LUNG TRANSPLANTATION

Transcription

1 LUNG TRANSPLANTATION Policy Number: 2015M0047A Effective Date: July 1, 2015 Table of Contents: Page: Cross Reference Policy: POLICY DESCRIPTION 2 Not Available COVERAGE RATIONALE/CLINICAL CONSIDERATIONS 2 BACKGROUND 4 REGULATORY STATUS 7 CLINICAL EVIDENCE 8 APPLICABLE CODES 9 REFERENCES 11 POLICY HISTORY/REVISION INFORMATION 13 INSTRUCTIONS: Medical Policy assists in administering UCare benefits when making coverage determinations for members under our health benefit plans. When deciding coverage, all reviewers must first identify enrollee eligibility, federal and state legislation or regulatory guidance regarding benefit mandates, and the member specific Evidence of Coverage (EOC) document must be referenced prior to using the medical policies. In the event of a conflict, the enrollee's specific benefit document and federal and state legislation and regulatory guidance supersede this Medical Policy. In the absence of benefit mandates or regulatory guidance that govern the service, procedure or treatment, or when the member s EOC document is silent or not specific, medical policies help to clarify which healthcare services may or may not be covered. This Medical Policy is provided for informational purposes and does not constitute medical advice. In addition to medical policies, UCare also uses tools developed by third parties, such as the InterQual Guidelines, to assist us in administering health benefits. The InterQual Guidelines are intended to be used in connection with the independent professional medical judgment of a qualified health care provider and do not constitute the practice of medicine or medical advice. Other Policies and Coverage Determination Guidelines may also apply. UCare reserves the right, in its sole discretion, to modify its Policies and Guidelines as necessary and to provide benefits otherwise excluded by medical policies when necessitated by operational considerations. Page. 1 of 13

2 POLICY DESCRIPTION: Lung transplantation is a surgical procedure to replace one or both diseased lungs with a healthy lung or lungs from a cadaveric or living donor. The purpose of the procedure is to extend the life expectancy and enhance the quality of life for patients with end-stage lung disease who have no other medical or surgical treatment options. In a lobar transplant, a lobe of the donor's lung is excised, sized appropriately for the recipient's thoracic dimensions and is transplanted. Donors for lobar transplant have been primarily living related donors, with one lobe obtained from each of two donors (e.g. mother and father). In situations where a bilateral transplant is required, there are also cases of cadaver lobe transplants. COVERAGE RATIONALE / CLINICAL CONSIDERATIONS: Single lung, double lung, and/or lobar transplantation are considered MEDICALLY NECESSARY for patients with irreversible, progressively disabling, end-stage lung disease who are failing maximal medical therapy, or for whom no effective medical therapy exists. Potential candidates should be well informed and demonstrate adequate health behavior and a willingness to adhere to the health care transplanting institution's selection criteria. Specific indications include the conditions listed below, but not limited to: Alpha-1 antitrypsin deficiency Primary pulmonary hypertension Cystic fibrosis Bronchopulmonary dysplasia Lymphangiomyomatosis- with end-stage pulmonary disease Recurrent pulmonary embolism End-stage pulmonary vascular disease including: primary pulmonary hypertension, Eisenmengers syndrome with correctable intra-cardiac defect and reversible RV dysfunction. Graft-versus-host disease or failed primary lung graft Obstructive lung disease (e.g., Bilateral bronchiectasis, bronchiolitis obliterans, chronic obstructive pulmonary disease, emphysema) Restrictive lung disease (e.g., interstitial pulmonary fibrosis, Idiopathic pulmonary fibrosis, sarcoidosis, systemic sclerosis or scleroderma, eosinophilic granuloma or histiocytosis X, collagen vascular disease, occupational lung disease, alveolar proteinosis) Indications For Retransplantation: Failed previous lung transplantation including, but not limited to one of the following: o Non-function or failure of the grafted organ o Rejection refractory to immunosuppressive therapy o Bronchiolitis obliterans (chronic rejection) o Airway complications not correctable by other measures The above Medical Necessity Criteria for initial transplantation must be met, and No history of behaviors since the previous transplant that would jeopardize a subsequent transplant Page. 2 of 13

3 Lung xenotransplantation (e.g., porcine xenografts) is considered EXPERIMENTAL AND/OR INVESTIGATIONAL for any pulmonary conditions because of insufficient evidence in the peer-reviewed literature. Clinical Considerations: All patients should meet the institution s criteria for acceptable cardiac, kidney and liver function. The Lung Allocation Score (LAS) is used to place patients on the lung waiting list. This is similar to the Model for End-Stage Liver Disease (MELD) system for liver transplantation. The LAS takes into account the severity of the illness pre-transplant including the likelihood of death on the waiting list and the likelihood of survival one year post-transplant. Waiting time on the list is no longer an important criterion. Additional studies to determine the patient s suitability for transplant may be requested if indicated, including, but not limited to: o Coronary angiography, echocardiogram or Multi Gated Acquisition Scan (MUGA) o Serology testing including, but not limited to, hepatitis A, B and C, human immunodeficiency virus (HIV), cytomegalovirus (CMV), varicella-zoster (VZV), Epstein Barr virus (EBV), herpes virus, serology testing for syphilis o Psychosocial evaluation and clearance o Mammogram for women if indicated or greater than age 40 o Pelvic exam with Pap smear for women if indicated or greater than age 18 o Dental exam with completion of required dental work prior to transplant o Imaging studies o Colonoscopy for persons over age 50 with removal of any polyps o Immunizations up to date when indicated: Hepatitis A, Hepatitis B, VZV, Tetanus-Diphtheria- Pertussis (Tdap), meningococcal, influenza and pneumonia o Carotid Doppler ultrasound for individuals with known coronary artery disease or greater than age 50 o Screening for peripheral artery disease (PAD) when indicated or greater than age 50. o Ophthalmology exam for persons with diabetes (as baseline) Patient/guardian and family/social support system are able to comply with the treatment regimen and the necessary follow-up. Inadequate funding to pay for immunosuppressive medications posttransplant are addressed and resolved. For patients with a recent history (24 months) of alcohol or other drug abuse, successful completion of chemical dependency assessment and 6 months of documented ongoing abstinence. Contraindications (subject to the judgment of the transplant center): In general, transplantation is contraindicated in the presence of any non-pulmonary medical condition that would itself significantly shorten life expectancy or make transplant success unlikely. Known current malignancy, including metastatic cancer Recent malignancy with high risk of recurrence Untreated systemic infection making immunosuppression unsafe, including chronic infection Irreversible multisystem organ failure Other irreversible end-stage disease not attributed to lung disease History of cancer with a moderate risk of recurrence Page. 3 of 13

4 Systemic disease that could be exacerbated by immunosuppression Inadequately controlled HIV/AIDS infection, defined as CD4 counts less than 200/mm 3, detectable HIV- 1 RNA (viral load), and/or presence of other complications from AIDS, such as opportunistic infection (e.g., aspergillus, tuberculosis, coccidioidomycosis, resistant fungal infections) or neoplasms (e.g., Kaposi's sarcoma, non-hodgkin's lymphoma) Psychosocial conditions or active chemical dependency affecting ability to adhere to therapy Coronary artery disease (CAD) not amenable to percutaneous intervention or bypass grafting, or associated with significant impairment of left ventricular function Colonization with highly resistant or highly virulent bacteria, fungi, or mycobacteria Irreversible severe brain damage Lung Specific: Decisions must often be made about whether to replace one or both lungs. The choice of single or double lung transplantation is a clinical decision that is left to the treating physicians Bilateral lung transplantation is typically required when chronic lung infection disease is present, e.g., associated with cystic fibrosis, chronic bronchitis, and bronchiectasis. Some, but not all, cases of pulmonary hypertension, alpha 1 antitrypsin deficiency, obstructive and/or rectrictive lung disease 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 BACKGROUND: Lung transplantation is a surgical procedure to replace one or both diseased lungs with a healthy lung or lungs from a donor. The purpose of the procedure is to extend the life expectancy and enhance the quality of life (QOL) for patients with end-stage lung disease. Since the early 1990s, more than 25,000 lung transplants have been performed at centers around the world. However, the number of donor organs available remains far fewer than the number of patients with end-stage lung disease who might potentially benefit from the procedure. The procedure has been modified and refined over the years, accompanied by advancements in the field of organ preservation and immunosuppression, and that has led to improvement in outcomes after lung transplantation. Subtle modifications have been made to exposure techniques, choice of suture materials and anastomotic techniques as experience has accumulated. Traditionally, survival has been the main focus of outcomes assessment in lung transplantation. Until 2006, guidelines for the selection of lung transplant candidates were based on the recommendations for the timing of referral for transplant and the timing of transplantation on survival estimation. In May 2005, the Organ Procurement and Transplantation Network (OPTN) changed the policy for lung allocation for transplantation in the United States from a system that allocated donor lungs based primarily on waiting time to a system that allocated lungs based primarily on a lung allocation score (LAS). The LAS system prioritizes access to transplant for patients with high-predicted survival benefit and short-predicted waiting list survival (Egan et al., 2006). The LAS takes into account various measures of a patient s health in order to direct donated organs towards the patients who would best benefit from a lung transplant. It reflects both the seriousness of each candidate s medical status before transplant and the likelihood of a successful transplant. The LAS is an important factor in determining the priority for receiving a lung transplant when a Page. 4 of 13

5 donor lung becomes available. The system continues to be evaluated and revised. Lung transplantation is associated with a significant degree of postoperative mortality and morbidity, which did not change after implementation of LAS. Graft failure and infections cause many of the deaths in the early posttransplantation period, while chronic rejection and infections cause many of the late posttransplantation deaths. Moreover, lung transplant recipients may suffer from morbidity associated with transplantation and immunosuppression. A common serious complication is the development of bronchiolitis obliterans syndrome (BOS), which affects more than 50% of long-term lung transplantation survivors and accounts for 57% of the deaths after 1 year. Lung transplantation cannot be assessed as an isolated surgical procedure. To assess the efficacy of lung transplantation, survival, patient reported outcomes, and QOL measurements need to be included. Factors that need to be considered include the underlying lung disease, the surgical protocol, and the allocation procedure. LAS Primary Diagnostic Groupings for Lung Transplant Candidates LAS Lung Disease Diagnostic Grouping Diagnoses Group A (obstructive lung disease) Group B (pulmonary vascular disease) Group C (CF or immunodeficiency disorders) Group D (restrictive lung disease) COPD w/ or w/o alpha 1-antitrypsin deficiency (α1ad) due to chronic bronchitis and/or emphysema lymphangioleiomyomatosis (LAM) Bronchiectasis, including primary ciliary dyskinesia Sarcoidosis w/ a mean pulmonary artery pressure (PAP) 30 mm Hg Idiopathic pulmonary arterial hypertension (iph), formerly known as primary pulmonary hypertension (PPH) Eisenmenger syndrome Other pulmonary vascular diseases Cystic fibrosis (CF) Immunodeficiency disorders such as hypogammaglobulinemia Idiopathic pulmonary fibrosis (ipf) Pulmonary fibrosis due to other causes Sarcoidosis w/ a mean PAP >30 mm Hg Obliterative bronchiolitis (nonretransplant) The implementation of the LAS had a significant impact on lung transplantation. The number of active wait listed lung candidates declined 54% from the pre-las levels to the levels at the end of There was also a reduction in median waiting-time, from 792 days in 2004 to 141 days in Waiting list mortality was reduced, and there was a shift in the predominant diagnosis group receiving transplantation. Idiopathic pulmonary fibrosis became the most common diagnosis group to receive a lung transplant in 2007 (i.e., 23.9% in 2004 versus 33.1% in 2007), while emphysema was the most common diagnosis in previous years (i.e., 36.8% in 2004 versus 29.6% in 2007). Despite candidates with increasingly higher LAS scores being transplanted in the LAS era, recipient death rates remained relatively stable since The number of retransplants and transplants performed yearly in candidates aged 65 years increased significantly since 1998, up 295% and 643%, respectively. The percentage of pediatric lung transplant recipients decreased (3.5% in 2007) (McCurry et al., 2009). Implementation of LAS had no effect on 1-year posttransplant survival rates in overall or diagnostic subgroups. The highest 1-year survival rates were seen in the diagnostic group C (89% pre-las versus 87% Page. 5 of 13

6 post-las), while the lowest survival rates were seen in diagnostic group B (76% pre-las versus 80% post- LAS). Pre-LAS 1-year survival was 86% and 77% in diagnostic groups A and D, compared with post-las survival of 85% and 80%, respectively (McCurry et al., 2009). Lung transplantation has significant postoperative mortality and morbidity rates, despite advances in surgical techniques and lung preservation. Compared with other solid organ transplantation programs, lung transplantation is associated with the smallest long-term survival benefit. Graft failure and infections cause many of the deaths in the early posttransplant period, whereas chronic rejection and infections cause many of the late posttransplant deaths. Moreover, lung transplant recipients may suffer from morbidity associated with transplantation and immunosuppression. A common serious complication is the development of bronchiolitis obliterans syndrome (BOS). BOS affects more than 50% of the long-term lung transplantation survivors and accounts for 57% of the deaths after 1 year. BOS is thought to represent a form of chronic allograft rejection and is characterized by a progressive loss of lung function and reduced patient survival. In addition to allograft rejection, the risk factors that contribute to the development of BOS include nonimmunologic factors such as viral infections and ischemic injury. Current management strategies for BOS focus on augmenting immunosuppression, which is often ineffective for established BOS. Additional complications following lung transplantation involve systemic hypertension, renal dysfunction, diabetes, and hyperlipidemia. More uncommon but devastating events such as blindness and stroke may also occur after lung transplantation. QOL measurements address some of these issues (Bando et al., 1995; Sundaresan et al., 1995; Hosenpud et al., 1996; Valentine et al., 1996; Trulock, 1997; Sundaresan, 1998; Estenne et al., 2002; Frost, 2002; Trulock et al., 2005; OPTN/SRTR, 2007). PROCEDURE DESCRIPTIONS Single Lung Transplantation: The operation begins when the donor lung arrives in the operating room. A single lung transplant requires 4 to 8 hours. A history of prior chest surgery may complicate the procedure and require additional time. The lung with worse pulmonary function is chosen for replacement. If both lungs function equally, then the right lung is usually favored for removal, because that avoids having to maneuver around the heart, as would be required for excision of the left lung. Single lung transplants are usually done through an incision extending from under the shoulder blade around the chest, ending near the sternum. An alternative method is an incision under the breastbone. Following excision of the native lung, the donor lung is wrapped in sponges soaked with a cold crystalloid solution and placed into the hemithorax. The bronchial anastomosis is performed first. The length of both the donor and recipient bronchi is minimized to preserve collateral blood supply and to achieve some degree of anastomotic overlap. After completion of the anastomoses, the lung is reinflated gently. A bronchoscopy is performed to clear remaining blood and mucus from the new lung. When the surgeon is satisfied with the performance of the lung, the chest incision is closed (Moffatt-Bruce, 2009). Double Lung Transplantation: A double lung transplant, also known as a bilateral transplant, can be executed sequentially, en bloc, or simultaneously. The most frequently performed double lung transplantation procedure is bilateral sequential single lung transplantation. The procedure requires 6 to 12 hours. For double lung transplants, an incision, known as a clamshell incision, is made across the entire chest, just below the breasts. Mobilization and pneumonectomy of the native lung and the implantation of the lung graft are conducted in the same manner as described for single lung transplantation. A bronchoscopy is performed before starting the surgery on the second lung. In 10% to 20% of double lung transplants, the patient is connected to a heart-lung machine (ACCP, 2005; Moffatt-Bruce, 2009). Common Approach to Immunosuppressive Therapy: Transplant rejection is a primary concern, both Page. 6 of 13

7 immediately after surgery and continuing throughout the patient s life. Signs of rejection are fever, flu-like symptoms, increased difficulty breathing, worsening pulmonary test results, increased chest pain or tenderness, or an increase or decrease in body weight > 2 kilograms (kg) per 24 hours. To prevent transplant rejection and subsequent damage to the new lung or lungs, patients must commit to a lifelong regimen of immunosuppressive drugs. Induction therapy is the use of very potent medications at the time of transplant. Its use is controversial, and approximately 50% of the centers use this form of immunosuppression therapy. Baseline immunosuppressant therapy prior to discharge includes: corticosteroids; a calcineurin inhibitor, tacrolimus (83%) or cyclosporine; and a cell-cycle inhibitor, either azathioprine or mycophenolate mofetil. Maintenance immunosuppression administered for the first year following transplantation is essentially the same as immunosuppression before discharge, except for an increase in the use of tacrolimus and sirolimus. The first line of treatment for an episode of acute rejection is high-dose intravenous steroid pulses. For ongoing or recurrent acute rejection, the strategy is to add rapamycin, a newer antiproliferative drug. The second choice for refractory acute rejection is treatment with antithrombocyte globulin (ATG) or OKT3. In refractory cases, high-dose intravenous immune globulin can be used (Hachem et al., 2008; Mulligan et al., 2008; Moffatt-Bruce, 2009). Treatment of chronic rejection (BOS) is the most difficult issue following lung transplantation. Patients taking a cyclosporine regimen should be switched to tacrolimus. For patients unresponsive to the change from cyclosporine to tacrolimus, high-dose steroid pulses and antithymocyte globulin are frequently used. Rapamycin may also be introduced as a fourth agent. Other possible therapies are total lympoid irradiation and photopheresis. As the episodes of rejection may reoccur throughout a patient s life, the exact choices and dosages of immunosuppressants will be modified over time. Inherent to the immunosuppressants, transplant patients are vulnerable to infections. Antibiotics may be prescribed to treat or prevent infections. Certain medications may also have nephrotoxic side effects or trigger allergic reactions. Close follow-up care is required to balance the benefits and potential risks of the drugs (Ohashi et al., 2003; Moffatt-Bruce, 2009). REGULATORY STATUS: 1. U.S. FOOD AND DRUG ADMINISTRATION (FDA): Lung transplantation is a procedure and is not subject to regulation by the FDA. However, immunosuppressive agents, coagulants, anticoagulants, organ preservation solutions, surgical and monitoring devices used before, during, and after surgery, and organ transport devices are subject to FDA regulation (FDA. 2009a; FDA, 2009b). 2. CENTERS FOR MEDICARE AND MEDICAID SERVICES (CMS): In 1995, Medicare approved coverage for deceased lung transplantation in selected patients. This national coverage policy stated that transplantation is a medically reasonable and necessary service for certain patients with end-stage lung disease, when performed by Medicare participating facilities that meet specific criteria, including patient selection criteria (CMS, 2005). On March 23, 2007, Medicare issued a final rule setting forth requirements that transplant centers must meet to participate in the Medicare program that moves Medicare covered transplant programs toward an outcome-focused system (CMS, 2007). The rule became effective on June 28, Transplant organ programs were defined as a component within a transplant hospital that provides transplantation of a particular type Page. 7 of 13

8 of organ. All organ transplant programs must be located in a hospital that has a Medicare provider agreement. In addition to meeting the transplant Conditions of Participation, the transplant program must also comply with the hospital Conditions of Participation (CMS, 2009). 3. MINNESOTA DEPARTMENT OF HUMAN SERVICES (DHS): Minnesota Health Care Programs (MHCP) coverage for organ and tissue transplant procedures is limited to those procedures covered by the Medicare program or approved by the DHS consulting contractor. Transplant coverage includes: preoperative evaluation, recipient and donor surgery, follow-up care for the recipient and live donor, and retrieval of organs, tissues. All transplant related services are billed under the recipient s ID number. CLINICAL EVIDENCE: SUMMARY: Lung transplantation (LTX) has become a viable treatment option for carefully selected patients with endstage pulmonary disease (ESPD). Single, double, and lobar-lung transplantation have all been performed successfully for a variety of diseases. Single-LTX appears to be most effective for patients with end-stage pulmonary fibrosis, while double-ltx is most effective for patients with end-stage chronic obstructive pulmonary disease (COPD) and cystic fibrosis (CF) in which cardiac function has been preserved. Lobar-LTX (from living donors or cadaver donors) is usually reserved for children or adolescents who are appropriate candidates for LTX and will not survive waiting for cadaver lungs. Indications for LTX in pediatric patients include pulmonary vascular disease, bronchiolitis obliterans, broncho-pulmonary dysplasia, graft failure due to viral pneumonitis, and CF. Chronic obstructive pulmonary disease and alpha 1-antitrypsin deficiency, the 2 principal causes of emphysema, are responsible for approximately 60 % of all single-ltx performed. Other indications for single-ltx include primary pulmonary hypertension, Eisenmenger's syndrome, as well as a variety of interstitial lung diseases (e.g., interstitial pulmonary fibrosis). Cystic fibrosis, emphysema, and alpha 1-antitrypsin deficiency are the most common indications for double-ltx, also known as bilateral single-ltx (sequential replacement of both lungs). Comparing patients who have undergone en bloc double-ltx to patients who have undergone bilateral single-ltx, studies have shown a better outcome for those who have undergone the bilateral sequential procedure. The latter is generally considered the procedure of choice for patients with any pulmonary disorder complicated by chronic airway infection, such as bronchiectasis, CF, and chronic bronchitis. The possibility of spillover of infection from the native lung to the allograft precludes single-ltx in such patients. Although LTX offers acceptable prospects for 5-year survival, chronic rejection and donor shortage remain to be major problems. To address the problem of donor shortage, living-donor lobar-ltx has been performed with satisfactory intermediate survival and functional results. In lobar-ltx, a lobe of the donor's lung is excised, sized appropriately for the recipient, and transplanted. Common indications for livingdonor bilateral lobar-ltx are CF and severe primary pulmonary hypertension. Based on available scientific evidence, there is no significant difference in effectiveness between living-donor lobar-ltx and cadaver lobar-ltx. Page. 8 of 13

9 Complications of LTX include re-implantation response and airway complications. Rejection may occur in the hyper-acute, acute, or chronic settings and requires judicious management with immunosuppression. Infection and malignancy remain potential complications of the commitment to lifelong systemic immunosuppression. Obese (greater than 20 % of ideal body weight), cachectic (less than 80 % of ideal body weight), mechanically ventilated or otherwise immobile patients are considered poor candidates for transplantation. APPLICABLE CODES: The Current Procedural Terminology (CPT ) codes and HCPCS codes listed in this policy are for reference purposes only. Listing of a service or device code in this policy does not imply that the service described by this code is a covered or non-covered health service. The inclusion of a code does not imply any right to reimbursement or guarantee claims payment. Other medical policies and coverage determination guidelines may apply. HCPCS Codes S2060 S2061 S2152 ICD-9 Diagnosis Description Lobar lung transplantation Donor lobectomy (lung) for transplantation, living donor Solid organ(s), complete or segmental, single organ or combination of organs; deceased or living donor(s), procurements, transplantation, and related complications including: drugs; supplies; hospitalization with outpatient follow-up; medical/surgical, diagnostic, emergency, and rehabilitative services; and the number of days of pre-and post-transplant care in the global definition Description Tuberculosis of lung code range Tuberculous fibrosis of lung code range Unspecified pulmonary tuberculosis code range 135 Sarcoidosis; lung involvement in other diseases classified elsewhere Lymphangiomyomatosis lung , Cystic fibrosis code range Alpha-1 antitrypsin deficiency Eosinophilic granuloma , Malignant hypertensive heart disease code range , Benign hypertensive heart disease code range , Unspecified hypertensive heart disease code range Iatrogenic pulmonary embolism and infarction Other pulmonary embolism and infarction Septic pulmonary embolism Primary pulmonary hypertension Chronic pulmonary embolism Other chronic pulmonary heart diseases Unspecified chronic pulmonary heart disease , Obstructive chronic bronchitis code range Bronchiolitis obliterans Emphysematous bleb Emphysema Page. 9 of 13

10 494.0, Bronchiectasis code range 496 Chronic obstructive pulmonary disease 500 Coal workers' pneumoconiosis 501 Asbestosis 502 Pneumoconiosis due to other silica or silicates 503 Pneumoconiosis due to other inorganic dust 504 Pneumonopathy due to inhalation of other dust 505 Unspecified pneumoconiosis Pulmonary fibrosis due to fumes and vapors Fibrosis of the lungs following radiation 515 Pulmonary fibrosis, post-inflammatory Idiopathic pulmonary fibrosis Lung involvement in other diseases classified elsewhere Interstitial Emphysema Compensatory Emphysema Pulmonary eosinophilia Scleroderma Eisenmenger's syndrome Congenital bronchiectasis Bronchopulmonary dysplasia ICD-9 Procedure Transplant from live related donor Transplant from live non-related donor Transplant from cadaver Thoracoscopic segmental resection of lung Other and unspecified segmental resection of lung Thoracoscopic lobectomy of lung Other lobectomy of lung Thoracoscopic pneumonectomy Other and unspecified pneumonectomy Lung transplantation, NOS Unilateral lung transplantation Bilateral lung transplantation Cardiopulmonary bypass ICD-10 Codes Description Description 0BYM0Z0 Transplantation of bilateral lungs, allogeneic, open approach 0BYM0Z1 Transplantation of bilateral lungs, syngeneic, open approach D86.1 Sarcoidosis of lung E84.0-E84.9 Cystic fibrosis E88.81-E88.9 Metabolic syndrome, specified and unspecified Other pulmonary heart disease I38-I39 Endocarditis, valve specified and endocarditis and heart valve disorders in diseases classified elsewhere J40-J47.9 Chronic lower respiratory disease J60.0-J67.9 Lung diseases due to external agents J67.0-J67.9 Hypersensitivity pneumonitis due to organic dust J69.0-J69.8 Pneumonitis due to solids and liquids Page. 10 of 13

11 J80-J84.9 J85-J86.9 J90-J91.8 J95.00-J95.89 J96.0-J99 M31.2-M31.31 M32.0-M32.9 M34.0-M34.9 Q20.0-Q28.9 Q33.0-Q34.9 P19.0-P29.9 T T Z94.2-Z94.3 CPT Codes Other respiratory diseases affecting the interstituim Suppurative and necrotic conditions of the lower respiratory tract Pleural effusion Intraoperative and postprocedural complications and disorders of respiratory system, not elsewhere classified Other diseases of respiratory system Wegener s granulomatosis Systemic lupus erythematous (SLE) Systemic sclerosis (scleroderma) Congenital malformations of the circulatory system Congenital malformations of the respiratory system Respiratory and cardiovascular disorders specific to the perinatal period Complications of lung transplants Lung transplant status; heart and lung transplant status Description Anesthesia for heart transplant or heart/lung transplant Physiological support for harvesting of organ(s) from brain - dead patient Removal of lung; other than total pneumonectomy; single lobe (lobectomy) Removal of lung; other than total pneumonectomy; two lobes (bilobectomy) Donor pneumonectomy (including cold preservation), from cadaver donor Lung transplant, single; without cardiopulmonary bypass Lung transplant, single; with cardiopulmonary bypass Lung transplant, double (bilateral, sequential, or en bloc); without cardiopulmonary bypass Lung transplant, double (bilateral, sequential, or en bloc); with cardiopulmonary bypass Backbench standard preparation of cadaver donor lung allograft prior to transplantation, including dissection of allograft from surrounding soft tissues to prepare pulmonary venous/atrial cuff, pulmonary artery, and bronchus; unilateral Backbench standard preparation of cadaver donor lung allograft prior to transplantation, including dissection of allograft from surrounding soft tissues to prepare pulmonary venous/atrial cuff, pulmonary artery, and bronchus; bilateral CPT is a registered trademark of the American Medical Association. REFERENCES: 1. Almenar-Bonet L, Sánchez-Lázaro IJ, Martínez-Dolz L. Is age a limiting factor for access to transplantation? Transplant Proc. 2011;43(6): American Thoracic Society / European Respiratory Society Task Force. Standards for the Diagnosis and Management of Patients with COPD. Version 1.2. New York: American Thoracic Society; Updated September 8, Accessed December 5, Arcasoy S, Kotloff R. Medical progress: lung transplantation. N Engl J Med. 1999;340(14): Baz MA, Scott MP, Staples ED, et al. Lung transplantation after long-term mechanical ventilation. Chest 2001;119: Burch M, Aurora P. Current status of paediatric heart, lung and heart-lung transplantation. Arch Dis Child. 2004;89: Cohen RG, Starnes VA. Living donor lung transplantation. World Journal of Surgery. February 2001;25(2): Cordova FC. Medical pneumoplasty, surgical resection, or lung transplant. Med Clin North Am. 2012;96(4): Page. 11 of 13

12 8. Culver DA, Mazzone PJ, Khandwala F, et al. Discordant utility of ideal body weight and body mass index as predictors of mortality in lung transplant recipients. J Heart Lung Transplant. 2005;24(2): Delmonico FL, Miller C, Davis J, et al. Live Organ Donor Consensus Group. Consensus statement on the live organ donor. JAMA. December 2000;284(22): De Bleser L, Dobbels F, Berben L, et. al. The spectrum of nonadherence with medication in heart, liver, and lung transplant patients assessed in various ways. Transpl Int. 2011;24(9): de la Torre MM, Delgado M, Paradela M, et al. Influence of body mass index in the postoperative evolution after lung transplantation. Transplant Proc. 2010;42(8): De Soyza A, McDowell A, Archer L, et al. Burkholderia cepacia complex genomovars and pulmonary transplantation outcomes in patients with cystic fibrosis. Lancet. November 2001;358 (9295): González-Castro A, Llorca J, Suberviola B, Díaz-Regañón G, Ordóñez J, Miñambres E. Influence of nutritional status in lung transplant recipients. Transplant Proc. 2006;38(8): Hayes, Inc. Hayes Directory Report: Lung Transplantation. November Search last updated November Lansdale, PA. 15. Hayes, Inc. Hayes Directory Report: Lung Transplantation Induction and Maintenance Immunosuppressive Therapy. January Lansdale, PA. 16. Institute for Clinical Systems Improvement (ICSI). ICSI Health Care Guideline: Chronic Obstructive Pulmonary Disease. 8th ed. March Jørgensen HS, Nakayama H, Raaschou HO, et al. Outcome and time course of recovery in stroke. Part II: time course of recovery. The Copenhagen Stroke Study. Arch Phys Med Rehabil. 1995;76(5): Kienzl-Wagner K, Pratschke J, Brandacher G. Proteomics--a blessing or a curse? Application of proteomics technology to transplant medicine. Transplantation. 2011;92(5): Korb-Savoldelli V, Sabatier B, Gillaizeau F, et al. Non-adherence with drug treatment after heart or lung transplantation in adults: a systematic review. Patient Educ Couns. 2010;81(2): Lederer DJ, Kawut SM, Wickersham N, et al. Obesity and primary graft dysfunction after lung transplantation: the Lung Transplant Outcomes Group Obesity Study. Am J Respir Crit Care Med. 2011;184(9): Lederer DJ, Wilt JS, D'Ovidio F, et al. Obesity and underweight are associated with an increased risk of death after lung transplantation. Am J Respir Crit Care Med. 2009;180(9): Levine SM. A survey of clinical practice of lung transplantation in North America. Chest. April 2004;125(4): Lu BS, Bhorade SM. Lung transplantation for interstitial lung disease. Clin Chest Med. 2004;25: Madill J, Gutierrez C, Grossman J, et.al. Toronto Lung Transplant Program. Nutritional assessment of the lung transplant patient: body mass index as a predictor of 90-day mortality following transplantation. J Heart Lung Transplant. 2001;20(3): Mandell GL, Bennett JE, Dolan R. eds. Mandell, Douglas, and Bennett's Principles And Practice Of Infectious Diseases. 7th ed. Philadelphia PA. Churchill Livingstone Elsevier Martins PN, Pratschke J, Pascher A, et al. Age and immune response in organ transplantation. Transplantation. January 2005;79(2): Martinu T, Babyak MA, O Connell DF, et al. Baseline 6-min walk distance predicts survival in lung transplant candidates. Am J Transpl. 2008:8: Maurer JR. International guidelines for the selection of lung transplant candidates. The International Society for Heart and Lung Transplantation, the American Thoracic Society, the American Society of Transplant Physicians, the European Respiratory Society. J Heart Lung Transplantation. 1998;17(7): Maurer JR, Zamel N. Lung Transplantation. In: Mason RJ, Murray JF, Broaddus VC, Nadel JA, eds. Murray and Nadel s Textbook of Respiratory Medicine. 4th ed. Philadelphia, PA: Saunders; 2005: Nathan SD. Lung transplantation: Disease specific considerations for referral. Chest. March 2005;127(3): National Heart Lung and Blood Institute. Clinical Guideline on the identification, evaluation and treatment of overweight and obesity in adults. September Page. 12 of 13

13 Accessed December 5, Orens J, Estenne M, Arcasoy S, et al. International guidelines for the selection of lung transplant candidates: 2006 update a consensus report from the pulmonary scientific council of the international society for heart and lung transplantation. J Heart Lung Transplant 2006; 25: Rich S, McLaughlin VV. Pulmonary Hypertension. In: Zipes DP, Libby P, Bonow RO, Braunwald E, eds. Braunwald: Heart Disease: A Textbook of Cardiovascular Medicine. 7th ed. Philadelphia, PA: Saunders; 2005: Roland ME. Perspective: solid-organ transplantation in HIV-infected patients in the potent antiretroviral therapy era. Top HIV Med. July/August 2004;12(3): Sato M, Keshavjee S. Bronchiolitis obliterans syndrome: alloimmune-dependent and independent injury with aberrant tissue remodeling. Semin Thorac Cardiovasc Surg. 2008;20: Shah AS, Nwakanma L, Simpkins C, et.al. Pretransplant panel reactive antibodies in human lung transplantation:an analysis of over 10,000 patients. Ann Thorac Surg. 2008;85(6): Singer JP, Yusen RD. Defining patient-reported outcomes in chronic obstructive pulmonary disease:the patientcentered experience. Med Clin North Am. 2012;96(4): Starnes VA, Bowdish ME, Woo MS et al. A decade of living lobar lung transplantation: recipient outcomes. J Thoracic Cardiovasc Surg. January 2004;127(1): Sweet SC. Pediatric lung transplantation: update Pediatr Clin N Am. 2003;50: Townsend S. Congenital Heart Disease. In: Sabiston Textbook of Surgery. 17th ed. Philadelphia, PA: W. B. Saunders; 2004: Truog RD. The ethics of organ donation by living donors. N Engl J Med. August 2005;353(5): United Network for Organ Sharing. Resources: what is the lung allocation system? Accessed December 5, Wells WJ, Barr ML. The ethics of living donor lung transplantation. Thorac Surg Clin. November 2005;15(4): Woo MS. An overview of paediatric lung transplantation. Paediatr Respir Rev. 2004;5(3): POLICY HISTORY: DATE ACTION/DESCRIPTION 12/09/2013 New Policy 2013M0047A. Reviewed by Interim Medical Policy Committee. 12/19/2013 Reviewed and approved by the Quality Improvement Advisory and Credentialing Council (QIACC). 01/01/2014 Published to UCare.org 07/01/2015 Policy Update: Added applicable ICD-10 codes to the Coding Section. The list of codes may not be all-inclusive and does not denote coverage. Policy identification number updated to 2015M0047A. Page. 13 of 13