Latest Review Date: August 2024

Category:  Surgery                                                                 

POLICY:

Heart/lung transplantation may be considered medically necessary for carefully selected individuals with end-stage cardiac and pulmonary disease including, but not limited to, one of the following diagnoses:

• Irreversible primary pulmonary hypertension with heart failure;
• Non-specific 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 individuals who meet criteria for heart/lung transplantation.

In all other situations, heart/lung transplantation is considered investigational.

POLICY GUIDELINES:

General Criteria

Potential contraindications for solid organ transplant that are subject to the judgment of the transplant center include the following:

• Known current malignancy, including metastatic cancer;
• Recent malignancy with high incidence of recurrence;
• History of cancer with a moderate risk of recurrence;
• Systemic disease that could be exacerbated by immunosuppression;
• Untreated systemic infection making immunosuppression unsafe, including chronic infection;
• Other irreversible end-stage disease not attributed to heart or lung disease; or
• Psychosocial conditions or chemical dependency affecting ability to adhere to therapy.

Heart/Lung-Specific Criteria

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, the heart shall be allocated to the heart/lung candidate from the same donor "after the heart has been offered to all heart and heart-lung potential transplant recipients in allocation classifications 1 through 4". Candidates with allocation classifications 1 through 4 fall within adult status 1 or 2 or pediatric status 1A.

Specific criteria for prioritizing donor thoracic organs for transplant are provided by the Organ Procurement and Transplantation Network (OPTN) and implemented through a contract with UNOS. Donor thoracic organs are prioritized by UNOS on the basis of recipient medical urgency, distance from donor hospital, and pediatric status. Individuals who are most severely ill (status 1A) are given highest priority.

The following factors are considered in assessing the severity of cardiac illness: reliance on continuous mechanical ventilation, infusion of intravenous inotropes, and/or dependency on mechanical circulatory support (i.e., total artificial heart, intra-aortic balloon pump, extracorporeal membrane oxygenator, ventricular assist device). Factors considered in assessing the severity of pulmonary illness include increased pulmonary artery systolic pressure, pulmonary arterial hypertension, and/or elevated pulmonary vascular resistance.

Additional criteria may be considered in pediatric individuals, including diagnosis of an OPTN-approved congenital heart disease diagnosis, presence of ductal dependent pulmonary or systemic circulation, and diagnosis of hypertrophic or restrictive cardiomyopathy while less than 1-year-old. Of note, pediatric heart transplant candidates who remain on the waiting list at the time of their 18th birthday without receiving a transplant continue to qualify for medical urgency status based on the pediatric criteria.

In both adult and pediatric individuals, isolated cardiac or pulmonary transplantations are preferred to combined heart/lung transplantation when medical or surgical management-other than organ transplantation-is available.

Individuals who are considered temporarily unsuitable to receive a thoracic organ transplant may be assigned an inactive status.

DESCRIPTION OF PROCEDURE OR SERVICE:

Solid organ transplantation offers a treatment option for individuals with different types of end stage organ failure that can be lifesaving or provide significant improvements to an individual’s quality of life. Many advances have been made in the last several decades to reduce perioperative complications. Available data supports improvement in long-term survival as well as improved quality of life particularly for liver, kidney, pancreas, heart, and lung transplants. Allograft rejection remains a key early and late complication risk for any organ transplantation. Transplant recipients require life-long immunosuppression to prevent rejection. Individuals are prioritized for transplant by mortality risk and severity of illness criteria developed by Organ Procurement and Transplantation Network and United Network of Organ Sharing.

Most heart/lung transplant 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. It is possible that pulmonary hypertension could lead to a reversal of the intracardiac shunting and inadequate peripheral oxygenation or cyanosis.

Heart/Lung Transplant

Combined heart/lung transplantation is intended to prolong survival and improve function in individuals with end-stage cardiac and pulmonary diseases. Due to corrective surgical techniques and improved medical management of pulmonary hypertension, the total number of individuals with Eisenmenger syndrome has seen a decline in recent years. Additionally, heart/lung transplants have not increased appreciably, but for other indications, 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. For those indications, individual survival rates following heart/lung transplantations are similar to lung transplant rates. Bronchiolitis obliterans syndrome is a major complication. One-, 5-, and 10-year individual survival rates for heart/lung transplants performed between 1982 and 2014 were estimated at 63%, 45%, and 32%, respectively.

In 2023, 46,630 transplants were performed in the United States procured from more than 16,000 deceased donors and 6,900 living donors. Of these transplants, 54 individuals received heart/lung transplants in the US in 2023 (total 1537 heart-lung transplants done to date in US). As of June 2024, 41 individuals were on the waiting list for heart/lung transplants.

KEY POINTS:

The most recent literature update was performed through June 19, 2024.

Summary of Evidence

For individuals who have end-stage cardiac and pulmonary disease who receive combined heart/lung transplant, the evidence includes a systemic reviews case series and registry data. Relevant outcomes are overall survival, symptoms, morbid events, and treatment-related morbidity and mortality. The available literature reports on outcomes after heart/lung transplantation. Given the exceedingly poor expected survival rates without transplantation, this evidence is sufficient to demonstrate that heart/lung transplantation provides a survival benefit in appropriately selected individuals. A transplant may be the only option for some individuals with end-stage cardiopulmonary disease. Heart/lung transplant is contraindicated for individuals in whom the procedure is expected to be futile due to comorbid disease or for whom posttransplantation care is expected to worsen comorbid conditions significantly. The evidence is sufficient to determine that the technology results in an improvement in the net health outcome.

For individuals who have a combined heart/lung transplant complicated by graft failure or severe dysfunction of the heart/lung and who receive a combined heart/lung transplant, the evidence includes case series and registry data. Relevant outcomes are overall survival, symptoms, morbid events, and treatment-related morbidity and mortality. The available literature describes outcomes after heart/lung transplantation. Given the exceedingly poor expected survival rates without transplantation, this evidence is sufficient to demonstrate that heart/lung transplantation provides a survival benefit in appropriately selected individuals. Transplant may be the only option for some individuals with end-stage cardiopulmonary disease. Heart/lung transplant is contraindicated for individuals in whom the procedure is expected to be futile due to comorbid disease or for whom posttransplantation care is expected to worsen comorbid conditions significantly. The evidence is sufficient to determine that the technology results in an improvement in the net health outcome.

Practice Guidelines and Position Statements

International Society for Heart and Lung Transplantation

In 2021, The International Society for Heart and Lung Transplantation updated its consensus-based guidelines on the selection of lung transplant recipients. These guidelines made the following statements about lung transplantation:

“Lung transplantation should be considered for adults with chronic, end-stage lung disease who meet all the following general criteria:

• High (>50%) risk of death from lung disease within 2 years if lung transplantation is not performed.
• High (>80%) likelihood of 5-year post-transplant survival from a general medical perspective provided that there is adequate graft function.”

For combined heart/lung transplant, the guidelines state:

"Candidates should meet the criteria for lung transplant listing and have significant dysfunction of one or more additional organs, or meet the listing criteria for a non-pulmonary organ transplant and have significant pulmonary dysfunction." The guideline goes on to state: "The primary indication for heart-lung transplant is pulmonary hypertension, either secondary to idiopathic pulmonary arterial hypertension or congenital heart disease (CHD)."

The guidelines also mentioned "...candidates free from complex CHD or left ventricular compromise can achieve comparable outcomes with isolated bilateral lung transplant. Similarly, patients with advanced lung disease and cardiac pathology amenable to surgical repair may be candidates for lung transplant concurrent with the appropriate corrective cardiac procedure."

U.S. Preventive Services Task Force Recommendations

Not applicable.

KEY WORDS:

Heart transplant, lung transplant, transplantation, heart/lung transplant, HLT

APPROVED BY GOVERNING BODIES:

Solid organ transplants are a surgical procedure and, as such, are not subject to regulation by the U.S. Food and Drug Administration.

The U.S. Food and Drug Administration regulates human cells and tissues intended for implantation, transplantation, or infusion through the Center for Biologics Evaluation and Research, under Code of Federal Regulation Title 21, parts 1270 and 1271. Solid organs used for transplantation are subject to these regulations.

BENEFIT APPLICATION:

Coverage is subject to member’s specific benefits.  Group specific policy will supersede this policy when applicable.

ITS: Home Policy provisions apply

FEP contracts: Special benefit consideration may apply. Refer to member’s benefit plan.

CURRENT CODING:

CPT Codes:

REFERENCES:

1. 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 2012; 31(10):1087-1095.
2. Benden C, Goldfarb SB, Edwards LB, et al. The registry of the International Society for Heart and Lung Transplantation: seventeenth official pediatric lung and heart-lung transplantation report--2014; focus theme: retransplantation. J Heart Lung Transplant. Oct 2014; 33(10):1025-1033.
3. Black CK, Termanini KM, Aguirre O et al. Solid organ transplantation in the 21st century. Ann Transl Med. 2018 Oct; six (20).
4. 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. Oct 2010; 29(10):1104-1118.
5. Gilbert S, Dauber JH, Hattler BG, et al. Lung and heart-lung transplantation at the University of Pittsburgh: 1982-2002. Clin Transplant 2002; 253-261.
6. Goldfarb SB, Levvey BJ, Edwards LB, et al. The Registry of the International Society for Heart and Lung Transplantation: Nineteenth Pediatric Lung and Heart-Lung Transplantation Report-2016; Focus Theme: Primary Diagnostic Indications for Transplant. J Heart Lung Transplant. Oct 2016; 35(10):1196-1205.
7. Hill C, Maxwell B, Boulate D, et al. Heart-lung vs. double-lung transplantation for idiopathic pulmonary arterial hypertension. Clin Transplant. Dec 2015; 29(12):1067-1075.
8. International Society for Heart and Lung Transplantation. Pediatric Heart/Lung Transplantation Statistics. 2016; www.ishlt.org/registries/slides.asp?slides=heartLungRegistry.
9. IOM (Institute of Medicine). 2011. Clinical Practice Guidelines We Can Trust. Washington, DC: The National Academies Press.
10. Jayarajan SN, Taghavi S, Komaroff E, et al. Impact of extracorporeal membrane oxygenation or mechanical ventilation as bridge to combined heart-lung transplantation on short-term and long-term survival. Transplantation. Jan 15 2014; 97(1):111-115.
11. Kalogeropoulos AP, Georgiopoulou VV, Giamouzis G, et al. Utility of the Seattle Heart Failure Model in patients with advanced heart failure. J Am Coll Cardiol. Jan 27 2009; 53(4):334-342.
12. Koval CE, Farr M, Krisl J et al. Heart or lung transplant outcomes in HIV-infected recipients. J. Heart Lung Transplant. 2019 Dec; 38(12).
13. Leard LE, Holm AM, Valapour M, et al. Consensus document for the selection of lung transplant candidates: An update from the International Society for Heart and Lung Transplantation. J Heart Lung Transplant. Nov 2021; 40(11): 1349-1379.
14. Mistiaen WP. Heart transplantation in patients with previous malignancy. An overview. Acta Cardiol. Apr 2015; 70(2):123-130.
15. Oliveira GH, Hardaway BW, Kucheryavaya AY, et al. Characteristics and survival of patients with chemotherapy-induced cardiomyopathy undergoing heart transplantation. J Heart Lung Transplant. Aug 2012; 31(8):805-810.
16. Organ Procurement and Transplantation Network (OPTN). Organ Procurement and Transplantation Network Policies. 2024; optn.transplant.hrsa.gov/media/1200/optn_policies.pdf.
17. Organ Procurement and Transplantation Network (OPTN). View Data Reports. n.d.; optn.transplant.hrsa.gov/data/view-data-reports/.
18. Riggs KW, Chapman JL, Schecter M et al. Pediatric heart-lung transplantation: A contemporary analysis of outcomes. Pediatr Transplant. 2020 May; 24(3):  e13682.
19. Sertic F, Han J, Diagne D et al. Not all septal defects are equal. Outcomes of Bilateral Lung Transplant with Cardiac Defect Repair vs. Combined Heart-Lung Transplant in Patients with Eisenmenger's Syndrome in the United States. Chest. Nov 2020; 158(5): 2097-2106.
20. Shuhaiber JH, Kim JB, Gibbons RD. Repeat heart-lung transplantation outcome in the United States. J Heart Lung Transplant 2008; 27(10):1122-1127.
21. Sigurdardottir V, Bjortuft O, Eiskjaer H, et al. Long-term follow-up of lung and heart transplant recipients with pre-transplant malignancies. J Heart Lung Transplant. Dec 2012; 31(12):1276-1280.
22. Spahr JE, West SC. Heart-lung transplantation: pediatric indications and outcomes. J Thorac Dis. Aug 2014; 6(8):1129-1137.
23. The British Transplantation Society. Kidney & Pancreas Transplantation in Patients with HIV. Second Edition. British Transplantation Society Guidelines. 2015; bts.org.uk/wp-content/uploads/2017/04/02_BTS_Kidney_Pancreas_HIV.pdf.
24. United Network for Organ Sharing (UNOS). Lung CAS summary data updated. 2024; unos.org/news/lung-cas-score-summary/.
25. United Network for Organ Sharing (UNOS). Heart/Lung: Submitting LAS exception requests for candidates diagnosed with PH. 2024; unos.org/news/submitting-las-exception-requests-for-candidates-diagnosed-withph/.
26. United Network for Organ Sharing (UNOS). Policy 6.6.F. Organ distribution: allocation of thoracic organs. UNOS Policies and Bylaws. optn.transplant.hrsa.gov/media/eavh5bf3/optn_policies.pdf.
27. United Network for Organ Sharing (UNOS). Transplant trends. 2024; unos.org/data/transplant-trends/.
28. Working Party of the British Transplantation Society. Kidney and Pancreas Transplantation in Patients with HIV. SecondEdition (Revised). British Transplantation Society Guidelines. Macclesfield, UK: British Transplantation Society; Published2015. Updated 2017. bts.org.uk/wp-content/uploads/2017/04/02_BTS_Kidney_Pancreas_HIV.pdf. 
29. Yan HJ, Zheng XY, Huang H, et al. Double-lung versus heart-lung transplantation for end-stage cardiopulmonary disease:a systematic review and meta-analysis. Surg Today. Sep 2023; 53(9): 1001-1012.
30. Yoosabai A, Mehta A, Kang W, et al. Pretransplant malignancy as a risk factor for posttransplant malignancy after heart transplantation. Transplantation. Feb 2015; 99(2):345-350.
31. Yusen RD, Edwards LB, Kucheryavaya AY, et al. The Registry of the International Society for Heart and Lung Transplantation: thirty-first adult lung and heart-lung transplant report--2014; focus theme: retransplantation. J Heart Lung Transplant. Oct 2014; 33(10):1009-1024. 
32. Yusen RD, Edwards LB, Dipchand AI, et al. The Registry of the International Society for Heart and Lung Transplantation:Thirty-third Adult Lung and Heart-Lung Transplant Report-2016; Focus Theme: Primary Diagnostic Indications forTransplant. J Heart Lung Transplant. Oct 2016; 35(10): 1170-1184.

POLICY HISTORY:

Medical Policy Group, July 2009 (2)

Medical Policy Administration Committee, August 2009

Available for comment August 10-September 23, 2009

Medical Policy Group, November 2010 (1): Description updated, Key Points updated, no policy changes, References updated

Medical Policy Panel, November 2011

Medical Policy Group, December 2011 (3): 2012 Code changes: 33960, 33961

Medical Policy Group, February 2012 (4): Updated Policy-diagnosis list, Description, and References

Medical Policy Administration Committee March 2012

Available for comment March 15 – April 30, 2012

Medical Policy Group, November 2012 (3): 2012 Updates to Description, Key Points and References

Medical Policy Panel, November 2013

Medical Policy Group, November 2013 (3): Updates to Description, Key Points and References; policy statement updated to include coverage for heart/lung retransplantation after a failed primary heart/lung transplant

Available for comment January 15 through March 7, 2014

Medical Policy Panel, November 2014

Medical Policy Group, November 2014 (3): 2015 Annual Coding update and annual review.  Added codes 33946-33949, 33951-33959, 33962-33966, 33969, 33984-33989 to current coding and moved 33960, 33961 and 36822 to previous coding. Added policy statement to include all other situations is investigational for heart/lung transplant.  Updated Description, Key Points, and References

Medical Policy Panel, November 2015

Medical Policy Group, December 2015 (2): 2015 Updates to Description, Key Points, and References; updated additional guidelines in the policy statement section; no change in policy statement or intent.

Medical Policy Panel, September 2017

Medical Policy Group, October 2017 (7): 2017 Updates to Description, Key Points, and References; Policy statement- updated guidelines; added HIV criteria; removed policy information from 2013. No change in intent.

Medical Policy Administration Committee October 2017

Available for comment October 10 through November 23, 2017

Medical Policy Panel, August 2018

Medical Policy Group, August 2018 (3): Updates to Description, Key Points, Approved by Governing Bodies, and References; Previous coding section removed: CPT 33960, 33961, and 36822; deleted effective 01/01/15. No change in policy statement or intent.

Medical Policy Panel, August 2019

Medical Policy Group, September 2019 (3): 2019 Updates to Key Points. No change to policy statement or intent.

Medical Policy Panel, August 2020

Medical Policy Group, September 2020 (3): 2020 Updates to Description, Key Points, Approved by Governing Bodies, and References. No changes to policy statement or intent.

Medical Policy Panel, August 2021

Medical Policy Group, August 2021 (3): 2021 Updates to Description and Key Points. Policy statement updated to remove “not medically necessary”, no other changes to policy statement or intent.

Medical Policy Panel, August 2022

Medical Policy Group, August 2022 (3): 2022 Updates to Description, Key Points, Practice Guidelines and Position Statements, and References. Removed HIV positive patient criteria from policy section to reflect standard of care guidelines from the American Society of Transplantation. Removed absolute contraindications in policy guidelines and changed verbiage to “Potential contraindications for solid organ transplant that are subject to the judgment of the transplant center.” No other changes to policy statement or intent.

Medical Policy Panel, August 2023

Medical Policy Group, October 2023 (5): Updates to Description, Key Points, Benefit Application, and References. No change to Policy Statement.

Medical Policy Panel, August 2024

Medical Policy Group, August 2024 (3): Updates to Description, Key Points, and References. No change to the policy statement or intent.

This medical policy is not an authorization, certification, explanation of benefits, or a contract. Eligibility and benefits are determined on a case-by-case basis according to the terms of the member’s plan in effect as of the date services are rendered. All medical policies are based on (i) research of current medical literature and (ii) review of common medical practices in the treatment and diagnosis of disease as of the date hereof. Physicians and other providers are solely responsible for all aspects of medical care and treatment, including the type, quality, and levels of care and treatment.

This policy is intended to be used for adjudication of claims (including pre-admission certification, pre-determinations, and pre-procedure review) in Blue Cross and Blue Shield’s administration of plan contracts.

The plan does not approve or deny procedures, services, testing, or equipment for our members. Our decisions concern coverage only. The decision of whether or not to have a certain test, treatment or procedure is one made between the physician and his/her patient. The plan administers benefits based on the member’s contract and corporate medical policies. Physicians should always exercise their best medical judgment in providing the care they feel is most appropriate for their patients. Needed care should not be delayed or refused because of a coverage determination.

As a general rule, benefits are payable under health plans only in cases of medical necessity and only if services or supplies are not investigational, provided the customer group contracts have such coverage.

The following Association Technology Evaluation Criteria must be met for a service/supply to be considered for coverage:

1. The technology must have final approval from the appropriate government regulatory bodies;

2. The scientific evidence must permit conclusions concerning the effect of the technology on health outcomes;

3. The technology must improve the net health outcome;

4. The technology must be as beneficial as any established alternatives;

5. The improvement must be attainable outside the investigational setting.

Medical Necessity means that health care services (e.g., procedures, treatments, supplies, devices, equipment, facilities or drugs) that a physician, exercising prudent clinical judgment, would provide to a patient for the purpose of preventing, evaluating, diagnosing or treating an illness, injury or disease or its symptoms, and that are:

1. In accordance with generally accepted standards of medical practice; and

2. Clinically appropriate in terms of type, frequency, extent, site and duration and considered effective for the patient’s illness, injury or disease; and

3. Not primarily for the convenience of the patient, physician or other health care provider; and

4. Not more costly than an alternative service or sequence of services at least as likely to produce equivalent therapeutic or diagnostic results as to the diagnosis or treatment of that patient’s illness, injury or disease.