manual. Transmittal Letter PHY-140. January 1, 2014. Accessed at: http://www.mass.gov/eohhs/docs/masshealth/regs-provider/regs- physician.pdf

130 CMR 450.000. Commonwealth of Massachusetts. Code of Massachusetts Regulations. Division of Medical Assistance. Administrative and Billing Regulations. Accessed at: http://www.mass.gov/courts/docs/lawlib/116-130cmr/130cmr450.pdf

Transplantation of Lung or Lobar Lung

Centers of Medicare & Medicaid Services (CMS). State Operations Manual. Appendix A - Survey Protocol, Regulations and Interpretive Guidelines for Hospitals. Rev. 151. 11-20-15. Accessed at: https://www.cms.gov/Regulations-and- Guidance/Guidance/Manuals/Downloads/som107ap_a_hospitals.pdf

Chapter He-W 500 Medical Assistance. New Hampshire Code of Administrative Rules. Accessed at: http://www.gencourt.state.nh.us/rules/state_agencies/he-w500.html

New Hampshire Medicaid. Hospitals, Hospital-Based Rural Health Clinics (RHC-HB), and Swing Bed Hospitals.* Provider Manual. Volume II. December 2014.

Disclaimer Information: + Medical Policies are the Plan’s guidelines for determining the medical necessity of certain services or supplies for purposes of determining coverage. These Policies may also describe when a service or supply is considered experimental or investigational, or cosmetic. In making coverage decisions, the Plan uses these guidelines and other Plan Policies, as well as the Member’s benefit document, and when appropriate, coordinates with the Member’s health care Providers to consider the individual Member’s health care needs. Plan Policies are developed in accordance with applicable state and federal laws and regulations, and accrediting organization standards (including NCQA). Medical Policies are also developed, as appropriate, with consideration of the medical necessity definitions in various Plan products, review of current literature, consultation with practicing Providers in the Plan’s service area who are medical experts in the particular field, and adherence to FDA and other government agency policies. Applicable state or federal mandates, as well as the Member’s benefit document, take precedence over these guidelines. Policies are reviewed and updated on an annual basis, or more frequently as needed. Treating providers are solely responsible for the medical advice and treatment of Members. The use of this Policy is neither a guarantee of payment nor a final prediction of how a specific claim(s) will be adjudicated. Reimbursement is based on many factors, including member eligibility and benefits on the date of service; medical necessity; utilization management guidelines (when applicable); coordination of benefits; adherence with applicable Plan policies and procedures; clinical coding criteria; claim editing logic; and the applicable Plan – Provider agreement.

Transplantation of Lung or Lobar Lung