Clinical Policy Title: Lung transplants

Transcription

1 Clinical Policy Title: Lung transplants Clinical Policy Number: CCP.1202 Effective Date: January 1, 2016 Initial Review Date: October 21, 2015 Most Recent Review Date: October 2, 2018 Next Review Date: October 2019 Policy contains: Deceased donor lung transplant. Living donor lung transplant. Lung transplant. Pulmonary transplant. Related policies: CCP.1138 CCP.1210 CCP.1209 CCP.1211 CCP.1034 CCP.1201 CCP.1212 Corneal transplantation (keratoplasty) Heart valve transplantation Bone marrow transplantation for cancer other than breast cancer Kidney transplantation Heart transplantation Pancreas transplantation Liver transplantation ABOUT THIS POLICY: Select Health of South Carolina has developed clinical policies to assist with making coverage determinations. Select Health of South Carolina s clinical policies are based on guidelines from established industry sources, such as the Centers for Medicare & Medicaid Services (CMS), state regulatory agencies, the American Medical Association (AMA), medical specialty professional societies, and peer-reviewed professional literature. These clinical policies along with other sources, such as plan benefits and state and federal laws and regulatory requirements, including any state- or plan-specific definition of medically necessary, and the specific facts of the particular situation are considered by Select Health of South Carolina when making coverage determinations. In the event of conflict between this clinical policy and plan benefits and/or state or federal laws and/or regulatory requirements, the plan benefits and/or state and federal laws and/or regulatory requirements shall control. Select Health of South Carolina s clinical policies are for informational purposes only and not intended as medical advice or to direct treatment. Physicians and other health care providers are solely responsible for the treatment decisions for their patients. Select Health of South Carolina s clinical policies are reflective of evidence-based medicine at the time of review. As medical science evolves, Select Health of South Carolina will update its clinical policies as necessary. Select Health of South Carolina s clinical policies are not guarantees of payment. Coverage policy Select Health of South Carolina considers lung transplants to be clinically proven and, therefore, medically necessary in cases of end-stage lung disease with less than a 50 percent chance of a 2- to 3-year survival and with a functional status of New York Heart Association class III or IV that cannot be successfully treated with alternative medical and surgical therapies. Categories of lung disease for which transplant may be indicated include, but are not limited to advanced cystic fibrosis, idiopathic pulmonary fibrosis, and primary pulmonary hypertension (Mehra, 2016; Hook, 2012; Orens, 2006).. Limitations: Select Health of South Carolina considers lung transplantation for all other conditions not listed in the coverage policy above to be not medically necessary. 1

2 Absolute contradictions for lung transplant include, but are not limited to: 1. Malignancy in the last two years, with the exception of cutaneous squamous and basal cell tumors. 2. Untreatable advanced dysfunction of another major organ system (e.g., heart, liver, or kidney). 3. Coronary artery disease not amenable to percutaneous intervention or bypass grafting, or associated with significant impairment of left ventricular function. 4. Non-curable chronic extrapulmonary infection including chronic active viral hepatitis B and C. 5. Significant chest wall/spinal deformity. 6. Documented nonadherence or inability to follow through with medical therapy or office follow-up. 7. Untreatable psychiatric or psychologic condition associated with the inability to cooperate or comply with medical therapy. 8. Absence of a consistent or reliable social support system. 9. Substance addiction that is either active or within the last six months (Orens, 2006). Relative contradictions for lung transplant include, but are not limited to: 1. Critical or unstable clinical condition (e.g., shock, mechanical ventilation or extra-corporeal membrane oxygenation). 2. Severely limited functional status with poor rehabilitation potential. 3. Colonization with highly resistant or highly virulent bacteria, fungi, or mycobacteria. 4. Severe obesity (BMI > 35 kg/m 2 ). 5. Severe or symptomatic osteoporosis. 6. Mechanical ventilation, except for carefully selected candidates without other acute or chronic organ dysfunction, who are able to actively participate in a meaningful rehabilitation program. 7. Other medical conditions that have not resulted in end-stage organ damage, such as diabetes mellitus, systemic hypertension, peptic ulcer disease, or gastroesophageal reflux, unless they have been optimally treated before transplantation. 8. Patients with coronary artery disease who have undergone percutaneous intervention before transplantation or coronary artery bypass grafting concurrent with the procedure (Orens, 2006). Alternative covered services: Maximum medical management of Chronic Obstructive Pulmonary Disease. Maximum medical management of pulmonary arterial hypertension. Background Lung transplantation or pulmonary transplantation is a surgical procedure in which a patient's diseased lung(s) are partially or totally replaced by healthy lungs from a donor. Donor lungs can be retrieved from a living donor or a deceased donor. A living donor can only donate one lung lobe. With some lung diseases, a recipient may only need to receive a single lung. With other lung diseases, such as cystic fibrosis, it is imperative that a recipient receive two lungs. While lung transplants carry certain associated risks, like lifethreatening complications and infections, especially in the first year after surgery, they can also extend life 2

3 expectancy and enhance the quality of life for end-stage pulmonary patients (National Heart, Lung, and Blood Institute, 2016). The annual number of lung transplants worldwide has risen from 89 to more than 3,700 from 1988 to 2012 (Hachem, 2015; Yusen, 2015). In 2015, of the 4234 procedures reported to the International Society of Heart & Lung Transplantation, 93.2 percent were performed in Europe and North America, 3.5 percent had a previous transplant, 2.2 percent were under age 18, and 18.8 percent were 65 and older. Another 46 persons received a heart/lung transplant in 2015 (International Society of Heart & Lung Transplantation, 2017). On September 19, 2017, a total of 1365 registered persons were awaiting a lung transplant, plus an additional 41 for heart/lung transplants (U.S. Health Services Resource Administration, 2017). By mid-2017, 30,800 lung transplants had been performed in the U.S. (Lung Institute, 2014). Current oneyear and five-year survival rates of lung transplants in the U.S. are 78 and 51 percent, respectively. Median survival for bilateral lung transplants are greater than that for single lung transplants (6.6 versus 4.6 years) (Lung Institute, 2014). There is waiting list for lung transplants, which prompted a 2005 change in the U.S. to assign priority to candidates who have a higher predicted waiting list mortality rate and a greater predicated survival benefit from transplantation, calculated as the Lung Allocation Score. Since the change was implemented, waiting time and waiting list mortality rates among transplant candidates have plunged (Hook, 2012). Transplant physicians and surgeons representing the International Society of Heart and Lung Transplantation, the American Society of Transplant Physicians, the American Thoracic Society, the European Respiratory Society, and the Thoracic Society of Australia and New Zealand have agreed on acceptable guidelines for candidates for lung transplantation (Orens, 2006). Lung transplantation should be considered for patients with advanced lung disease whose clinical status has progressively declined despite maximal medical or surgical therapy. Candidates are usually symptomatic during activities of daily living and have a limited expected survival over the next two years. In addition, the ideal candidate should be free of significant other organ dysfunction and extrapulmonary manifestations of a systemic disease. Guidelines for recipient selection have been developed by the American Thoracic Society and the International Society of Heart and Lung Transplantation (Orens, 2006). A deceased donor, also known as cadaveric donor, is the most common donor source used for lung transplantation. From 1993 to 2006 in the U.S., a total of 12,168 lungs were transplanted using deceased donors lungs, compared to only 460 live donors (Yusen, 2014). Use of a live donor as a source for lung transplantation was initiated in 1993 due to the higher demand than supply for patients waiting for lung transplantation. Deceased donor transplantation is preferred to avoid the risk to two healthy donors (Solomon, 2010). In 1995, the United Network for Organ Sharing changed the method for allocating donated cadaver lungs for individuals over age 12 by assigning each candidate a lung allocation score based on survival benefit and urgency rather than waiting time (Mulligan, 2008). In contrast, allocation to children under age 12 3

4 continues to be based on waiting time. Preferential transplantation of sicker patients has not resulted in an increase in early mortality following transplantation (Kotloff, 2010). Searches Select Health of South Carolina searched PubMed and the databases of: UK National Health Services Centre for Reviews and Dissemination. Agency for Healthcare Research and Quality s National Guideline Clearinghouse and other evidence-based practice centers. The Centers for Medicare & Medicaid Services. We conducted searches on August 10, Search terms were: MeSH chronic obstructive pulmonary disease, frailty, interstitial lung disease, lung transplantation, obesity, pulmonary arterial hypertension, sarcopenia, and cystic fibrosis. We included: Systematic reviews, which pool results from multiple studies to achieve larger sample sizes and greater precision of effect estimation than in smaller primary studies. Systematic reviews use predetermined transparent methods to minimize bias, effectively treating the review as a scientific endeavor, and are thus rated highest in evidence-grading hierarchies. Guidelines based on systematic reviews. Economic analyses, such as cost-effectiveness, and benefit or utility studies (but not simple cost studies), reporting both costs and outcomes sometimes referred to as efficiency studies which also rank near the top of evidence hierarchies. Findings A number of guidelines affecting lung transplants have been published. The definitive work addressing criteria on selecting lung transplant patients, and contraindications against performing surgery, was written by the Pulmonary Scientific Council of the International Society for Heart and Lung Transplantation, and its recommendations are reflected in the coverage policy portion of this document (Orens, 2006). Ten years later, an update to these guidelines was published, addressing each of the major selection criteria; some were changed, many remained unchanged (Mehra, 2016). A review of the 2006 guideline was helpful in identifying contraindications for transplants, and for making changes to selection criteria (Hook, 2012). A report from the International Society for Heart and Lung Transplantation made similar recommendations for transplant indications in 2016 (Lund, 2016). The same group of international experts that wrote the 2006 guidelines recently developed a consensus opinion on timing of referral and listing for lung transplantation. The panel included lung allocation scores and expanded indications for lung transplant; the key factor is to make an early referral to allow for proper evaluation and patient education (Weill, 2015). 4

5 The National Institute for Health and Care Excellence has also published a guideline on selection of patients for lung transplantation (National Institute for Health and Care Excellence, 2006). The number of persons in the U.S. receiving lung transplants in 2010, by most common diagnosis, includes the following (Hook, 2012): DIAGNOSIS CASES (%) Interstitial lung disease 683 (39) COPD/emphysema 413 (23) Cystic fibrosis 218 (12) Lung retransplantation 80 ( 5) Sarcoidosis 54 ( 3) All other 170 (18) TOTAL 1770 (100) The number of Americans awaiting a lung transplant rose 10 percent from 1997 to 2006, but the median time-to-transplant for listed patients decreased by 87 percent, from 1053 to 132 days. The institution of a new system in May 2005 to allocate resources based on survival benefit and urgency rather than waiting time is credited with recent changes (Mulligan, 2008). Lung re-transplants are now being performed in increasing numbers. The median survival rate for those with re-transplants is lower than that of transplants (2.6 versus 5.6 years), and the five-year survival rate is lower as well, at 34.5 versus 53.3 percent. Careful selection of candidates for re-transplant is advised (Thomas, 2015). The relative proportion of bilateral procedures as a part of all lung transplantations has been recently rising (Yusen, 2013). However, due to conflicting, low-quality evidence, researchers at Washington University in St. Louis could not recommend a particular procedure, when either is possible (Puri, 2015). One study of 8,778 lung transplant patients showed a significantly lower survival (P <.001) for single lung transplants. Nearly 92 percent of the patients had a lung allocation score over 75, representing patients more likely to benefit from a lung transplant (Black, 2014). A study of 580 Canadian lung transplant patients with cystic fibrosis found the five- and ten- year survival rates to be 67 and 50 percent, with significantly higher death rates for young and old patients, plus those with pancreatic sufficiency or B cepacia infection (Stephenson, 2015). A study comparing single and bilateral lung transplant outcomes (n=111) found more comorbid conditions and longer lengths of hospital stay after bilateral lung transplants, but no difference in survival between single and bilateral procedures (De Oliveira, 2012). A systematic review of six studies (n=1305) showed enhancement in quality of life for lung transplant candidates in five of the studies, using the SF-36 questionnaire and the six-minute walk test (Hoffman, 2017). A large systematic review of 73 studies showed that quality of life after lung transplants is 5

6 enhanced, especially during the first year after the procedure; more after bilateral transplants; more in physical health and functioning; and more than after heart and liver transplants (Singer, 2013). A study of quality of life changes for 136 patients one year after lung transplant, using the Medical Outcomes Study 36-Item Short-Form Health Survey documented a significant increase from baseline (P <.0001). The improvement in mental component score was insignificant (Finlen Copeland, 2013). Some studies have identified factors that increase the risk of mortality in lung transplant patients. A review of 13 cohort studies (n=40,742) reviewed weight at surgery, and found significantly elevated relative risks compared to normal weight for underweight (1.36), obesity (1.90), and overweight plus underweight (1.36) (Upala, 2016). Clinical risk factors identified in 13 studies (n=10,042) of primary graft dysfunction, the main cause of elevated morbidity and mortality, include female gender, African American, idiopathic pulmonary fibrosis, sarcoidosis, primary pulmonary hypertension, elevated body mass index, and use of cardiopulmonary bypass (Liu, 2014). Non-adherence to medical regimens after transplants was the focus of a systematic review. While nonadherence rates varied across risk factors, they were not a significant factor in mortality (Hu, 2017). While lung transplantations from deceased donors remain a small minority of all such procedures, one meta-analysis of nine studies (n=301) compared mortality deceased and live (conventional) donors. One year survival ranged from 50 to 100 percent among transplants from deceased donors, compared to 72 to 88 percent from live donors. One of the studies included nearly half of the transplants (138 of 301); oneyear survival was significantly lower (65.1 versus 84.1 percent for live transplants, P <.001) (Eberlein, 2017). A comparison of lungs transplanted after >12 hours ex-vivo preservation by splitting one cold ischemic time into two shorter ones (n=97, average minutes) with lungs transplanted with single waits <12 hours (n=809, average minutes) was made. Median length of stay for both hospital and intensive-care unit length of stay did not differ significantly between groups (23.0 versus 25.5 days, P =.60, and 4.0 versus 4.0 days, P =.53). Primary graft dysfunction grade was not significantly different between the groups 72 hours after transplantation (P =.85), and there was no significant difference in survival (P =.61) (Yeung, 2017). Policy updates: A total of three peer-reviewed references were added to, and two guidelines/other and one peer-reviewed reference removed from this policy in August,2018. Summary of clinical evidence: 6

7 Citation Stephenson (2015) Survival after lung transplants Black (2014) Impact of Lung Allocation Score on survival rates five and 10 years after lung transplant Liu (2014) Content, Methods, Recommendations Key points: Study of 580 Canadians with cystic fibrosis receiving a lung transplant from Post-transplant 1-, 3-, and 5-year survival was 87.8 percent (%) 66.7%, and 50.2%. Median post-transplant survival was 3.3 and years in patients with and without B cepacia infection, which was significant (hazard ratio = 2.63). Pancreatic sufficiency and youngest/oldest ages had the poorest survival were significant negative predictors of survival. Risk of death after transplant for patients infected with B cepacia was highest within the first year (hazard ratio (HR) = 6.29) but remained elevated >1 year (HR = 1.92). Key points: Study of survival rate for 8778 lung transplant patients from January 2005 to June United Network for Organ Sharing Thoracic Transplant Database used for analysis. 92% (8,050) patients had a Lung Allocation Score less than 75 (higher scores suggest patient is more likely to benefit from transplant). Survival significantly lower (P <0.001) in patients with a high Lung Allocation Score who received a single lung vs. bilateral transplant, a much greater difference than in the low Lung Allocation Score population. Key points: Risk factors for primary graft dysfunction Systematic review + meta-analysis of 13 studies (n=10,042) on risk factors for primary graft dysfunction, the main cause of early morbidity/mortality after lung transplant; primary graft dysfunction has nearly a four-fold increase in mortality after lung transplant. Significantly associated with risk of primary graft dysfunction were female gender (Odds Ratio (OR) 1.38) African American (OR 1.82), idiopathic pulmonary fibrosis (OR 1.78), sarcoidosis (OR 4.25), primary pulmonary hypertension (OR 3.73), Body Mass Index 25 kg/m2 (OR 1.82), and use of cardiopulmonary bypass (OR 2.29). Not significantly associated with risk of pulmonary graft dysfunction were age, cystic fibrosis, secondary pulmonary hypertension, intra-operative inhaled nitric oxide, or lung transplant type (single or bilateral). Singer (2013) Key points: Quality of life trends after lung transplant Systematic review of 73 studies on quality of life after lung transplant. Improvements after surgery were greater after bilateral transplants. Improvements were greater than after heart and liver transplants. The greatest improvements were in physical health and functioning domains. Improvements were strongest in the first year after surgery, less stable patterns after. Lung transplant quality of life less studied than heart, liver, kidney transplants. References Professional society guidelines/other: Hachem RR. Lung transplantation: an overview. Up to Date, last updated November 2, Accessed August 10, Lung Institute. Lung transplant survival rates. Lung Institute, November 21, Accessed,August 10,

8 International Society of Heart & Lung Transplantation. International Thoracic Organ Transplant Registry Quarterly Reports. Addison TX: International Society of Heart & Lung Transplantation, Accessed August 10, Kotloff RM. : Mason RJ, Broaddus VC, et al. Lung transplantation. In Murray & Nadel s textbook of respiratory medicine. 5th ed. New York: W.B. Saunders Company; Accessed August 10, Mehra MR, Canter CE, Hannan MM, et al. The 2016 International Society for Heart Lung Transplantation listing criteria for heart transplantation: A 10-year update. J Heart Lung Transplant. 2016;35(1):1-23. Doi: /j.healun National Institute for Health and Care Excellence. Living donor lung transplantation for end stage lung disease. London: National Institute for Health and Care Excellence, May, Accessed September 21, Orens JB, 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 Trans. 2006;25(7): U.S. Centers for Medicare and Medicaid Services. New Medicare Hospital Conditions of Participation for Transplant Centers. March 22, 2007(a). Accessed August 10, U.S. Centers for Medicare and Medicaid Services. Medicare Program; Hospital Conditions of Participation: Requirements for Approval and Re-Approval of Transplant Centers To Perform Organ Transplants; Final Rule March 30, 2007(b). Certification/GuidanceforLawsAndRegulations/Downloads/TransplantFinalLawandReg.pdf. Accessed August 10, U.S. Health Services Resource Administration. Organ Procurement and Registration Network, United Network for Organ Sharing. Richmond VA: UNOS, Accessed August 10, U.S. National Heart, Lung, and Blood Institute. Lung transplant. Bethesda MD: National Heart, Lung, and Blood Institute, last updated December 9, Accessed August 10,

9 Weill D, Benden C, Corris PA, et al. A consensus document for the selection of lung transplant candidates: an update from the Pulmonary Transplantation Council of the International Society for Heart and Lung Transplantation J Heart Lung Transplant. 2015;34(1):1 15. Doi: /j.healun Peer-reviewed references: Black MC, Trivedi J, Schumer EM, et al. Double lung transplants have significantly improved survival compared with single lung transplants in high lung allocation score patients. Ann Thorac Surg ;98(5): Doi: /j.athoracsur De Oliveira NC, Osaki S, Maloney J, Cornwell RD, Meyer KC. Lung transplant for interstitial lung disease: outcomes for single versus bilateral lung transplantation. Interact Cardiovasc Thorac Surg. 2012;14(3): Doi: /icvts/ivr085. Eberlein M, Reed RM, Chahla M, et al. Lobar lung transplantation from deceased donors: A systematic review. World J Transplant. 2017;7(1): Doi: /wjt.v7.i1.70. Finlen Copeland CA, Vock DM, Pieper K, Mark DB, Palmer SM. Impact of lung transplantation on recipient quality of life: a serial, prospective, multicenter analysis through the first posttransplant year. Chest. 2013;143(3): Doi: /chest Hoffman M, Chaves G, Ribeiro-Samora GA, Britto RR, Parreira VF. Effects of pulmonary rehabilitation in lung transplant candidates: a systematic review. BMJ Open. 2017;7(2):e Doi: /bmjopen Hook JL, Lederer DJ. Selecting lung transplant candidates: where do current guidelines fall short? Expert Rev. Respir Med. 2012;6(1): Doi: /ers Hu L, Lingler JH, Sereika SM, et al. Nonadherence to the medical regimen after lung transplantation: a systematic review. Heart Lung. 2017;46(3): Doi: /j.hrtlng Liu Y, Liu Y, Su L, Jiang SJ. Recipient-related clinical risk factors for primary graft dysfunction after lung transplantation: a systematic review and meta-analysis. PLoS One. 2014;9(3):e Doi: /journal.pone Lund LH, Edwards LB, Dipchand AI, et al. The Registry of the International Society for Heart and Lung Transplantation: Thirty-third Official Adult Lung and Heart-Lung Transplantation report--2016; Focus Theme: Primary diagnostic indications for transplant. J Heart Lung Transplant 2016; 35(10): Doi: /j.healun Mulligan MS, Shearon TH, Weill D, Pagani FD, Moore J, Murray S. Heart and lung transplantation in the United States, Am J Transplant. 2008;8(4 Pt 2): Doi: /j x. 9

10 Puri V, Patterson GA, Meyers BF. Single versus bilateral lung transplantation: do guidelines exist? Thorac Surg Clin. 2015;25(1): Doi: /j.thorsurg Singer J, Chen J, Blanc PD, Leard LE, Kukreja J, Chen H. A thematic analysis of quality of life in lung transplant: the existing evidence and imlications for future directions. Am J Transplant. 2013;13(4): Doi: /ajt Stephenson AL, Sykes J, Berthiaume Y, et al. Clinical and demographic factors associated with post lung transplantation survival in individuals with cystic fibrosis. J Heart Lung Transplant. 2015;34(9): Doi: /j.healun Thomas M, Belli EV, Rawal B, Agnew RC, Landolfo KP. Survival after lung retransplantation in the United States in the current era (2004 to 2013): better or worse? Ann Thorac Surg. 2015;100(2): Doi: /j.athoracsur Upala S, Panichsillapakit T, Wijarnpreecha K, Jaruvongvanich V, Sanquankeo A. Underweight and obesity increase the risk of mortality after lung transplantation: a systematic review and meta-analysis. Transpl Int. 2016;29(3): Doi: /tri Yeung JC, Krueger T, Yasufuku K, et al. Outcomes after transplantation of lungs preserved for more than 12 h: a retrospective study. Lancet Respir Med. 2017;5(2): Doi: /S (16)30323-X. Yusen RD, Christie JD, Edwards LB, et al. 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;32: Doi: /j.healun Yusen RD, Hong BA, Messersmith EE, et al. Morbidity and mortality of live lung donation: results from the RELIVE study. Am J Transplant. 2014;14(8): Centers for Medicare & Medicaid National Coverage Determinations: No National Coverage Determinations identified as of the writing of this policy. 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 (Centers for Medicare & Medicaid Services, 2007a). 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 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 (Centers for Medicare & Medicaid Services, 2007b). Local Coverage Determinations: 10

11 No Local Coverage Determinations identified as of the writing of this policy. Commonly submitted codes Below are the most commonly submitted codes for the service(s)/item(s) subject to this policy. This is not an exhaustive list of codes. Providers are expected to consult the appropriate coding manuals and bill accordingly. CPT Code Description Comment 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 ICD 10 Code Description Comments C96.6 Unifocal Langerhans-cell histiocytosis [eosinophilic granuloma] D86.0 Sarcoidosis of lung D D Graft-versus-host disease E E84.9 Cystic fibrosis E88.01 Alpha-1-antitrypsin deficiency I27.0 Primary pulmonary hypertension J42 Unspecified chronic bronchitis [bronchiolitis obliterans] J J43.9 Emphysema J J44.9 Chronic obstructive pulmonary disease J J47.9 Bronchiectasis J61 Pneumoconiosis due to asbestos and other mineral fibers J J67.9 Allergic alveolitis J84.10 Pulmonary fibrosis, unspecified J84.81 Lymphangioleiomyomatosis [with end-stage pulmonary disease] J84.89 Other specified interstitial pulmonary diseases J99 Respiratory disorders in diseases classified elsewhere M34.81 Systemic sclerosis with lung involvement Q21.8 Other congenital malformations of cardiac septa [Eisenmenger's defect or complex] Q33.0 Congenital cystic lung Q33.3 Agenesis of lung Q33.4 Congenital bronchiectasis Q33.6 Congenital hypoplasia and dysplasia of lung T T Complications of lung transplant HCPCS Level II Code N/A Description Comment 11