Clinical Policy Title: Lung transplants

Transcription

1 Clinical Plicy Title: Lung transplants Clinical Plicy Number: Effective Date: January 1, 2016 Initial Review Date: Octber 21, 2015 Mst Recent Review Date: Nvember 18, 2015 Next Review Date: Octber 2016 Plicy cntains: Lung transplants. Related plicies: CP# Crneal transplants (keratplasty) CP# Heart valves transplants CP# Bne marrw transplants CP# Kidney transplants CP# Heart transplants CP# Pancreas transplants ABOUT THIS POLICY: Keystne VIP Chice has develped clinical plicies t assist with making cverage determinatins. Keystne VIP Chice s clinical plicies are based n guidelines frm established industry surces, such as the Centers fr Medicare & Medicaid Services (CMS), state regulatry agencies, the American Medical Assciatin (AMA), medical specialty prfessinal scieties, and peer-reviewed prfessinal literature. These clinical plicies alng with ther surces, such as plan benefits and state and federal laws and regulatry requirements, including any state- r plan-specific definitin f medically necessary, and the specific facts f the particular situatin are cnsidered by Keystne VIP Chice when making cverage determinatins. In the event f cnflict between this clinical plicy and plan benefits and/r state r federal laws and/r regulatry requirements, the plan benefits and/r state and federal laws and/r regulatry requirements shall cntrl. Keystne VIP Chice s clinical plicies are fr infrmatinal purpses nly and nt intended as medical advice r t direct treatment. Physicians and ther health care prviders are slely respnsible fr the treatment decisins fr their patients. Keystne VIP Chice s clinical plicies are reflective f evidence-based medicine at the time f review. As medical science evlves, Keystne VIP Chice will update its clinical plicies as necessary. Keystne VIP Chice s clinical plicies are nt guarantees f payment. Cverage plicy Keystne VIP Chice cnsiders lung transplants t be clinically prven and therefre, medically necessary when any, but nt limited t, f the fllwing criteria are met: Obstructive lung disease (e.g., emphysema, including alpha 1-antitrypsin deficiency; chrnic bstructive pulmnary disease [COPD]; brnchilitis bliterans; and brnchiectasis) in patients wh meet any f the fllwing: Disease is prgressive, despite maximal treatment including medicatin, pulmnary rehabilitatin and xygen therapy. Individual is nt a candidate fr endscpic r lung vlume reductin surgery (LVRS). Simultaneus referral f individual with COPD fr bth lung transplant and LVRS evaluatin is apprpriate. BODE index f 5 6: 1

2 The Bde Index is a cmpsite scre f bdy mass index (BMI), airway bstructin (percent predicted FEV1) (O), dyspnea (D), and exercise capacity (E). The Bde Index calculatr is available at PaCO2 > 50 mmhg r 6.6 kilpascals (kpa ) and/r PaO2 < 60 mmhg r 8 kpa. FEV1 < 25 percent predicted. Brnchpulmnary dysplasia. Cngenital heart disease (Eisenmenger's defect r cmplex) when any f the fllwing is met: Signs f right ventricular failure (i.e., prgressive hepatmegaly, ascites, marked deteriratin in functinal capacity (New Yrk Heart Assciatin [NYHA] Class III). Pulmnary hypertensin with mean pulmnary artery pressure greater than 20 mm Hg by right heart catheterizatin. Cystic fibrsis in patients wh display any f the fllwing: FEV1 that has fallen t 30 percent r individual with advanced disease with rapidly falling FEV1 despite ptimal therapy (particularly in a female patient), infected with nntuberculus mycbacterial (NTM) disease r BMI cepacia cmplex and/r with diabetes. A six-minute walk distance < 400 m. Develpment f pulmnary hypertensin in the absence f a hypxic exacerbatin (as defined by a systlic pulmnary arterial pressure (PAP) > 35 mm Hg n echcardigraphy r mean PAP > 25 mm Hg measured by right heart catheterizatin). Clinical decline characterized by increasing frequency f exacerbatins assciated with any f the fllwing: An episde f acute respiratry failure requiring nninvasive ventilatin. Increasing antibitic resistance and pr clinical utcmes. Recvery frm exacerbatins. Wrsening nutritinal status despite supplementatin. Pneumthrax. Life-threatening hemptysis despite brnchial emblizatin. Primary pulmnary hypertensin in patients wh meet any f the fllwing: NYHA Functinal Class III r IV symptms during escalating therapy. Rapidly prgressive disease (assuming weight and rehabilitatin cncerns are nt present). Use f parenteral targeted pulmnary arterial hypertensin (PAH) therapy regardless f symptms r NYHA Functinal Class. Knwn r suspected pulmnary ven-cclusive disease (PVOD) r pulmnary capillary hemangimatsis. Restrictive lung disease (e.g., idipathic pulmnary fibrsis, desquamative interstitial fibrsis, pstchemtherapy, allergic alvelitis, systemic sclersis [sclerderma], cllagen vascular disease, asbestsis r esinphilic granulma). Pulmnary fibrsis in patients wh meet any f the fllwing: Presence f cr pulmnale (indicative f severe pulmnary fibrsis) r pulmnary hypertensin. Diffusing capacity fr carbn mnxide (DLCO) is less than 60 percent expected. Ttal lung capacity (TLC) less than 70 percent expected. Sarcidsis in patients wh meet any f the fllwing: Presence f cr pulmnale (indicative f severe pulmnary fibrsis) r pulmnary hypertensin. Ttal lung capacity less than 70 percent predicted. Diffusin capacity (DLCO) less than 60 percent expected. 2

3 Lymphangileimymatsis (LAM) with end-stage pulmnary disease. Graft versus hst disease r failed primary lung graft. Including the pre-transplant, and pst-discharge services, and the treatment f cmplicatins as apprved by the plan. Limitatins: Keystne VIP Chice cnsiders all ther cnditins diseases nt listed in the cverage plicy abve fr lung transplantatins, are nt medically necessary. Nte: The fllwing CPT/HCPCS cdes are nt included in the Pennsylvania Medicaid fee schedule: Backbench standard preparatin f cadaver dnr lung allgraft prir t transplantatin, including dissectin f allgraft frm surrunding sft tissues t prepare pulmnary venus/atrial cuff, pulmnary artery, and brnchus; unilateral Backbench standard preparatin f cadaver dnr lung allgraft prir t transplantatin, including dissectin f allgraft frm surrunding sft tissues t prepare pulmnary venus/atrial cuff, pulmnary artery, and brnchus; bilateral Relative cntraindicatins fr adults and children include, but may nt be limited t: Age apprpriateness: 65 years f age fr single lung 65 years f age fr duble lung transplant. 55 years f age fr heart and lung transplant. Active smker (less than six mnths since quitting). Active substance abuse. Chrnic mechanical ventilatin (unless tlerating three hurs f physical therapy/day and is free f bacterial clnizatin). Previus lung transplant (rare exceptins fr Jhn Hpkins Hspital primary transplant patients). Severe diffuse crnary artery disease (especially with pr ejectin fractin EF). End-stage renal disease (creatinine clearance < 40 mg/min). End-stage liver disease. Bne marrw dysfunctin. Human immundeficiency virus (HIV). (HIV inclusin criteria: CD4 cunt greater than r equal t 200 cells/ml fr at least 6 mnths, undetectable HIV viremia fr 6 mnths, adherence t HAART regimen fr greater than 6 mnths) Severe lcal r systemic infectin. Severe neurlgic deficits. Untreatable psychiatric. Lung transplantatin shuld nt be ffered t adults with a recent histry f malignancy. Mrbid besity (BMI > 35). Severe malnutritin/cachexia. Chrnic prednisne use > 20 mg/day. Symptmatic steprsis. 3

4 Psychiatric r scial prblems (including nncmpliance). Financial prblems (n prescriptin cverage). Previus thracic surgery/prcedure. Lack f family r scial supprt. Cancer in the last five years, except lcalized skin (never melanma). Clnizatin with resistant rganisms. Alternative cvered services: Maximum medical management f COPD. Maximum medical management f pulmnary arterial hypertensin. Backgrund Lung transplantatin r pulmnary transplantatin is a surgical prcedure in which a patient's diseased lungs are partially r ttally replaced by lungs that cme frm a dnr. Dnr lungs can be retrieved frm a living dnr r a deceased dnr. A living dnr can nly dnate ne lung lbe. With sme lung diseases a recipient may nly need t receive a single lung. With ther lung diseases, such as cystic fibrsis, it is imperative that a recipient receive tw lungs. While lung transplants carry certain assciated risks, they can als extend life expectancy and enhance the quality f life fr end-stage pulmnary patients. Mre than 6,400 lung transplants have been perfrmed since the first successful peratins in the early 1980s. In 2010, 1,770 lung transplant prcedures were perfrmed in the U.S., yet 2,469 new candidates were added t the waiting list the same year. Lung transplant prgrams nw exist in many cuntries. Internatinally, the number f dnr rgans available is far fewer than the number f patients with endstage lung disease. Because f this, many candidates die n the waiting list, and the average wait t receive a dnr rgan may apprach tw years. Overall survivals are between 60 percent and 65 percent at tw years and apprximately 40 percent at five years. Cnsidering the resurce limitatins and the imprtance f assuring ptimum utcmes, we believe that internatinal guidelines fr selectin f apprpriate candidates fr lung transplant will ensure a fair distributin f dnr rgans. Transplant physicians and surgens representing the Internatinal Sciety f Heart and Lung Transplantatin, the American Sciety f Transplant Physicians, the American Thracic Sciety, the Eurpean Respiratry Sciety, and the Thracic Sciety f Australia and New Zealand have agreed n the infrmatin in the fllwing dcument as acceptable guidelines fr candidates fr lung transplantatin. Lung transplantatin shuld be cnsidered fr patients with advanced lung disease whse clinical status has prgressively declined despite maximal medical r surgical therapy. Candidates are usually symptmatic during activities f daily living and have a limited expected survival ver the next tw years. In additin, the ideal candidate shuld be free f significant ther rgan dysfunctin and extrapulmnary manifestatins f a systemic disease. Guidelines fr recipient selectin have been develped by the American Thracic Sciety and the Internatinal Sciety f Heart and Lung Transplantatin, 4

5 Emphysema is a frm f COPD defined by abnrmal and permanent enlargement f the airspaces distal t the terminal brnchiles. It is assciated with the destructin f the alvelar walls. Emphysema causes dyspnea thrugh airflw limitatin, hyperinflatin and lss f gas exchanging surfaces in the lungs (als knwn as increased physilgic dead space). LVRS (als called reductin pneumplasty r bilateral pneumectmy) is a surgical technique that may be beneficial fr sme patients with advanced emphysema wh have pr cntrl f their disease despite maximal medical therapy. LVRS entails reducing the lung vlume by wedge excisin f emphysematus tissue. The mechanisms by which LVRS might prvide benefit are nt knwn with certainty. It has been suggested that LVRS reduces the size mismatching between the hyperinflated lungs and the chest cavity, thereby restring the utward circumferential pull n the brnchiles (i.e., increasing elastic recil) and imprving expiratry airflw. As an example, in a study f 20 patients underging vlume reductin surgery, 16 experienced an increase in elastic recil. The patients with imprved elastic recil had a significantly greater increase in exercise capacity than the fur withut increased elastic recil. Lung transplant types and the cnditin: Bilateral lung transplant (BLT): Cystic fibrsis. Brnchiectasis. Pulmnary hypertensin. Emphysema. Pulmnary fibrsis (idipathic r secndary t sclerderma r ther disease states). Single lung transplant (SLT): Emphysema. Pulmnary fibrsis (idipathic r secndary t sclerderma r ther disease states). Heart and lung transplant: Same as SLT and BLT with: Pr left ventricular functin r irreversible right ventricle functin. Surgically irreparable cngenital heart defects. Deceased dnr lung transplantatin: A deceased dnr, als knwn as cadaveric dnr, is the mst cmmn dnr surce used fr lung transplantatin. In 1995, the United Netwrk fr Organ Sharing (UNOS) changed the methd fr allcating dnated cadaver lungs fr individuals ver age 12 by assigning each candidate a lung allcatin scre based n survival benefit and urgency rather than waiting time (Mulligan, 2008). In cntrast, allcatin t children under age 12 cntinues t be based n waiting time. Preferential transplantatin f sicker patients has nt resulted in an increase in early mrtality fllwing transplantatin (Ktlff, 2010). Accrding t the Organ Prcurement and Transplantatin Netwrk ([OPTN], 2014) natinal data fr deceased dnr primary lung transplantatin perfrmed between 1997 and 2004, graft survival rates were 83.1 percent, 62.1 percent, and 46.2 percent, respectively, at ne, three and five years (based n OPTN data as f July 4, 2014). 5

6 Living dnr lung transplantatin (LDLT): Use f a live dnr as a surce fr lung transplantatin was initiated in 1993 due t the higher demand than supply fr patients waiting fr lung transplantatin. Althugh LDLT may be apprpriate fr a highly selected individual wh likely wuld nt survive waiting times fr a deceased dnr, it is nw rarely perfrmed. Accrding t the OPTN annual reprt (2012), nly ne LDLT was perfrmed in 2012, with fur LDLT perfrmed between 2007 and Survival data fr LDLT perfrmed in 2012 were nt published in the annual reprt. This prcedure requires the dnatin f ne lung lbe frm each f tw living dnrs. Majr cmplicatins have included pleural effusin, brnchial stump fistula, bi-lbectmy, hemrrhage phrenic nerve injury, pulmnary artery thrmbsis and brnchial stricture. Minr cmplicatins include persistent air leak, arrhythmia and pneumnia (Slmn, 2010). Deceased dnr transplantatin is preferred t avid the risk t tw healthy dnrs (Slmn, 2010). Lung transplantatin shuld nt be ffered t adults with a recent histry f malignancy. A tw-year disease-free interval cmbined with a lw predicted risk f recurrence after lung transplantatin may be reasnable, fr instance, in nn-melanma lcalized skin cancer that has been treated apprpriately. Hwever, a five-year disease-free interval is prudent in mst cases, particularly fr patients with a histry f hematlgic malignancy, sarcma, melanma, r cancers f the breast, bladder r kidney. Unfrtunately, fr a prtin f patients with a histry f cancer, the risk f recurrence may remain t high t prceed with lung transplantatin even after a five-year disease-free interval. Lung transplantatin in children Lung transplantatin in children is evlving. Diseases that are ptentially amenable t lung transplantatin include primary pulmnary hypertensin, pulmnary hypertensin assciated with structural heart disease, pulmnary vein stensis, pulmnary hypertensin assciated with parenchymal lung disease, and cngenital abnrmalities f lung develpment r f lung adaptatin t extrauterine life. As in adults, maximal medical therapy including vasdilatrs and supplemental xygen shuld be instituted befre children are cnsidered fr transplantatin. Since the diagnses are varied and the disease spectra diverse, prgnstic indicatrs have been difficult t develp; thus empirical criteria are the primary means f selecting candidates. Searches Keystne VIP Chice searched PubMed and the databases f: UK Natinal Health Services Centre fr Reviews and Disseminatin. Agency fr Healthcare Research and Quality s Natinal Guideline Clearinghuse and ther evidence-based practice centers. The Centers fr Medicare & Medicaid Services (CMS). 6

7 We cnducted searches n September 14, Search terms were: MeSH chrnic bstructive pulmnary disease, frailty, interstitial lung disease, lung transplantatin, besity, pulmnary arterial hypertensin, sarcpenia and cystic fibrsis. We included: Findings Systematic reviews, which pl results frm multiple studies t achieve larger sample sizes and greater precisin f effect estimatin than in smaller primary studies. Systematic reviews use predetermined transparent methds t minimize bias, effectively treating the review as a scientific endeavr, and are thus rated highest in evidence-grading hierarchies. Guidelines based n systematic reviews. Ecnmic analyses, such as cst-effectiveness, and benefit r utility studies (but nt simple cst studies), reprting bth csts and utcmes smetimes referred t as efficiency studies which als rank near the tp f evidence hierarchies. Accrding t the 2014 Registry reprt, the median survival fr all adult recipients is 5.7 years, but bilateral lung recipients appear t have a better median survival than single lung recipients (7 versus 4.5 years, respectively.) (Fr a figure shwing updated infrmatin, please see the Internatinal Sciety fr Heart and Lung Transplantatin slide set "Overall Lung and Adult Lung Transplantatin Statistics," slide titled Number f Lung Transplants Perfrmed by Year and Prcedure Type at ISHLT Registry. Hwever, it is unclear if this survival advantage is directly related t the type f peratin r t the underlying recipient characteristics. The bilgical needs and circumstances f candidates yunger than age 12 are different frm either adlescent r adult candidates. One key difference is the size and lung capacity f dnrs and patients amng these age ranges. Fr this reasn, lung allcatin plicy differs fr these grups f candidates and is designed t suit their unique needs. Children yunger than age 12 have pririty fr all dnrs f similar age and size within a 1,000-mile radius befre any lder candidates wuld be cnsidered. In sme circumstances, a transplant center may determine that a child's cnditin warrants a reduced size transplant frm an adult dnr. If the center wishes t cnsider this additinal treatment ptin, these children will have access t adult rgans nce they are ffered t adlescents and adults in the same allcatin zne. In 2012, there were 460 pediatric rgan dnrs in the United States, including 114 between the ages f 6 and 10 years. Althugh there were nly 11 lung dnrs in that age grup, that number likely reflects lw demand (tw lung transplants in recipients aged 6 10) as much as rgan availability. Althugh definitive patient selectin criteria fr lung transplantatin have nt been established, there is evidence t indicate that ptential recipients with chrnic lung disease culd be cnsidered if they are nt eligible fr further medical r surgical therapy and if they have a < 50 percent chance f surviving fr 24 t 36 mnths, when transplantatin is expected t cnfer a survival advantage, and when there are n cntraindicatins. Internatinal guidelines fr selectin f lung transplantatin candidates, as set ut by the ISHLT, cntain general and disease-specific selectin criteria, and abslute and relative cntraindicatins. These guidelines aim t facilitate the selectin prcess and t prmte a fair distributin f dnr rgans. Hwever, the final decisin t place a candidate n the waiting list resides with the expertise and practice f individual transplant centers and will vary frm cuntry t cuntry. Evidence frm registry and chrt studies n deceased dnr lung transplantatin indicated that a substantial number f the recipients will derive a survival advantage frm the prcedure, tgether with 7

8 imprvement in quality f life (QOL). Evidence was cntrversial as t which diagnstic grup f patients benefited the mst in survival r health-related quality f life (HRQOL) utcmes, hw lng after transplantatin the survival benefit was reached, and which type f transplantatin (single r bilateral) was assciated with lnger survival. Evidence indicated that lung transplantatin was assciated with lifethreatening cmplicatins, such as rejectin t the allgraft and infectin. In additin, lifelng cmmitment t immunsuppressive medicatins was assciated with inherent adverse effects. Thus, transplantatin will imprve survival and QOL, but at the same time, it will intrduce new restrictins and cmplicatins. Plicy updates: Nne. Summary f clinical evidence: Citatin Stephensn AL, et al. (May 2015). Clinical and demgraphic factrs assciated with pst lung transplantatin survival in individuals with cystic fibrsis. Kaltman JK (2007) Pediatric Cardimypathy Registry (PCMR). Frm 1990 t 200. Rbertsn et al (2012). Evaluated the safety f fundplicatin in LTX recipients and its effects n quality f life. Cntent, Methds, Recmmendatins Key pints: Cntemprary studies evaluating pst-transplant survival are limited and ften include data frm single centers r selected sub-grups. The purpse f this study was t evaluate verall transplant survival and t identify risk factrs assciated with death after transplant. After lung transplantatin, five-year survival in Canadians with Cystic Fibrsis CF is 67%, and 50% f patients live > 10 years. Despite these impressive prbabilities, age at transplant, pancreatic sufficiency and B cepacia infectin remain imprtant determinants f survival after lung transplantatin. Key pints: This ppulatin-based study used data frm the Pediatric Cardimypathy Registry (PCMR). Frm 1990 t 2007, the PCMR, led by the University f Miami Lenard M. Miller Schl f Medicine, enrlled 1,731 children (18 years f age r yunger) diagnsed with pediatric dilated cardimypathy, the mst cmmn heart muscle disease. Dilated cardimypathy can lead t heart valve prblems, arrhythmias (irregular heartbeats), bld clts in the heart and even heart failure. Key pints: Between June 1, 2008 and December 31, 2010, a prspective study f LTX recipients underging fundplicatin was undertaken. Quality f life was assessed befre and after surgery. Bdy mass index (BMI) and pulmnary functin were fllwed-up. A ttal f 16 patients, mean +/- SD age f 38 +/-11.9 yrs, underwent laparscpic Nissen fundplicatin. There was n peri-perative mrtality r majr cmplicatins. Mean +/- SD hspital stay was 2.6 +/- 0.9 days; 15 ut f 16 patients were satisfied with the results f surgery pst-fundplicatin. There was a significant imprvement in reflux symptm index and DeMeester questinnaires and gastr-intestinal quality f life index scres at 6 mnths Snell GI, et al. (2000). Outcmes frm paired single-lung transplants Key pints: Simultaneus, paired SLTs frm a single rgan dnr are ne way t maximize lung transplant pprtunities. 8

9 Citatin frm the same dnr. TJ.J Heart Lung Transplant Nv. Black MC, et al. (2014). Duble lung transplants have significantly imprved survival cmpared with single lung transplants in high lung allcatin scre patients. Ann Thrac Surg Kirshbm PM, et al. (2002). Use f extracrpreal membrane xygenatin in pediatric thracic rgan transplantatin. J Thrac Cardivasc Surg Jan. Cntent, Methds, Recmmendatins Paired transplants allw cmparisn between left and right SLTs and als prvide insight int the relevance f dnr vs recipient factrs in rejectin utcmes. The general utcmes f right and left transplants are similar, althugh we bserved increased six-mnth t tw-year mrtality assciated with left lung transplantatin. The lack f crrelatin between the incidence f acute rejectin episdes r the severity f BOS in paired allgraft recipients suggests that "dnr factrs" are nt the dminant cause. Key pints: The UNOS Thracic Transplant Database fr lung transplants frm January 2005 t June 2012 was used fr analysis. Prpensity matching was used t minimize differences between the high and lw LAS grups and between SLTs and BLTs in the high LAS grup. Despite a higher perative mrbidity, patients wh had a high LAS did substantially better in survival if tw lungs were transplanted rather than nly ne, with a larger difference in survival than fr patients with a lwer LAS. Key pints: Mechanical cardirespiratry supprt is ccasinally required befre r after pediatric thracic rgan transplantatin. Extracrpreal membrane xygenatin is the mst cmmnly used mechanical supprt technique in children. The gal f this study was t examine the indicatins fr initiatin and utcmes after peritransplant use f extracrpreal membrane xygenatin. Glssary Burkhlderia cepacia cmplex (B. cepacia) Cnsists f different species f bacteria that are fund in the natural envirnment. Sme f these species pse serius risks t the health f a persn with cystic fibrsis. Chrnic bstructive pulmnary disease (COPD) A prgressive disease that makes it hard t breathe. There are tw main frms f COPD: chrnic brnchitis, which invlves a lng-term cugh with mucus, and emphysema, which invlves damage t the lungs ver time. Mst peple with COPD have a cmbinatin f bth cnditins. Extracrpreal life supprt (ECLS) ECLS systems are mechanical devices t temprarily supprt the failing heart and lung. It is a further develpment f a cnventinal heart-lung machine (HLM). Cmpared t the HLM, it is smaller, and has been reduced t nly main cmpnents, such as the centrifugal pump and a membrane xygenatr. It is highly mbile, and can be used bth in and utside f the hspital. Extracrpreal membrane xygenatin (ECMO) A treatment that uses a pump t circulate bld thrugh an artificial lung back int the bldstream f a very ill baby. This system prvides heart-lung bypass supprt utside f the baby's bdy. It may help supprt a child wh is awaiting a heart r lung transplant. Eisenmenger's defect r cmplex Is a cnditin that affects bld flw frm the heart t the lungs in sme peple wh were brn with structural prblems f the heart. 9

10 References Prfessinal sciety guidelines/ther: Hsenpud J. D., Bennett L. E., Keck B. M., et al.the registry f the internatinal sciety fr heart and lung transplantatin: furteenth fficial reprt J. Heart Lung Transplant heart muscle disease Natinal Institutes f Health, the Natinal Heart, Lung, and Bld Institute (NHLBI), July 25, Available at: URL: Accessed September 15, Jhn Hpkins Medicine website: Natinal Heart, Lung, and Bld Institute (NHLBI) Internatinal Guidelines fr the Selectin f Lung Transplant Candidates", American Jurnal f Respiratry and Critical Care Medicine, Vl. 158, N. 1 (1998), pp URL: Accessed September 15, U.S. Dept. f Health and Human Services, Rckville, MD, 1996 annual reprt f the U.S. scientific registry fr transplant recipients and the rgan prcurement and transplantatin netwrk transplant data UNOS, Richmnd, VA, and the Divisin f Transplantatin, Bureau f Health Resurces Develpment, Health Resurces and Services Administratin. Organ Prcurement and Transplantatin Netwrk. Accessed May 14, 2015, Peer-reviewed references: Anne L. Stephensn, Jenna Sykes, Yves Berthiaume et al.,clinical and demgraphic factrs assciated with pst lung transplantatin survival in individuals with cystic fibrsis.lianne G. Singer, Shawn D. Aarn, Gerge A. Whitmre, Sanja Stanjevic DOI: p , September 2015Vlume 34, Issue 9, Pages Published nline: May Armitage J. M., Kurland G., Michaels M., et al.critical issues in pediatric lung transplantatin. J. Thrac. Cardivasc. Surg Black MC, Trivedi J, Schumer EM et al. Duble lung transplants have significantly imprved survival cmpared with single lung transplants in high lung allcatin scre patients. Ann Thrac Surg Nv;98(5): di: /j.athracsur Epub 2014 Aug 7.PMID: Brantigan OC, Muller E, Kress MB. A surgical apprach t pulmnary emphysema. Am Rev Respir Dis 1959; 80:194. David Weill, Christian Benden, Paul A. Crris, Jhn H. Dark, R. Duane Davis, Shaf Keshavjee, David J. Lederer, Michael J. Mulligan, and thersdoi: The Jurnal f Heart and Lung Transplantatin, Vl. 34, Issue 1, p1 15 Published nline: June

11 Fessler HE, Permutt S. Lung vlume reductin surgery and airflw limitatin. Am J Respir Crit Care Med 1998; 157:715. Fessler HE, Scharf SM, Ingenit EP, et al. Physilgic basis fr imprved pulmnary functin after lung vlume reductin. Prc Am Thrac Sc 2008; 5:416. Ingenit EP, Lring SH, My ML, et al. Interpreting imprvement in expiratry flws after lung vlume reductin surgery in terms f flw limitatin thery. Am J Respir Crit Care Med 2001; 163:1074. Ingenit EP, Lring SH, My ML, et al. Cmparisn f physilgical and radilgical screening fr lung vlume reductin surgery. Am J Respir Crit Care Med 2001; 163:1068. Keller CA, Ruppel G, Hibbett A, et al. Thracscpic lung vlume reductin surgery reduces dyspnea and imprves exercise capacity in patients with emphysema. Am J Respir Crit Care Med 1997; 156:60. Kirshbm PM, Bridges ND, Myung RJ, et al., Use f extracrpreal membrane xygenatin in pediatric thracic rgan transplantatin. J Thrac Cardivasc Surg Jan;123(1):130-6.PMID: Krutsinger D, Reed RM,, De Oliveira NC, et al. Lung transplant fr interstitial lung disease: utcmes fr single versus bilateral lung transplantatin.j Heart Lung Transplant May;34(5): di: /j.healun Epub 2014 Nv 10.PMID: Martinez FJ, de Oca MM, Whyte RI, et al. Lung-vlume reductin imprves dyspnea, dynamic hyperinflatin, and respiratry muscle functin. Am J Respir Crit Care Med 1997; 155:1984. Ktlff RM. : Masn RJ, Braddus VC, et al. Lung transplantatin. InMurray & Nadel s textbk f respiratry medicine. 5th ed. New Yrk: W.B. Saunders Cmpany; Mulligan MS, Sheardn TH, Weill D, Pagani FD, Mre J, Murray S. Heart and lung transplantatin in the United States, Am J Transplant Apr;8(4 Pt 2): Orens JB, et al. Internatinal guidelines fr the selectin f lung transplant candidates: 2006 update--a cnsensus reprt frm the Pulmnary Scientific Cuncil f the Internatinal Sciety fr Heart and Lung Transplantatin. Jurnal f Heart and Lung Transplantatin 2006;25(7): DOI: /j.healun Rbertsn AG, Krishnan A, Ward C, et al. Anti-reflux surgery in lung transplant recipients: Outcmes and effects n quality f life. Eur Respir J. 2012;39(3): Sciurba FC. Early and lng-term functinal utcmes fllwing lung vlume reductin surgery. Clin Chest Med 1997; 18:259. Sciurba FC, Rgers RM, Keenan RJ, et al. Imprvement in pulmnary functin and elastic recil after lungreductin surgery fr diffuse emphysema. N Engl J Med 1996; 334:1095. Snell GI, Shiraishi T, Griffiths A, et al, Outcmes frm paired single-lung transplants frm the same dnr. TJ.J Heart Lung Transplant Nv;19(11): PMID:

12 The registry f the Internatinal Sciety fr Heart and Lung Transplantatin: thirty-first adult lung and heart-lung transplant reprt--2014; fcus theme: re-transplantatin. Yusen RD, Edwards LB, Kucheryavaya AY, et.al., Internatinal Sciety fr Heart and Lung Transplantatin, the registry f the Internatinal Sciety fr heart and lung transplantatin: thirty-first adult lung and heartlung transplant reprt--2014; fcus theme: retransplantatin. J Heart Lung Transplant Oct;33(10): di: /j.healun Epub 2014 Aug 14. Clinical trials: Searched clinicaltrials.gv n September 15, 2015, using terms lung transplants Open Studies. 272 studies fund, relevant tw cited belw: NCT Transplantatin f lungs btained frm Nn-Heart-Beating Dnrs (NHBDs) after ex- viv perfusin w/ STEEN Slutin Device: STEEN Slutin, University f Nrth Carlina, Chapel Hill. Last verified: July Available at: ClinicalTrials.gv.. Accessed September 15, NCT , Genme Transplant Dynamics, Natinal Heart, Lung, and Bld Institute (NHLBI), Last updated: June 20, Available at: ClinicalTrials.gv. Accessed September 15, CMS Natinal Cverage Determinatins (NCDs): Cardiac Rehabilitatin Prgrams fr Chrnic Heart Failure; Lung Vlume Reductin Surgery (Reductin Pneumplasty) (240.1): Accessed Octber 2, Lcal Cverage Determinatins (LCDs): Article-Immunsuppressive Drugs Plicy Article Effective Octber 2015 (A52474). trid=137&cntrver=1. Accessed Octber 2, Cmmnly submitted cdes Belw are the mst cmmnly submitted cdes fr the service(s)/item(s) subject t this plicy. This is nt an exhaustive list f cdes. Prviders are expected t cnsult the apprpriate cding manuals and bill accrdingly. CPT Cde Descriptin Cmment Lung transplant, single; withut cardipulmnary bypass Lung transplant, single; with cardipulmnary bypass Lung transplant, duble (bilateral sequential r en blc); withut cardipulmnary bypass 12

13 Lung transplant, duble (bilateral sequential r en blc); with cardipulmnary bypass Backbench standard preparatin f cadaver dnr lung allgraft prir t transplantatin, including dissectin f allgraft frm surrunding sft tissues t prepare pulmnary venus/atrial cuff, pulmnary artery, and brnchus; unilateral Backbench standard preparatin f cadaver dnr lung allgraft prir t transplantatin, including dissectin f allgraft frm surrunding sft tissues t prepare pulmnary venus/atrial cuff, pulmnary artery, and brnchus; bilateral ICD-9 Cde Descriptin Cmment 135 Sarcidsis Alpha-1-antitrypsin deficiency Cystic fibrsis Other specified disrders f metablism [esinphilic granulma] Graft-versus-hst disease Primary pulmnary hypertensin Unspecified chrnic brnchitis (brnchilitis bliterans) Other emphysema Brnchiectasis Extrinsic allergic alvelitis 496 Chrnic airway bstructin, nt elsewhere classified 501 Asbestsis 515 Pst-inflammatry pulmnary fibrsis Idipathic fibrsing alvelitis Lymphangileimymatsis [with end-stage pulmnary disease] Lung invlvement in systemic sclersis Lung invlvement in ther diseases classified elsewhere Ventricular septal defect [Eisenmenger's defect r cmplrx] Cngenital cystic lung Agenesis, hypplasia, and dysplasia f lung Cngenital brnchiectasis Cmplicatins f transplanted rgan, lung ICD 10 Cde Descriptin Cmments C96.6 Unifcal Langerhans-cell histicytsis [esinphilic granulma] D86.0 Sarcidsis f lung D Graft-versus-hst disease D E E84.9 Cystic fibrsis E88.01 Alpha-1-antitrypsin deficiency I27.0 Primary pulmnary hypertensin J42 Unspecified chrnic brnchitis [brnchilitis bliterans] J J43.9 Emphysema J J44.9 Chrnic bstructive pulmnary disease J J47.9 Brnchiectasis J61 Pneumcnisis due t asbests and ther mineral fibers J J67.9 Allergic alvelitis J84.10 Pulmnary fibrsis, unspecified 13

14 J84.81 Lymphangileimymatsis [with end-stage pulmnary disease] J84.89 Other specified interstitial pulmnary diseases J99 Respiratry disrders in diseases classified elsewhere M34.81 Systemic sclersis with lung invlvement Q21.8 Other cngenital malfrmatins f cardiac septa [Eisenmenger's defect r cmplex] Q33.0 Cngenital cystic lung Q33.3 Agenesis f lung Q33.4 Cngenital brnchiectasis Q33.6 Cngenital hypplasia and dysplasia f lung T Cmplicatins f lung transplant T HCPCS Level II Cde S2060 Lbar lung transplantatin Descriptin Cmment S2061 S2152 Dnr lbectmy (lung) fr transplantatin, living dnr Slid rgan(s), cmplete r segmental, single rgan r cmbinatin f rgans; deceased r living dnr (s), prcurement, transplantatin, and related cmplicatins; including: drugs; supplies; hspitalizatin with utpatient fllw-up; medical/surgical, diagnstic, emergency, and rehabilitative services, and the number f days f pre and psttransplant care in the glbal definitin 14