|MP 7.03.08||Heart/Lung Transplant|
|Original Policy Date
|Last Review Status/Date
Reviewed with literature search/11:2014
|Return to Medical Policy Index|
Our medical policies are designed for informational purposes only and are not an authorization, or an explanation of benefits, or a contract. Receipt of benefits is subject to satisfaction of all terms and conditions of the coverage. Medical technology is constantly changing, and we reserve the right to review and update our policies periodically.
Combined heart/lung transplantation is intended to prolong survival and improve function in patients with end-stage cardiac and pulmonary diseases. The majority of recipients have Eisenmenger syndrome (37%), followed by idiopathic pulmonary artery hypertension (28%) and cystic fibrosis (14%). Eisenmenger syndrome is a form of congenital heart disease in which systemic-to-pulmonary shunting leads to pulmonary vascular resistance. Eventually, pulmonary hypertension may lead to a reversal of the intracardiac shunting and inadequate peripheral oxygenation, or cyanosis.(2)
However, the total number of patients with Eisenmenger syndrome has been declining in recent years, as a result of corrective surgical techniques and improved medical management of pulmonary hypertension. Heart/lung transplants have not increased appreciably for other indications either, as it has become more common to transplant a single or double lung and maximize medical therapy for heart failure, rather than perform a combined transplant. In these, patient survival rates are similar to lung transplant rates. Bronchiolitis obliterans syndrome is a major complication; 1-, 5-, and 10-year patient survival rates are 68%, 50%, and 40%, respectively.(2)
In 2013, 23 individuals received heart/lung transplants in the United States. As of the end of September 2014, there were 51 patients on the waiting list for heart/lung transplants and 12 individuals had received transplants during the year to date.(3)
Heart/lung transplantation may be considered medically necessary for carefully selected patients with end-stage cardiac and pulmonary disease including, but not limited to, one of the following diagnoses:
- irreversible primary pulmonary hypertension with heart failure;
- nonspecific severe pulmonary fibrosis, with severe heart failure;
- Eisenmenger complex with irreversible pulmonary hypertension and heart failure;
- cystic fibrosis with severe heart failure;
- chronic obstructive pulmonary disease with heart failure;
- emphysema with severe heart failure;
- pulmonary fibrosis with uncontrollable pulmonary hypertension or heart failure.
Heart/lung retransplantation after a failed primary heart/lung transplant may be considered medically necessary in patients who meet criteria for heart/lung transplantation.
Heart/lung transplantation is considered investigational in all other situations.
Potential contraindications subject to the judgment of the transplant center:
- Known current malignancy, including metastatic cancer
- Recent malignancy with high risk of recurrence
- Untreated systemic infection making immunosuppression unsafe, including chronic infection
- Other irreversible end-stage disease not attributed to heart or lung disease
- History of cancer with a moderate risk of recurrence
- Systemic disease that could be exacerbated by immunosuppression
- Psychosocial conditions or chemical dependency affecting ability to adhere to therapy
When the candidate is eligible to receive a heart in accordance with United Network for Organ Sharing (UNOS) guidelines for cardiac transplantation, the lung(s) shall be allocated to the heart-lung candidate from the same donor. When the candidate is eligible to receive a lung in accordance with the UNOS Lung Allocation System (LAS), the heart shall be allocated to the heart-lung candidate from the same donor if no suitable Status 1A isolated heart candidates are eligible to receive the heart. Status 1A is described below.(1)
The United Network for Organ Sharing (UNOS) prioritizes donor thoracic organs according to the severity of illness as follows:
A patient is admitted to the listing transplant center hospital and has at least 1 of the following devices or therapies in place:
- Mechanical circulatory support for acute hemodynamic decompensation that includes at least 1 of the following:
- Left and/or right ventricular assist device implanted
- Total artificial heart
- Intra-aortic balloon pump, or
- Extracorporeal membrane oxygenator (ECMO)
- Mechanical circulatory support
- Mechanical ventilation
- Continuous infusion of inotropes and continuous monitoring of left ventricular filling pressures
- If criteria a, b, c, and d are not met, such status can be obtained by application to the applicable Regional Review Board
A patient has at least 1 of the following devices or therapies in place:
- left and/or right ventricular device implanted, or
- continuous infusion of intravenous inotropes
A patient who does not meet Status 1A or 1B is listed as Status 2.
Status 7 patients are considered temporarily unsuitable to receive a thoracic organ transplant.
BlueCard/National Account Issues
Heart/lung transplants should be considered for coverage under the Transplant benefit.
What is covered under the scope of the Human Organ Transplant (HOT) benefit needs to be considered. Typically, the following are covered under the HOT benefit:
- hospitalization of the recipient for medically recognized transplants from a donor to a transplant recipient;
- evaluation tests requiring hospitalization to determine the suitability of both potential and actual donors, when such tests cannot be safely and effectively performed on an outpatient basis;
- hospital room, board, and general nursing in semi-private rooms;
- special care units, such as coronary and intensive care;
- hospital ancillary services;
- physicians’ services for surgery, technical assistance, administration of anesthetics, and medical care;
- acquisition, preparation, transportation, and storage of organ;
- diagnostic services;
- drugs that require a prescription by federal law.
Expenses incurred in the evaluation and procurement of organs and tissues are benefits when billed by the hospital. Included in these expenses may be specific charges for participation with registries for organ procurement, operating rooms, supplies, use of hospital equipment, and transportation of the tissue or organ to be evaluated.
Administration of products with a specific transplant benefit needs to be defined as to:
- when the benefit begins (at the time of admission for the transplant or once the patient is determined eligible for a transplant, which may include tests or office visits prior to transplant);
- when the benefit ends (at the time of discharge from the hospital or at the end of required follow-up, including the immunosuppressive drugs administered on an outpatient basis).
Coverage usually is not provided for:
- HOT services for which the cost is covered/funded by governmental, foundational, or charitable grants;
- organs sold rather than donated to the recipient;
- an artificial organ.
This policy was originally created in 1996 and updated regularly with searches of the MEDLINE database. The most recent literature search was reviewed through September 16, 2014. Due to the nature of the population, there were no randomized controlled trials (RCTs) comparing heart/lung transplant to alternatives. Systematic reviews are based on case series and registry data. The extant RCTs compare surgical technique, infection prophylaxis, and immunosuppressive therapy and are not germane to this policy. The following is a summary of evidence based on registry data, case series, and expert opinion.
Patients who are eligible for heart/lung transplantation can be listed under both the heart and lung allocation systems in the United States. In 2005, United Network for Organ Sharing (UNOS) changed the method by which lungs were allocated, from one based on length of time on the waiting list, to a system that incorporates the severity of the patient’s underlying disease, as well as likelihood of survival. (1) However, it has been noted that the individual systems underestimate the severity of illness in patients with both end-stage heart and lung failure, and modification of the lung allocation score can be appealed for patients who meet the following criteria:
- Deterioration on optimal therapy
- Right arterial pressure greater than 15 mm Hg
- Cardiac index less than 1.8 L/min/m².
A 2014 analysis of data from the Organ Procurement and Transplantation Network reported on indications for pediatric heart/lung transplantation.(4) The number of pediatric heart/lung transplants has decreased in recent years, ie, 56 cases in 1993-1997; 21 cases in 2008-2013. The 3 most common indications for pediatric heart/lung transplant were primary pulmonary hypertension (n=55), congenital heart disease (n=37), and Eisenmenger syndrome (n=30). However, while 30 children received a heart/lung transplant for Eisenmenger syndrome through 2002, none have been performed for this indication since then. Pediatric heart/lung transplants have also been performed for other indications including alpha1 antitrypsin deficiency, pulmonary vascular disease, cystic fibrosis, and dilated cardiomyopathy
In 2012, the Registry of the International Society for Heart and Lung Transplantation (ISHLT) reported on pediatric heart/lung transplant data collected through June 2011.(5) In recent years, the number of heart/lung transplant procedures in children has decreased, and the number of lung transplants has increased. There have not been any heart/lung transplants in infants since 2007. Overall, survival rates after heart/lung transplants are comparable in children and adults (median half-life of 4.7 and 5.3 years, respectively). For pediatric heart/lung transplants that occurred between January 1990 and June 2010, the 5-year survival rate was 49%. The 2 leading causes of death in the first year after transplantation were noncytomegalovirus infection and graft failure. Beyond 3 years posttransplant, the major cause of death was bronchiolitis obliterans syndrome.
Repeat heart-lung transplant procedures have been performed; only 1 published study was found that reported on outcomes after repeat heart-lung transplants. The study, published by Shuhaiber and colleagues in 2008, involved a review of data from the UNOS registry.(6) The authors identified 799 primary heart-lung and 19 repeat heart-lung transplants. According to Kaplan-Meier survival analysis, the observed median survival times were 2.08 years after primary transplant and 0.34 years after repeat transplants. In addition, the authors analyzed survival data in matched pairs of primary and repeat transplant patients, who were matched on a number of potentially confounding demographic and clinical characteristics. Matches were not available for 4 repeat transplant patients. For the 15 repeat transplant patients with primary transplant matches, survival time did not differ significantly in the 2 groups. Being on a ventilator was statistically significantly associated with decreased survival time. The main limitation of this analysis is the small number of repeat transplant procedures performed.
Individual transplant centers may differ in their guidelines, and individual patient characteristics may vary within a specific condition. In general, heart transplantation is contraindicated in patients who are not expected to survive the procedure, or in whom patient-oriented outcomes, such as morbidity or mortality, are not expected to change due to comorbid conditions unaffected by transplantation, eg, imminently terminal cancer or other disease. Further, consideration is given to conditions in which the necessary immunosuppression would lead to hastened demise, such as active untreated infection. However, stable chronic infections have not always been shown to reduce life expectancy in heart transplant patients.
Concerns regarding a potential recipient’s history of cancer were based on the observation of significantly increased incidence of cancer in kidney transplant patients.(7) In fact, carcinogenesis is 2 to 4 times more common, primarily skin cancers, in both heart transplant and lung transplant patients, likely due to the higher doses of immunosuppression necessary for the prevention of allograft rejection.(2,8) The incidence of de novo cancer in heart transplant patients approaches 26% at 8 years posttransplant, the rate for lung transplant is 28% at 10 years. For renal transplant patients who had a malignancy treated prior to transplant, the incidence of recurrence ranged from zero to more than 25%, depending on the tumor type.(8,9) However, it should be noted that the availability of alternate treatment strategies informs recommendations for a waiting period following high-risk malignancies: in renal transplant, a delay in transplantation is possible due to dialysis; end-stage cardiopulmonary failure patients may not have an option. A small study (n=33) of survivors of lymphoproliferative cancers who subsequently received cardiac transplant had 1-, 5-, and 10-year survival rates of 77%, 64%, and 50%, respectively.(11) By comparison, overall 1-, 5-, and 10-year survival rates are expected to be 88%, 74%, and 55%, respectively for the general transplant candidate. The evaluation of a candidate who has a history of cancer must consider the prognosis and risk of recurrence from available information including tumor type and stage, response to therapy, and time since therapy was completed. Although evidence is limited, patients in whom cancer is thought to be cured should not be excluded from consideration for transplant. United Network for Organ Sharing (UNOS) has not addressed malignancy in current policies.
Solid organ transplant for patients who are HIV-positive (HIV+) has been controversial, due to the long-term prognosis for human immunodeficiency virus (HIV) positivity and the impact of immunosuppression on HIV disease. Although HIV+ transplant recipients may be a research interest of some transplant centers, the minimal data regarding long-term outcome in these patients consist primarily of case reports and abstract presentations of liver and kidney recipients. Nevertheless, some transplant surgeons would argue that HIV positivity is no longer an absolute contraindication to transplant due to the advent of highly active antiretroviral therapy (HAART), which has markedly changed the natural history of the disease.
As of February 2013, the United Network for Organ Sharing (UNOS) policy on HIV-positive transplant candidates states: “A potential candidate for organ transplantation whose test for HIV is positive should not be excluded from candidacy for organ transplantation unless there is a documented contraindication to transplantation based on local policy.” (Policy 4, Identification of Transmissible Diseases in Organ Recipients).(12)
In 2006, the British HIV Association and the British Transplantation Society Standards Committee published guidelines for kidney transplantation in patients with HIV disease.(13) These criteria may be extrapolated to other organs:
- CD4 count greater than 200 cells/ml for at least 6 months
- Undetectable HIV viremia (less than 50 HIV-1 RNA copies/ml) for at least 6 months
- Demonstrable adherence and a stable HAART regimen for at least 6 months
- Absence of AIDS-defining [acquired immunodeficiency syndrome] illness following successful immune reconstitution after HAART.
However, concerns have been raised about the extrapolation of these criteria to lung transplants.
Other Potential Contraindications
Considerations for heart transplantation and lung transplantation alone may also pertain to combined heart-lung transplantation. For example, cystic fibrosis accounts for the majority of pediatric candidates for heart-lung transplantation, and infection with Burkholderia species is associated with higher mortality in these patients. And, experience with kidney transplantation in patients infected with HIV in the era of HAART has opened discussion of transplantation of other solid organs in these patients. These topics are addressed more fully in the separate policies on heart transplantation and lung transplantation.
Summary of Evidence
The available literature, consisting of case series and registry data, describes outcomes after heart-lung transplantation. Given the exceedingly poor expected survival without transplantation, this evidence is sufficient to demonstrate that heart/lung transplantation provides a survival benefit in appropriately selected patients. It may be the only option for some patients with end-stage cardiopulmonary disease. Heart/lung transplant is contraindicated in patients in whom the procedure is expected to be futile due to comorbid disease or in whom posttransplantation care is expected to significantly worsen comorbid conditions. Based on this evidence and established guidelines, heart–lung transplant may be considered medically necessary for those who meet clinical criteria and do not have contraindications to the procedure. A very limited amount of data suggest that, after controlling for confounding variables, survival rates after primary and repeat heart/lung transplants is similar. Findings are not conclusive due to the small number of cases of repeat heart-lung transplants reported in the published literature. Repeat heart-lung transplantation may be considered medically necessary in patients with a failed prior transplant who meet the clinical criteria for heart-lung transplantation.
Practice Guidelines and Position Statements
A key publication is the 2006 guidelines from the Pulmonary Scientific Council of the International Society for Heart and Lung Transplantation.(14) The consensus-based guidelines state that,
Lung transplantation is now a generally accepted therapy for the management of a wide range of severe lung disorders…. However, the number of donor organs available remains far fewer than the number of patients with end-stage lung disease who might potentially benefit from the procedure. It is of primary importance, therefore, to optimize the use of this resource, such that the selection of patients who receive a transplant represents those with realistic prospects of favorable long-term outcomes. There is a clear ethical responsibility to respect these altruistic gifts from all donor families and to balance the medical resource requirement of one potential recipient against those of others in their society. These concepts apply equally to listing a candidate with the intention to transplant and potentially de-listing (perhaps only temporarily) a candidate whose health condition changes such that a successful outcome is no longer predicted.
Thus, for all patients, including those with end-stage cardiopulmonary disease and HIV infection, evaluation of a candidate for transplant needs to consider the probability of a successful transplant and the limited supply of organs available.
US Preventive Services Task Force Recommendationa
Medicare National Coverage
Heart/lung transplantation is covered under Medicare when performed in a facility that is approved by Medicare as meeting institutional coverage criteria.(15) The Centers for Medicare and Medicaid Services (CMS) has stated that under certain limited cases, exceptions to the criteria may be warranted if there is justification and if the facility ensures safety and efficacy objectives.
- United Network for Organ Sharing (UNOS). Organ distribution: allocation of thoracic organs. UNOS Policies and Bylaws. http://optn.transplant.hrsa.gov/PoliciesandBylaws2/policies/pdfs/policy_9.pdf. Accessed August 22, 2014.
- Christie JD, Edwards LB, Kucheryavaya AY, et al. The Registry of the International Society for Heart and Lung Transplantation: twenty-seventh official adult lung and heart-lung transplant report--2010. J Heart Lung Transplant. 2010;29(10):1104-1118. PMID
- Organ Procurement and Transplantation Network (OPTN).http://optn.transplant.hrsa.gov/converge/latestData/step2.asp Accessed September 26, 2014.
- Spahr JE, West SC. Heart-lung transplantation: pediatric indications and outcomes. J Thorac Dis. Aug 2014;6(8):1129-1137. PMID 25132980
- Benden C, Edwards LB, Kucheryavaya AY, et al. The registry of the International Society for Heart and Lung Transplantation: fifteenth pediatric lung and heart-lung transplantation report-2012. J Heart Lung Transplant. Oct 2012;31(10):1087-1095. PMID 22975098
- Shuhaiber JH, Kim JB, Gibbons RD. Repeat heart-lung transplantation outcome in the United States. J Heart Lung Transplant. Oct 2008;27(10):1122-1127. PMID 18926404
- Kasiske BL, Snyder JJ, Gilbertson DT, et al. Cancer after kidney transplantation in the United States. Am J Transplant. 2004;4(6):905-913. PMID
- Taylor DO, Edwards LB, Boucek MM, et al. Registry of the International Society for Heart and Lung Transplantation: twenty-second official adult heart transplant report--2005. J Heart Lung Transplant. 2005;24(8):945-955. PMID
- Otley CC, Hirose R, Salasche SJ. Skin cancer as a contraindication to organ transplantation. Am J Transplant. 2005;5(9):2079-2084. PMID
- Trofe J, Buell JF, Woodle ES, et al. Recurrence risk after organ transplantation in patients with a history of Hodgkin disease or non-Hodgkin lymphoma. Transplantation. 2004;78(7):972-977. PMID
- Taylor DO, Farhoud HH, Kfoury G, et al. Cardiac transplantation in survivors of lymphoma: a multi-institutional survey. Transplantation. 2000;69(10):2112-2115. PMID
- Organ Procurement and Transplantation Network (OPTN).http://optn.transplant.hrsa.gov/governance/policies/. Accessed August 22, 2014.
- Bhagani S, Sweny P, Brook G. Guidelines for kidney transplantation in patients with HIV disease. HIV Med. 2006;7(3):133-139. PMID
- 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 Transplant. 2006;25(7):745-755. PMID
- Center for Medicare and Medicaid Services (CMS). Decision Memo for TRANSPLANT Centers: Re-Evaluation of Criteria for Medicare Approval (CAG-00061N) http://www.cms.gov/medicare-coverage-database/details/nca-decision-memo.aspx?NCAId=75&fromdb=true&. Accessed August 22, 2014.
|CPT||33930||Donor cardiectomy-pneumonectomy (including cold preservation)|
|33933||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|
|33935||Heart-lung transplant with recipient cardiectomy-pneumonectomy|
|33960–33961||Prolonged extracorporeal circulation code range|
|ICD-9 Procedure||33.6||Combined heart-lung transplantation|
|ICD-9 Diagnosis||416.0||Primary pulmonary hypertension|
|416.8||Other chronic pulmonary heart diseases (includes pulmonary hypertension, secondary)|
|428.1-428.9||Heart failure, code range|
|491.20-491.22||Chronic obstructive bronchitis code range (includes COPD)|
|492-0-492.8||Emphysema code range|
|745.4||Eisenmenger complex or syndrome|
|ICD-10-CM (effective 10/1/15)||E84.0, E84.8-E84.9||Cystic fibrosis code range|
|I27.0||Primary pulmonary hypertension|
|I27.1-I27.9||Other pulmonary heart diseases (includes Eisenmenger's complex)|
|I50.1-I50.9||Heart failure code range|
|J43.0-J43.9||Emphysema code range|
|J44.0-J44.9||Other chronic obstructive pulmonary disease code range|
|J84.1||Other interstitial pulmonary diseases with fibrosis|
|ICD-10-PCS (effective 10/1/15)||02YA0Z0||Surgical, heart and great vessels, transplantation, heart, open, allogeneic|
|0BYK0Z0, 0BYL0Z0, 0BYM0Z0||Surgical, respiratory system, transplantation, open, allogeneic, code by bilateral, left or right lung(s)|
|Type of Service||Surgery|
|Place of Service||Inpatient|
|07/31/96||Add to Surgery section, Transplants subsection||New policy|
|12/18/02||Replace policy||Policy updated; no change in policy statement|
|02/25/04||Replace policy||Policy updated, references added; policy statement revised to indicate that transplantation is investigational in HIV+ recipients. Additional criteria added to Policy Guidelines section to be consistent with other transplant policies|
|03/15/05||Replace policy||Policy updated with literature review; no change in policy statement; Reference number 6 added|
|04/1/05||Replace policy||Policy revised; HIV positivity deleted as an investigational indication for transplantation. Reference number 6 revised|
|9/27/05||Replace policy||Policy corrected; HIV positivity statement removed from policy section and CPT coding updated|
|04/25/06||Replace policy||Policy updated with literature review; no change in policy statement|
|06/12/08||Replace policy||Policy updated with literature search, reference numbers 7 and 8 added. Policy statement unchanged. Policy guidelines changed with updated UNOS policy and with comments about “absence of non-curable extrapulmonary infection'|
|07/09/09||Replace policy||Policy updated with literature search, no new references added. Policy statements unchanged.|
|11/10/11||Replace policy||Policy updated with literature search, no new references added. Severe heart failure added to second bullet point of medically necessary statement. Contraindications moved to Policy Guidelines. Absolute and relative contraindications combined and wording changed to be consistent with other solid organ transplant policies.|
|1/12/12||Replace policy- correction only||Partial paragraph deleted from Rationale section entitled “Pediatric Considerations.”|
|11/08/12||Replace Policy||Policy updated with literature search. No change to policy statement. Reference 4 added; other references renumbered or removed.|
|11/14/13||Replace policy||Policy updated with literature search through September 26, 2013. Two policy statements added; 1 on retransplantation and 1 stated that all other indications are considered investigational. References 5 and 11 added; other references renumbered or removed|
|11/13/14||Replace policy||Policy updated with literature review through September 16, 2014. Policy statements unchanged. Reference 4 added.|