JOHNS HOPKINS HEALTHCARE

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1 Page 1 of 23 ACTION: New Policy Effective Date: 03/2003 Revising : Review Dates: 10/22/04, 10/21/05, 10/19/06, Superseding Archiving Retiring 06/25/08, 06/04/09, 06/04/10, 01/07/11, 08/20/13, 12/06/13, 09/04/15, 09/01/17 Johns Hopkins HealthCare LLC (JHHC) provides a full spectrum of health care products and services for Employer Health Programs, Priority Partners, Advantage MD, and US Family Health Plan. Each line of business possesses its own unique contract and guidelines which, for benefit and payment purposes, should be consulted to know what benefits are available for reimbursement. Specific contract benefits, guidelines or policies supersede the information outlined in this policy. POLICY: For US Family Health Plan see TRICARE Policy Manual M, February 1, 2008, Heart-Lung and Lung Transplantation: Chapter 4, Section 24.1; Simultaneous Pancreas-Kidney (SPK), Pancreas-after-Kidney (PAK) and Pancreas Transplant Alone (PTA), and Pancreatic Islet Cell Transplantation: Chapter 4, Section 24.7; Combined Liver-Kidney Transplantation (CLKT): Chapter 4, Section 24.6; Heart Transplantation: Chapter 4, Section 24.2; Small Intestine (SI), Combined Small Intestine-Liver (SI/L) and Multivisceral Transplantation: Chapter 4, Section 24.4; Combined Heart-Kidney Transplantation (CHKT): Chapter 4, Section 24.3; Kidney Transplantation: Chapter 4, Section 24.8; High Dose Chemotherapy (HDC) and Stem Cell Transplantation: Chapter 4, Section 23.1; Liver Transplantation: Chapter 4, Section For Advantage MD, see Medicare Coverage Database: National Coverage Determination (NCD) for Adult Liver Transplantation (260.1) National Coverage Determination (NCD) for Pediatric Liver Transplantation (260.2) National Coverage Determination (NCD) for Pancreas Transplants (260.3) National Coverage Determination (NCD) for Islet Cell Transplantation in the Context of a Clinical Trial ( ) National Coverage Determination (NCD) for Heart Transplants (260.9) National Coverage Determination (NCD) for Intestinal and Multi-Visceral Transplantation (260.5) I. All transplants must be performed at a facility certified for the type of organ transplant requested. II. All solid organ transplants require Medical Director review for authorization prior to listing. A comprehensive medical and psychosocial evaluation is required in order to address the general contraindications in Section III (below) as well as any organ specific medical

2 Page 2 of 23 necessity criteria. The evaluation must also specifically address the patient s life expectancy with transplant and ability to benefit from transplantation. III. The following contraindications apply to all solid organ transplants: A. Major psychiatric illness that cannot be managed sufficiently to allow post-transplant care and safety B. Evidence of significant non-compliance C. Multiple uncorrectable congenital anomalies D. Severe neurological deficit E. Life expectancy with transplant < 5 years F. Active substance abuse (drugs, alcohol) 1. All patients with a current or past history of drug and/or alcohol abuse must have a comprehensive evaluation by a psychiatrist or psychologist with expertise in the diagnosis and treatment of addiction, AND; 2. Patients with current drug and/or alcohol abuse must comply with the treatment recommendations based on the assessment for a minimum of 90 days. Documentation of compliance and sobriety must be submitted with the request for listing, AND; 3. Patients with current drug and/or alcohol abuse must have a plan for on-going monitoring and treatment during the pre- and post-transplant period G. Advanced cardiopulmonary disease H. Significant organ system failure (other than the organ being transplanted) IV. Once the evaluation has been completed, JHHC will make a determination regarding approval of the transplant using the organ-specific criteria below: A. When benefits are provided under the member s contract, JHHC considers Heart Transplantation medically necessary when the member meets the above listed requirements AND the transplanting institution's protocol eligibility criteria 1. In the absence of a protocol, JHHC considers heart transplantation medically necessary for heart failure with irreversible underlying etiology when the member meets the above listed criteria in sections I, II and III, AND when ALL of the following criteria are met: a. New York Heart Association (NYHA) classification of heart failure III or IV (see Appendix), -- does not apply to pediatric members, AND; b. Member has potential for conditioning and rehabilitation after transplant (i.e., member is not moribund), AND; c. No malignancy (except for non-melanomatous skin cancers) or malignancy has been completely resected or (upon individual case review) malignancy has been adequately treated with no substantial likelihood of recurrence with acceptable future risks, AND; d. Adequate pulmonary, liver and renal function, AND;

3 Page 3 of 23 e. Absence of active infections that are not effectively treated, AND; f. Member has any of the following conditions (not an all-inclusive list): i. Cardiac arrhythmia ii. Cardiac re-transplantation due to graft failure iii. Cardiomyopathy due to nutritional, metabolic, hypertrophic or restrictive etiologies iv. Congenital heart disease v. End-stage ventricular failure vi. Idiopathic dilated cardiomyopathy vii. Inability to be weaned from temporary cardiac-assist devices after myocardial infarction or non-transplant cardiac surgery viii. Intractable coronary artery disease ix. Myocarditis x. Post-partum cardiomyopathy xi. Right ventricular dysplasia/cardiomyopathy xii. Valvular heart disease g Absence of active or recurrent pancreatitis, AND; h Absence of diabetes with severe end-organ damage (neuropathy, nephropathy with declining renal function and proliferative retinopathy), AND; i. No uncontrolled and/or untreated psychiatric disorders that interfere with j compliance to a strict treatment regimen, AND; No active alcohol or chemical dependency that interferes with compliance to a strict treatment regimen. 2. Heart transplant is considered not medically necessary for persons with any of the following contraindications: Presence of irreversible end-organ diseases (e.g. renal, hepatic, pulmonary) (unless person is to undergo dual organ transplantation, e.g. heart-lung, heart-kidney, etc.), OR; Presence of severe pulmonary hypertension with irreversibly high pulmonary vascular resistance, OR, Presence of a recent intra-cranial cerebrovascular event with significant persistent deficit, OR; Presence of a bleeding peptic ulcer, OR; Presence of hepatitis B antigen, OR; Presence of diverticulitis, OR; Presence of immediately life-threatening neuromuscular disorders, OR; Presence of HIV/AIDS with profound immunosuppression (CD4 count of less than 200 cells/mm3), OR; Presence of AL amyloidosis (although amyloidosis is considered a contraindication to heart transplantation, exceptions may be made in circumstances where curative therapy of amyloidosis has been performed or is planned (e.g. stem cell transplantation in primary

4 Page 4 of 23 amyloidosis, liver transplantation in familial amyloidosis). Unless specific benefits are provided under the member s contract, JHHC considers cytokine gene polymorphism testing experimental and investigational for evaluating graft rejection following heart transplantation as it does not meet Technology Evaluation Criteria (TEC) #2-5. B. When benefits are provided under the member s contract, JHHC considers a Food and Drug Administration-approved total artificial heart (e.g., CardioWest Total Artificial Heart, SynCardia Systems, Tucson, AZ) medically necessary when used as a bridge to transplant for transplant-eligible members who are at imminent risk of death (NYHA Class IV) due to biventricular failure who are awaiting heart transplantation. Unless specific benefits are provided under the member s contract, JHHC considers the use of a total artificial heart (e.g., ABIOCOR, Total Artificial Heart, SynCardia temporary Total Artifical Heart (formerly known as CardioWest Total Artificial Heart) as permanent treatment (destination)(i.e., as an alternative to heart transplantation) experimental and investigational as it does not meet Technology Evaluation Criteria (TEC) #2-5. C. When benefits are provided under the member s contract, JHHC considers Heart-Lung Transplantation medically necessary when the member meets the above listed requirements AND the transplanting institution's protocol eligibility criteria. 1. In the absence of a protocol, JHHC considers heart-lung transplantation medically necessary for severe refractory heart failure plus either end-stage lung disease or irreversible pulmonary hypertension when the member meets the above listed criteria in sections I, II and III, AND when ALL of the following criteria are met: a. Absence of chronic high-dose steroid therapy. Due to problems in bronchial healing, persons receiving high-dose steroids are considered inappropriate candidates, AND; b. Absence of acute or chronic active infections that are not effectively treated, AND; c. Absence of malignancy (other than non-melanomatous skin cancers) or malignancy has been completely resected or (upon medical review) it is determined that malignancy has been treated with small likelihood of recurrence and acceptable future risks, AND; d. Adequate functional status. Active rehabilitation is considered important to the success of transplantation. Under established guidelines, mechanically ventilated or otherwise immobile persons are considered poor candidates for transplantation; however, bridge to transplant with ambulatory ECMO does not, in itself, rule out candidacy for heart-lung transplantation, AND; e. Adequate liver and kidney function, defined as a bilirubin of less than 2.5 mg/dl and a creatinine clearance of greater than 50 ml/min/kg, AND;

5 Page 5 of 23 f. Life expectancy (in the absence of cardiopulmonary disease) of greater than 2 years, AND; g. No active alcohol or chemical dependency that interferes with compliance to a strict treatment regimen, AND; h. No uncontrolled and/or untreated psychiatric disorders that interfere with compliance to a strict treatment regimen, AND; i. Member has any of the following conditions (not an all-inclusive list): i. Chronic obstructive pulmonary disease with severe heart failure* ii. Congenital heart disease associated with pulmonary hypertension that are not amenable to lung transplantation and repair by standard cardiac surgery iii. Cystic fibrosis with severe heart failure* iv. Eisenmenger s complex with irreversible pulmonary hypertension and severe heart failure* v. Irreversible primary pulmonary hypertension with severe heart failure* vi. Connective tissue disease or other causes of severe pulmonary fibrosis with uncontrollable pulmonary hypertension or severe heart failure* vii. Severe coronary artery disease or cardiomyopathy with irreversible pulmonary hypertension 2. Heart-lung transplantation is considered not medically necessary where lung transplantation alone will restore right ventricular function; every attempt should be made to preserve the heart. 3. Heart-lung transplantation may be considered medically necessary for other congenital cardiopulmonary anomalies upon individual case review. * Note ~ Severe (New York Heart Association (NYHA) classification III or IV (see Appendix) heart failure where right ventricular function would not be restored with lung transplant alone. D. When benefits are provided under the member s contract, JHHC considers Pancreas Transplantation alone (PTA) without Kidney Transplant medically necessary when the member meets the above listed requirements in sections I, II and III, AND the transplanting institution's protocol eligibility criteria. 1. In the absence of a protocol, JHHC considers pancreas transplantation alone (PTA) without kidney transplant medically necessary when the member meets the above listed criteria in sections I, II and III, AND when ALL of the following criteria are met: a. Absence of ongoing or recurrent active infections that are not effectively treated, AND; b. Member has adequate cardiac status (e.g., no angiographic evidence of significant coronary artery disease, ejection fraction greater than or equal to 40 %, no myocardial infarction in last 6 months, negative stress test), AND;

6 Page 6 of 23 c. No malignancy (except for non-melanomatous skin cancers) or malignancy has been completely resected, OR (upon medical review) malignancy has been adequately treated such that the risk of recurrence is small, AND; d. Member has a history of labile (brittle) insulin-dependent diabetes mellitus (IDDM), AND; e. Member has recurrent, acute and severe metabolic and potentially lifethreatening complications requiring medical attention, as documented by chart notes, frequent emergency room visits and/or hospitalizations. They may include: i. Hyperglycemia, OR; ii. Hypoglycemia, OR; iii. Hypoglycemic unawareness associated with high risk of injury, OR; iv. Ketoacidosis, AND; j. Member has consistent failure of exogenous insulin-based management, defined as inability to achieve sufficient glycemic control (HbA1c of greater than 8.0) or recurrent hypoglycemic unawareness, despite aggressive conventional therapy including all of the following: i. Adjusting frequencies and amounts of insulin injected, AND; ii. Measuring multiple blood glucose levels on a daily basis, AND; iii. Modifying diet and exercise, AND; iv. Monitoring HgbA1c levels. 2. Pancreas retransplantation after a failed primary pancreas transplant is considered medically necessary when member meets the selection criteria stated above. 3. Pancreas retransplantation after 2 or more prior failed pancreas transplants may be considered medically necessary upon individual case review. 4. Pancreas transplant is considered medically necessary for members with the following relative contraindications to pancreas transplant only if the requesting physician documents that these relative contraindications were considered, and has determined that the benefits of pancreas transplant outweigh the risks in these members. Relative contraindications to PTA include the following: a. Ejection fraction 35 % to 40 %, OR; b. Severe peripheral vascular disease. 5. Islet cell autotransplantation (i.e., transplantation of the member's own islet cells) medically necessary for members undergoing near-total or total pancreatic resection for severe, refractory chronic pancreatitis. 6. Unless specific benefits are provided under the member s contract, JHHC considers islet cell allotransplantation (i.e. transplantation of islet cells from a donor) experimental and investigational as it does not meet Technology Evaluation Criteria (TEC) # Partial pancreas transplant from a living donor is considered an acceptable alternative to cadaveric transplant for persons who meet medical necessity criteria for pancreas transplant.

7 Page 7 of 23 E. When benefits are provided under the member s contract JHHC considers Simultaneous Pancreas-Kidney Transplantation (SPK) and Simultaneous Cadaver- Donor Pancreas and Living-Donor Kidney (SPLK) Transplantation medically necessary for members with diabetes and end-stage renal disease (ESRD) who meet the above listed requirements in sections I, II and III AND the transplanting institution's protocol eligibility criteria. 1. In the absence of a protocol, JHHC considers SPK transplantation and SPLK transplantation medically necessary in persons with diabetes and ESRD when the member meets the above listed criteria in sections I, II and III, AND when ALL of the following criteria are met: a. Member has a creatinine clearance (Clcr), calculated by the Cockcroft-Gault formula (see Appendix), of less than 20 ml/min, or a directly measured glomerular filtration rate (GFR) of less than 20 ml/min, AND; b. Member has ESRD and requires dialysis or is expected to require dialysis in the next 12 months. 2. JHHC considers SPK and PAK transplantation medically necessary for persons with any of the following relative contraindications if the attending physician determines and documents that the potential benefits of SPK transplantation outweigh the risks. Relative contraindications to SPK transplantation include: a. Chronic liver disease b. Clinical evidence of severe cerebrovascular or peripheral vascular disease (e.g., ischemic ulcers, previous amputation secondary to vascular disease). Adequate peripheral arterial supply should be determined by standard evaluation in the vascular laboratory including Doppler examination and plethysmographic readings of systolic blood pressure. c. Past psychosocial abnormality d. Persons with body mass index (BMI) of 35 or higher and type 2 diabetes e. Structural genito-urinary abnormality or recurrent urinary tract infection f. Substance abuse history (other than persistent substance abuse as indicated in Section III, F.) g. Treated malignancy (SPK transplantation is considered medically necessary in persons with malignant neoplasm if the neoplasm has been adequately treated and the risk of recurrence is small) h. Uncontrolled hypertension When benefits are provided under the member s contract, JHHC considers Kidney Transplantation medically necessary for members who meet the above listed requirements in sections I, II and III AND the transplanting institution's protocol eligibility criteria, AND meets ALL of the following criteria: 1. Absence of malignancy or the malignancy has had curative therapy (e.g., surgical resection of non-invasive squamous cell or basal cell skin cancer) or the estimated risk of recurrence of the malignancy is less than 10 % within the next 2 years. For example, renal cell carcinoma treated by nephrectomy with no

8 Page 8 of 23 evidence of metastatic disease 2 years after the nephrectomy, prostate cancer with negative prostate-specific antigen levels after treatment, surgically treated colon cancer, thyroid cancer with normal thyroglobulin levels after therapy, and others. Women should have a negative Pap smear within the past 3 years and mammography, where indicated, within the past 2 years, AND; 2. Absence of systemic infection, AND; 3. Attending physician determines that there is no prohibitive cardiovascular, pulmonary and hepatic risk, AND; 4. Severity of disease is equal to at least ONE of the following: a. Member is already on hemodialysis or continuous ambulatory peritoneal dialysis (CAPD), OR; b. Member has chronic renal failure with anticipated deterioration to end stage renal disease, where member is seeking precertification for cadaveric kidney transplantation**, OR; c. Member has end stage renal disease, evidenced by a creatinine clearance below 20 ml/min or development of symptoms of uremia, and member is seeking precertification for a living donor kidney transplantation. 5. Given waiting periods for cadaveric donors averaging 1 to 4 years, kidney transplantation is considered medically necessary for persons with severe chronic renal failure with anticipated progression to end stage renal disease. Severe chronic renal failure is defined as a creatinine clearance of less than 30 ml/min. 6. Kidney transplant is not considered medically necessary for persons who do not meet the transplanting institution's protocol selection criteria, or in the absence of a protocol, for persons who have any of the following (not an all-inclusive list): a. Active vasculitis, OR; b. Age over 70 years with severe co-morbidities, OR; c. Life threatening extra-renal congenital abnormalities, OR; d. Ongoing alcohol or drug abuse, OR; e. Severe neurological or mental impairment, in persons without adequate social support, such that the person is unable to adhere to the regimen necessary to preserve the transplant, OR; f. Untreated coagulation disorder F. When benefits are provided under the member s contract, JHHC considers Combined Kidney/Pancreas Transplantation medically necessary for members who meet the above listed requirements in sections I, II and III AND the transplanting institution's protocol eligibility criteria for persons undergoing kidney transplantation due to diabetic nephropathy. G. When benefits are provided under the member s contract, JHHC considers Liver Transplantation medically necessary for the indications listed below for adolescents

9 Page 9 of 23 and adults with either (i) a Model of End-stage Liver Disease (MELD) score (see Appendix) greater than 10; or (ii) who are approved for transplant by the United Network for Organ Sharing (UNOS) Regional Review Board, and for children less than 12 years of age who meet the transplanting institution's selection criteria. Requests for liver transplantation for adolescents and adults with a MELD score of 10 or less who have not been approved by the UNOS Regional Review Board are subject to medical necessity review. In the absence of an institution's selection criteria, JHHC considers liver transplantation medically necessary for adolescents and adults with a MELD score greater than 10 or who are approved by the UNOS Regional Review Board and for children who meet the medical necessity criteria specified below using orthotopic (normal anatomical position) liver transplantation (with cadaveric organ, reduced-size organ, living related organ, and split liver) for members with end-stage liver disease (ESLD) due to any of the following conditions, (not an all-inclusive list): 1. Cholestatic diseases: a. Biliary atresia b. Familial cholestatic syndromes c. Primary biliary cirrhosis d. Primary sclerosing cholangitis with development of secondary biliary cirrhosis 2. Hepatocellular diseases: a. Alcoholic cirrhosis b. Chronic active hepatitis with cirrhosis (hepatitis B or C) c. Cryptogenic cirrhosis d. Idiopathic autoimmune hepatitis e. Post-necrotic cirrhosis due to hepatitis B surface antigen negative state 3. Malignancies: a. Primary hepatocellular carcinoma confined to the liver when all of the following criteria are met: i. Any lung metastases that have been shown to be responsive to chemotherapy, AND; ii. Member is not a candidate for subtotal liver resection, AND; iii. Member meets UNOS criteria for tumor size and number, AND; iv. There is no identifiable extra-hepatic spread of tumor to surrounding lymph nodes, abdominal organs, bone or other sites, AND; v. There is no macrovascular involvement vi. These criteria are intended to be consistent with UNOS guidelines for selection of liver transplant candidates for hepato-cellular carcinoma (HCC). 4. Hepatoblastomas in children when all of the following criteria are met: a. Member is not a candidate for subtotal liver resection, AND; b. Member meets UNOS criteria for tumor size and number, AND; c. There is no identifiable extra-hepatic spread of tumor to surrounding

10 Page 10 of 23 lungs, abdominal organs, bone or other sites. (Note: Spread of hepatoblastoma to veins and lymph nodes does not disqualify a member for coverage of a liver transplant.) 5. Epithelioid hemangioendotheliomas 6. Intra-hepatic cholangiocarcinomas (i.e., cholangiocarcinomas confined to the liver); 7. Large, unresectable fibrolamellar HCCs; 8. Metastatic neuroendocrine tumors (carcinoid tumors, apudomas, gastrinomas, glucagonomas) in persons with severe symptoms and with metastases restricted to the liver, who are unresponsive to adjuvant therapy after aggressive surgical resection including excision of the primary lesion and reduction of hepatic metastases. 9. Vascular diseases such as Budd-Chiari syndrome, Veno-occlusive disease 10. Metabolic disorders and metabolic liver diseases with cirrhosis such as (not an all-inclusive list): a. Alpha 1-antitrypsin deficiency b. Hemochromatosis c. Inborn errors of metabolism d. Protoporphyria e. Wilson's disease 11. Miscellaneous: a. Familial amyloid polyneuropathy b. Polycystic disease of the liver c. Porto-pulmonary hypertension (pulmonary hypertension associated with liver disease or portal hypertension) in persons with a mean pulmonary artery pressure by catheterization of less than 35 mm Hg d. Toxic reactions (fulminant hepatic failure due to mushroom poisoning, acetaminophen (Tylenol) overdose, etc.) e. Trauma f. Hepato-pulmonary syndrome when ALL of the following selection criteria are met: i. Arterial hypoxemia (PaO2 less than 60 mm Hg or AaO2 gradient greater than 20 mm Hg in supine or standing position), AND; ii. Chronic liver disease with non-cirrhotic portal hypertension, AND; iii. Intrapulmonary vascular dilatation (as indicated by contrastenhanced echocardiography, technetium-99 macroaggregated albumin perfusion scan, or pulmonary angiography) 12. Retransplantation is considered medically necessary following a failed liver transplant if the initial transplant was performed for a covered indication. JHHC considers liver transplantation not medically necessary for members with any of the following absolute contraindications to liver transplantation: a. Active alcoholism or active substance abuse b. Active sepsis outside the biliary tract

11 Page 11 of 23 c. Other effective medical treatments or surgical options are available d. Presence of significant organ system failure other than kidney, liver or small bowel H. When benefits are provided under the member s contract, JHHC considers Lung Transplantation medically necessary for the following conditions when the member meets the above listed criteria in sections I, II and III, AND when ALL of the following criteria are met: 1. Qualifying Conditions for Lung Transplantation (not an all-inclusive list): a. Alpha1-antitrypsin deficiency: Persons who meet the emphysema/alpha1- antitrypsin deficiency disease-specific selection criteria below b. Broncho-pulmonary dysplasia c. Congenital heart disease (Eisenmenger's defect or complex): Persons who meet the disease-specific criteria for Eisenmenger's below d. Cystic fibrosis: Persons who meet the disease-specific selection criteria for cystic fibrosis e. Graft-versus-host disease or failed primary lung graft f. Lymphangioleiomyomatosis (LAM) with end-stage pulmonary disease g. Obstructive lung disease (e.g., bronchiectasis, bronchiolitis obliterans, chronic obstructive pulmonary disease (COPD), emphysema): For persons with pulmonary fibrosis, see the disease-specific selection criteria for pulmonary fibrosis below h. Primary pulmonary hypertension: Persons who meet the disease-specific selection criteria for primary pulmonary hypertension i. Restrictive lung disease (e.g., allergic alveolitis, asbestosis, collagen vascular disease, desquamative interstitial fibrosis, eosinophilic granuloma, idiopathic pulmonary fibrosis, post-chemotherapy, sarcoidosis, and systemic sclerosis [scleroderma]): For persons with sarcoidosis, see the disease-specific selection criteria below. 2. Disease-Specific Selection Criteria: a. Lung transplant for Cystic Fibrosis (CF) is considered medically necessary for persons who meet the general selection criteria for lung transplantation and exhibit AT LEAST 2 of the following signs and symptoms of clinical deterioration: i. Cycling intravenous antibiotic therapy ii. Decreasing forced expiratory volume in 1 second (FEV1) iii. Development of carbon dioxide (CO2) retention (pco2 greater than 50 mm Hg) iv. FEV1 less than 30 % predicted v. Increasing frequency of hospital admission vi. Increasing severe exacerbation of CF -- especially an episode requiring hospital admission

12 Page 12 of 23 vii. Initiation of supplemental enteral feeding by percutaneous endoscopic gastrostomy or parenteral nutrition viii. Non-invasive nocturnal mechanical ventilation ix. Recurrent massive hemoptysis x. Worsening arterial-alveolar (A-a) gradient requiring increasing concentrations of inspired oxygen (FiO2) xi. Recurrent pneumothorax b. Lung transplant for Emphysema (including alpha 1-antitrypsin deficiency) is considered medically necessary for persons who meet the general criteria for lung transplantation AND BOTH of the following clinical criteria: i. Hospitalizations for exacerbation of COPD associated with hypercapnia in the preceding year. Hypercapnia is defined as pco2 greater than or equal to 50 mm Hg with hospitalizations AND/OR the following associated factors: Declining body mass index Increasing oxygen requirements Reduced serum albumin Presence of cor pulmonale (defined as clinical diagnosis by a physician or any 2 of the following: enlarged pulmonary arteries on chest X-ray mean pulmonary artery pressure by right heart catheterization of greater than 25 mm Hg at rest or 30 mm Hg with exercise pedal edema or jugular venous distention right ventricular hypertrophy or right atrial enlargement on EKG BODE index of 7 or above (indicating 2 years or less survival) (see appendix). c. Lung transplant for Eisenmenger s Complex is considered medically necessary for persons who meet the general criteria for lung transplantation and ANY of the following disease-specific criteria: i. Marked deterioration in functional capacity (New York Heart Association (NYHA) Class III) ii. Pulmonary hypertension with mean pulmonary artery pressure by right heart catheterization greater than 25 mm Hg at rest or 30 mm Hg with exercise iii. Signs of right ventricular failure - progressive hepatomegaly, ascites d. Lung transplant for Pulmonary Fibrosis is considered medically necessary for persons who meet the general criteria for lung transplantation and ANY of the following disease-specific criteria: i. Diffusing capacity for carbon monoxide (DLCO) less than 60 % predicted

13 Page 13 of 23 ii. Presence of cor pulmonale (indicative of severe pulmonary fibrosis) or pulmonary hypertension iii. Total lung capacity (TLC) less than 70 % predicted e. Lung transplant for Pulmonary Hypertension is considered medically necessary for persons who meet the general criteria for lung transplantation plus ANY of the following criteria, and valvular disease has been excluded by echocardiography: i. Persons who are NYHA III, failing conventional vasodilators ii. (calcium channel blockers or endothelin receptor antagonists) Persons who are NYHA III, and have initiated or being considered for initiation of parenteral or subcutaneous vasodilator therapy iii. Pulmonary hypertension with mean pulmonary artery pressure by right heart catheterization of greater than 25 mm Hg at rest or 30 mm Hg with exercise, or pulmonary artery systolic pressure of 50 mm Hg or more defined by echocardiography or pulmonary angiography f. Lung transplant for Sarcoidosis is considered medically necessary for persons who meet the general criteria for lung transplantation plus ANY of the following disease-specific criteria: i. DLCO less than 60 % predicted ii. Presence of cor pulmonale (indicative of severe pulmonary fibrosis) or pulmonary hypertension iii. Total lung capacity less than 70 % predicted g. JHHC considers LOBAR (from living-related donors or cadaver donors) Lung Transplantation medically necessary for persons with endstage pulmonary disease when above listed criteria in sections I, II and III are met. I. When benefits are provided under the member s contract, JHHC considers Intestinal Transplantation medically necessary when the member meets the above listed requirements in sections I, II and III, AND the transplanting institution's protocol eligibility criteria. 1. In the absence of a protocol, JHHC considers Intestinal Transplantation medically necessary when the member meets the above listed criteria in sections I, II and III, AND when ALL of the following criteria are met: a. failed total parenteral nutrition (TPN), AND; b. Absence of acute or chronic active infections that are not effectively treated, AND; c. Adequate cardiovascular function (ejection fraction greater than or equal to 40 %), AND; 2. A combined intestinal and liver transplant is considered medically necessary for persons with advanced liver disease necessitating liver transplantation 3. In candidates for a combined transplant, adequacy of renal function should be assessed with a measured glomerular filtration rate (GFR), as a calculated GFR

14 Page 14 of 23 APPENDIX: is inaccurate in advanced liver disease. 4. Multi-visceral transplants from deceased donors are considered medically necessary for adults and children who meet criteria for the combined small bowel/liver transplant and require 1 or more abdominal visceral organs to be transplanted due to concomitant organ failure or anatomical abnormalities that preclude a small bowel/liver transplant. New York Heart Association (NYHA) classification: A. Class III: Persons with cardiac disease resulting in marked limitation of physical activity. They are comfortable at rest. Less than ordinary activity (i.e., mild exertion) causes fatigue, palpitation, dyspnea, or anginal pain B. Class IV: Persons with cardiac disease resulting in inability to carry on any physical activity without discomfort. Symptoms of cardiac insufficiency or of the anginal syndrome may be present even at rest. If any physical activity is undertaken, discomfort is increased. MELD Score Calculator: The BODE Index (Body-Mass Index, Airflow Obstruction, Dyspnea, and Exercise) is a multidimensional capacity index for COPD. The index uses the four factors for predicting the risk of death from the disease: FEV1, body mass index, dyspnea score and 6 minute walk test. BACKGROUND: Organ transplants are responsible for saving countless lives every year. Transplants are authorized when the health of a patient s organ is deteriorating and needs to be replaced by another matching, healthy organ. The most common organs that are subject to transplant procedure include the lungs, heart, liver, and kidneys. Patients that show signs of infections, heart problems, or substance abuse are not often considered candidates for transplant procedures. The success of organ transplants depends on numerous factors. These include organ type, the amount of organs being replaced, and the disease or condition that caused organ failure. Prior to surgery, physicians often consult with numerous medical professionals and psychiatrists to ensure the patient is considered an ideal candidate for organ transplant surgery.

15 Page 15 of 23 CODING INFORMATION: CPT Copyright 2017 American Medical Association. All rights reserved. CPT is a registered trademark of the American Medical Association. Note: The following CPT/HCPCS codes are included below for informational purposes. Inclusion or exclusion of a CPT/HCPCS code(s) below does not signify or imply member coverage or provider reimbursement. The member's specific benefit plan determines coverage and referral requirements. All inpatient admissions require preauthorization. PRE- AUTHORIZATION REQUIRED Compliance with the provision in this policy may be monitored and addressed through post payment data analysis and/or medical review audits Employer Health Programs (EHP) **See Specific Summary Plan Description (SPD) Priority Partners (PPMCO) refer to COMAR guidelines and PPMCO SPD then apply policy criteria US Family Health Plan (USFHP), TRICARE Medical Policy supersedes JHHC Medical Policy. If there is no Policy in TRICARE, apply the Medical Policy Criteria Advantage MD, LCD and NCD Medical Policy supersedes JHHC Medical Policy. If there is no LCD or NCD, apply the Medical Policy Criteria CPT CODES DESCRIPTION Donor pneumonectomy(s) (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 Donor cardiectomy-pneumonectomy (including cold preservation) Backbench standard preparation of cadaver donor heart/lung allograft prior to transplantation, including dissection of allograft from surrounding soft tissues to prepare aorta, superior vena cava, inferior vena cava, and trachea for implantation Heart-lung transplant with recipient cardiectomy-pneumonectomy

16 Page 16 of Donor cardiectomy (including cold preservation) Backbench standard preparation of cadaver donor heart allograft prior to transplantation, including dissection of allograft from surrounding soft tissues to prepare aorta, superior vena cava, inferior vena cava, pulmonary artery, and left atrium for implantation Heart transplant, with or without recipient cardiectomy Donor enterectomy (including cold preservation), open; from cadaver donor Donor enterectomy (including cold preservation), open; partial, from living donor Intestinal allotransplantation; from cadaver donor Intestinal allotransplantation; from living donor Removal of transplanted intestinal allograft, complete Backbench standard preparation of cadaver or living donor intestine allograft prior to transplantation, including mobilization and fashioning of the superior mesenteric artery and vein Backbench reconstruction of cadaver or living donor intestine allograft prior to transplantation; venous anastomosis, each Backbench reconstruction of cadaver or living donor intestine allograft prior to transplantation; arterial anastomosis, each Donor hepatectomy (including cold preservation), from cadaver donor Liver allotransplantation, orthotopic, partial or whole, from cadaver or living donor, any age Donor hepatectomy (including cold preservation), from living donor; left lateral segment only (segments II and III) Donor hepatectomy (including cold preservation), from living donor; total left lobectomy (segments II, III and IV) Donor hepatectomy (including cold preservation), from living donor; total right lobectomy (segments V, VI, VII and VIII) Backbench standard preparation of cadaver donor whole liver graft prior to allotransplantation, including cholecystectomy, if necessary, and dissection and removal of surrounding soft tissues to prepare the vena cava, portal vein, hepatic artery, and common bile duct for implantation; without trisegment or lobe split Backbench standard preparation of cadaver donor whole liver graft prior to allotransplantation, including cholecystectomy, if necessary, and dissection and removal of surrounding soft tissues to prepare the vena cava, portal vein, hepatic artery, and common bile duct for implantation; with trisegment split of whole liver graft into 2 partial liver grafts (ie, left lateral segment [segments II and III] and right trisegment [segments I and IV through VIII]) Backbench standard preparation of cadaver donor whole liver graft prior to allotransplantation, including cholecystectomy, if necessary, and dissection and removal of surrounding soft tissues to prepare the vena cava, portal vein, hepatic artery, and common bile duct for implantation; with lobe split of whole liver graft into 2 partial liver grafts (ie, left lobe [segments II, III, and IV] and right lobe [segments I and V through VIII])

17 Page 17 of Backbench reconstruction of cadaver or living donor liver graft prior to allotransplantation; venous anastomosis, each Backbench reconstruction of cadaver or living donor liver graft prior to allotransplantation; arterial anastomosis, each Pancreatectomy, total or subtotal, with autologous transplantation of pancreas or pancreatic islet cells Donor pancreatectomy (including cold preservation), with or without duodenal segment for transplantation Backbench standard preparation of cadaver donor pancreas allograft prior to transplantation, including dissection of allograft from surrounding soft tissues, splenectomy, duodenotomy, ligation of bile duct, ligation of mesenteric vessels, and Y-graft arterial anastomoses from iliac artery to superior mesenteric artery and to splenic artery Backbench reconstruction of cadaver donor pancreas allograft prior to transplantation, venous anastomosis, each Transplantation of pancreatic allograft Removal of transplanted pancreatic allograft Donor nephrectomy (including cold preservation); from cadaver donor, unilateral or bilateral Donor nephrectomy (including cold preservation); open, from living donor Backbench standard preparation of cadaver donor renal allograft prior to transplantation, including dissection and removal of perinephric fat, diaphragmatic and retroperitoneal attachments, excision of adrenal gland, and preparation of ureter(s), renal vein(s), and renal artery(s), ligating branches, as necessary Backbench standard preparation of living donor renal allograft (open or laparoscopic) prior to transplantation, including dissection and removal of perinephric fat and preparation of ureter(s), renal vein(s), and renal artery(s), ligating branches, as necessary Backbench reconstruction of cadaver or living donor renal allograft prior to transplantation; venous anastomosis, each Backbench reconstruction of cadaver or living donor renal allograft prior to transplantation; arterial anastomosis, each Backbench reconstruction of cadaver or living donor renal allograft prior to transplantation; ureteral anastomosis, each Recipient nephrectomy (separate procedure) Renal allotransplantation, implantation of graft; without recipient nephrectomy Renal allotransplantation, implantation of graft; with recipient nephrectomy Removal of transplanted renal allograft Renal autotransplantation, reimplantation of kidney HCPCS DESCRIPTION CODES S2053 Transplantation of small intestine and liver allografts

18 Page 18 of 23 S2054 S2055 S2060 S2061 S2065 S2152 Transplantation of multivisceral organs Harvesting of donor multivisceral organs, with preparation and maintenance of allografts; from cadaver donor Lobar lung transplantation Donor lobectomy (lung) for transplantation, living donor Simultaneous pancreas kidney transplantation Solid organ(s), complete or segmental, single organ or combination of organs; deceased or living donor (s), procurement, 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 ICD 10 CODES ARE FOR INFORMATIONAL PURPOSES ONLY ICD 10 DESCRIPTION CODES A A40.9 Streptococcal sepsis B16.0, B16.2 Acute hepatitis B with hepatic coma B16.1, B16.9, Acute hepatitis B without mention of hepatic coma B19.10 B17.10 Acute hepatitis C without hepatic coma B17.11 Acute hepatitis C with hepatic coma B18.0- B18.1 Chronic viral hepatitis B B18.2 Chronic viral hepatitis C B19.11 Unspecified viral hepatitis B with hepatic coma B B19.21 Unspecified viral hepatitis C C22.0 Liver cell carcinoma C22.1 Intrahepatic bile duct carcinoma C22.2 Hepatoblastoma C C34.92 Malignant neoplasm of bronchus and lung C96.6 Unifocal Langerhans-cell histiocytosis D37.6 Neoplasm of uncertain behavior of liver, gallbladder and bile ducts D86.0 Sarcoidosis of lung D D Graft-versus-host disease E Diabetes mellitus due to underlying condition with hypoglycemia without coma

19 Page 19 of 23 E E10.9 E E11.9 E E13.29 E15 E E16.2 E E72.9 Type 1 diabetes mellitus Type 2 diabetes mellitus Other specified diabetes mellitus with kidney complications Nondiabetic hypoglycemic coma Hypoglycemia Disorders of aromatic amino-acid metabolism, disorders of branched-chain aminoacid metabolism and fatty-acid metabolism and other disorders of amino-acid metabolism E79.0 Hyperuricemia without signs of inflammatory arthritis and tophaceous disease E80.0 Hereditary erythropoietic porphyria E83.01 Wilson's disease E83.10, E83.19 Other and unspecified disorders of iron metabolism E E Hemochromatosis E E84.9 Cystic fibrosis E85.1 Neuropathic heredofamilial amyloidosis E E86.9 Volume depletion E87.2 Acidosis E88.01 Alpha-1-antitrypsin deficiency E89.1 Postprocedural hypoinsulinemia I21.01 Acute myocardial infarction and other acute forms of ischemic heart disease I24.9 I I Chronic ischemic heart disease I I25.9 Other and unspecified forms of chronic ischemic heart disease I I27.9 Pulmonary heart disease I I39 Nonrheumatic mitral valve, aortic valve, tricuspid valve and pulmonary valve disorders I I43 Cardiomyopathy I I49.9 I I50.9 Cardiac dysrhythmias Heart failure

20 Page 20 of 23 I51.4 Myocarditis, unspecified I82.0 Budd-Chiari syndrome I I82.91 Embolism and thrombosis of other specified veins J40 - J47.9 Chronic lower respiratory diseases J61 Pneumoconiosis due to asbestos and other mineral fibers J J67.9 Allergic alveolitis J84.10 Pulmonary fibrosis, unspecified J J Idiopathic interstitial pneumonia J84.81 Lymphangioleiomyomatosis J84.89 Other specified interstitial pulmonary diseases J99 K70.2 Alcoholic fibrosis and sclerosis of liver K K70.31 Alcoholic cirrhosis of liver K K73.8 Chronic hepatitis, not elsewhere classified K74.3 Primary biliary cirrhosis K74.4 Secondary biliary cirrhosis K74.69 Other cirrhosis of liver K75.4 Autoimmune hepatitis K K75.9 Other specified inflammatory liver diseases K76.5 Hepatic veno-occlusive disease K76.81 Hepatopulmonary syndrome K83.0 Cholangitis K83.1 Obstruction of bile duct K K86.1 Chronic pancreatitis M31.0 Hypersensitivity angiitis M M35.9 Diffuse diseases of connective tissue N18.5 Chronic kidney disease, stage 5 N18.6 End stage renal disease O O90.9 Other complications of the puerperium, not elsewhere classified P P27.9 Chronic respiratory disease originating in the perinatal period Q Q28.9 Congenital malformations of the circulatory system

21 Page 21 of 23 Q33.0 Congenital cystic lung Q33.3 Agenesis of lung Q33.4 Congenital bronchiectasis Q33.6 Congenital hypoplasia and dysplasia of lung Q44.2 Atresia of bile ducts Q44.3 Congenital stenosis and stricture of bile ducts Q44.6 Cystic disease of liver R78.71 Abnormal lead level in blood R78.79 Finding of abnormal level of heavy metals in blood R78.89 Finding of other specified substances, not normally found in blood R79.0 Abnormal level of blood mineral R79.9 Abnormal finding of blood chemistry, unspecified T T Complications of heart transplant T T86.39 Complications of heart-lung transplant T T86.49 Complications of liver transplant T T Complications of lung transplant Z79.4 Long term (current) use of insulin Z Acquired total absence of pancreas Z Acquired partial absence of pancreas Z90.49 Acquired absence of other specified parts of digestive tract Z94.1 Heart transplant status REVENUE CODES DESCRIPTION 0360 Operating Room Services-General; Hospital; Outpatient REFERENCE STATEMENT: Analyses of the scientific and clinical references cited below were conducted and utilized by the Johns Hopkins HealthCare LLC (JHHC) Medical Policy Team during the development and implementation of this medical policy. Per NCQA standards, the Medical Policy Team will continue to monitor and review any newly published clinical evidence and adjust the references below accordingly if deemed necessary. REFERENCES: Aetna. (2017). Medical Coverage Policy: Heart Transplantation. Medical Retrieved:

22 Page 22 of 23 Aetna. (2017). Medical Coverage Policy: Kidney Transplantation. Medical Retrieved: Aetna. (2017). Medical Coverage Policy: Liver Transplantation. Medical Retrieved: Aetna. (2017) Medical Coverage Policy: Lung Transplantation. Medical Retrieved: Aetna. (2017). Medical Coverage Policy: Pancreas Kidney Transplantation. Medical Policy Number Retrieved: Aetna. (2017). Medical Coverage Policy: Pancreas Transplantation Alone (PTA) and Islet Cell Transplantation. Medical Retrieved: Anthem. (2017). Medical Coverage Policy: Heart/Lung Transplantation. TRANS Retrieved: Centers for Medicare and Medicaid (CMS).Medicare Program; Hospital Conditions of Participation: Requirements for Approval and Re-Approval of Transplant Centers To Perform Organ Transplants; Final Rule. 42 CFR Parts 405, 482, 488, and 498, at: Centers for Medicare and Medicaid (CMS). National Coverage Determinations (NCD) Chapter 1, Part 3, (Sections ), 190-Pathology and Laboratory, Histocompatibility Testing, Rev.1. 10/03.03, Typing or Matching for Preparation for Kidney, Bone Marrow Transplantation and Blood Platelet Transfusions, at: CIGNA. (2017). Medical Coverage Policy: Heart, Lung, Heart-Lung Transplantation. Coverage Retrieved: COMAR Transplant Centers. Retrieved: Hachem, R., Edwards, L., et al. (2008). The impact of induction on survival after lung transplantation: an analysis of the International Society for Heart and Lung Transplantation Registry. Clin. Transplant, 22(5), Hayes, Inc. (2010). Medical Technology Directory: Lung Transplantation Induction and Maintenance Immunosuppressive Therapy. Retrieved: Hayes, Inc. (2008). Medical Technology Directory: Simultaneous Pancreas-Kidney (SPK) Transplantation in Diabetic Patients. Retrieved: