Aetna considers heart-lung transplantation medically necessary for persons with severe refractory heart failure plus either end-stage lung disease or irreversible pulmonary hypertension, when the selection criteria listed below are met and no absolute contraindications listed below are present. Examples of qualifying conditions include the following:
Chronic obstructive pulmonary disease with severe heart failure*
Congenital heart disease associated with pulmonary hypertension that are not amenable to lung transplantation and repair by standard cardiac surgery
Cystic fibrosis with severe heart failure*
Eisenmenger’s complex with irreversible pulmonary hypertension and severe heart failure*
Irreversible primary pulmonary hypertension with severe heart failure*
Pulmonary fibrosis with uncontrollable pulmonary hypertension or severe heart failure*
Severe coronary artery disease or cardiomyopathy with irreversible pulmonary hypertension
Severe pulmonary fibrosis with severe heart failure*
* Severe (New York Heart Association (NYHA) classification III or IV (see Appendix)) heart failure where right ventricular function would not be restored with lung transplant alone.
Note: Heart-lung transplantation may be considered medically necessary for other congenital cardiopulmonary anomalies upon individual case review.
Selection Criteria: The member must meet the transplanting institution’s selection criteria. In the absence of an institution's selection criteria, Aetna considers heart-lung transplantation medically necessary when all of the criteria below are met:
Absence of chronic high-dose steroid therapy. Due to problems in bronchial healing, persons receiving high-dose steroids are considered inappropriate candidates; and
Absence of acute or chronic active infections that are not effectively treated; and
Absence of malignancy (other than non-melanomatous skin cancers) or malignancy has been completely resected or (upon medical review) it is determined that malignancy has been treated with small likelihood of recurrence and acceptable future risks; and
Adequate functional status. Active rehabilitation is considered important to the success of transplantation. Under established guidelines, mechanically ventilated or otherwise immobile persons are considered poor candidates for transplantation; however, bridge to transplant with ambulatory ECMO does not, in itself, rule out candidacy for heart-lung transplantation; and
Adequate liver and kidney function, defined as a bilirubin of less than 2.5 mg/dL and a creatinine clearance of greater than 50 ml/min/kg; and
Life expectancy (in the absence of cardiopulmonary disease) of greater than 2 years; and
No active alcohol or chemical dependency that interferes with compliance to a strict treatment regimen; and
No uncontrolled and/or untreated psychiatric disorders that interfere with compliance to a strict treatment regimen; and
HIV/AIDS, if present, is under adequate control, defined as:
CD4 count greater than 200 cells/mm3 for more than 6 months; and
HIV-1 RNA (viral load) undetectable; and
On stable anti-viral therapy more than 3 months; and
No other complications from AIDS, such as opportunistic infections (e.g., aspergillus, tuberculosis, Pneumocystis carinii pneumonia, toxoplasmosis encephalitis, cryptococcal meningitis, disseminated coccidioidomycosis, other resistant fungal infections) or neoplasms (e.g., Kaposi's sarcoma, non-Hodgkin’s lymphoma).
Heart-Lung transplant is considered not medically necessary for persons with any of the following contraindications because the risks of transplantation exceed the benefits:
Gastro-intestinal disease (e.g., bleeding peptic ulcer, diverticulitis, chronic hepatitis, active or recurrent pancreatitis)
Multi-system disease. Persons with potentially multi-system diseases such as systemic sclerosis (scleroderma) or other collagen vascular diseases such as systemic lupus erythematosus must be carefully evaluated to ensure that their disease is primarily confined to the lung. Persons with diabetes must be carefully evaluated to rule out significant diabetic complications such as nephropathy, neuropathy or retinopathy.
Other effective medical treatments or surgical options are available
Progressive neuromuscular disease
Refractory uncontrolled hypertension
Severe musculoskeletal disease with debilitating thoracic involvement
Smoking. Persons with a history of smoking must be abstinent for at least 3 months before being considered a candidate for lung transplant
Untreated or unstable cerebrovascular disease.
Reduced cardiac output and edema characterize patients with heart failure, while patients with respiratory failure exhibit abnormalities in oxygenation and carbon dioxide elimination that impair the functioning vital organs. These patients have a lower life expectancy and decreased quality of life. Since its introduction at Stanford University in 1981 and at Pittsburgh University in 1982 for the treatment of Eisenmenger's syndrome and terminal pulmonary vascular disease, heart-lung transplantation has become successful therapeutic options for patients with end-stage cardiopulmonary disease. Heart-lung transplantation is most frequently performed for patients with congenital heart disease (about 30 %), primary pulmonary hypertension (about 27 %), cystic fibrosis (about 16 %), miscellaneous conditions (about 15 %), lung emphysema (about 4 %), re-transplantation following a failed primary transplant (about 3 %), idiopathic pulmonary fibrosis (about 3 %) and alpha 1-antitrypsin deficiency (about 2 %).
Adults who have undergone heart-lung transplantation for congenital heart disease are expected to have survival comparable to that of adults without congenital heart disease. Furthermore, heart-lung transplantation results in survival comparable to that reported for single- or double-lung transplantation for patients with primary pulmonary hypertension. Obliterative bronchiolitis, a form of chronic rejection, is a significant cause of late death.
The frequency of heart-lung transplantation is partly limited by the number of available donor organs. There are fewer donor heart-lung preparations than donor heart preparations alone because brain death may be associated with neurogenic pulmonary edema. In addition, aspiration into the lung is common during the course of severe trauma and resuscitation. Prolonged ventilatory support may also predispose the potential donor to nosocomial infection, and direct thoracic trauma may result in pulmonary contusion. As a consequence, probably less than 20 % of potential heart donors have lungs that are suitable for heart-lung transplantation. It should be noted that heart-lung transplantation in some ways is a technically easier procedure than heart transplantation since the former requires only right atrial, aortic, and tracheal anastomoses, thus avoiding several of the anastomoses associated with heart transplantation.
Contraindications to heart-lung transplantation include irreversible end-organ diseases (e.g., renal, hepatic), active malignancy or infections, systemic diseases (e.g., autoimmune, vascular, amyloidosis), chronic gastro-intestinal disease (e.g., diverticulitis, active or recurrent pancreatitis, bleeding peptic ulcer), psychiatric disorders, cerebrovascular disease, progressive neuromuscular disease, and use of tobacco products. Under established guidelines, obese (greater than 20 % of ideal body weight), cachectic (less than 80 % of ideal body weight), mechanically ventilated or otherwise immobile patients are considered poor candidates for transplantation.
Early post-operative complications (within the first post-operative month) comprise acute isolated lung rejection, multi-organ failure, and bacterial pneumonia. Late post-operative complications (after 1 post-operative month) comprise viral pneumonia, fungal infection, tuberculosis, and chronic obliterative bronchiolitis.
Zheng et al (2011) examined the safety and possible benefits of laparoscopic anti-reflux surgery in pediatric patients following lung and heart-lung transplantation. An Institutional Review Board-approved retrospective chart review was performed to evaluate the outcomes and complications of laparoscopic anti-reflux surgery in pediatric lung and heart-lung transplant patients. Spirometry data were collected for bronchiolitis obliterans syndrome (BOS) staging using BOS criteria for children. A total of 25 lung and heart-lung transplants were performed between January 2003 and July 2009. Eleven transplant recipients, including 6 double-lung and 5 heart-lung, with a median age of 11.7 years (range of 5.1 to 18.4 years), underwent a total of 12 laparoscopic Nissen fundoplications at a median of 427 days after transplant (range of 51 to 2310 days). The diagnosis of gastro-esophageal reflux disease (GERD) was made based upon clinical impression, pH probe study, gastric emptying study, and/or esophagram in all patients. Three patients already had a gastrostomy tube in place and 2 had one placed at the time of fundoplication. There were no conversions to open surgery, 30-day re-admissions, or 30-day mortalities. Complications included 1 exploratory laparoscopy for free air 6 days after laparoscopic Nissen fundoplication for a gastric perforation that had spontaneously sealed. Another patient required a revision laparoscopic Nissen 822 days following the initial fundoplication for a para-esophageal hernia and recurrent GERD. The average length of hospital stay was 4.4 +/- 1.7 days. Nine of the 12 fundoplications were performed in patients with baseline spirometry values prior to fundoplication and who could also complete spirometry reliably. One of these 9 operations was associated with improvement in BOS stage 6 months after fundoplication; 7 were associated with no change in BOS stage; and 1 was associated with a decline in BOS stage. The authors concluded that it is feasible to perform laparoscopic Nissen fundoplication in pediatric lung and heart-lung transplant recipients without mortality or significant morbidity for the treatment of GERD. The real effect on pulmonary function can not be assessed due to the small sample size and lack of reproducible spirometry in the younger patients. The authors stated that additional studies are needed to elucidate the relationship between anti-reflux surgery and the potential for improving pulmonary allograft function and survival in children that has been previously observed in adult patients.
The New York Heart Association (NYHA) classification of heart failure is one of the many parameters used for selecting heart-lung recipient. It is a 4-tier system that categorizes patients based on subjective impression of the degree of functional compromise. The 4 NYHA functional classes are as follows:
Patients with cardiac disease but without resulting limitation of physical activity. Ordinary physical activity does not cause undue fatigue, palpitation, dyspnea, or anginal pain. Symptoms only occur on severe exertion.
Patients with cardiac disease resulting in slight limitation of physical activity. They are comfortable at rest. Ordinary physical activity (e.g., moderate physical exertion such as carrying shopping bags up several flights or stairs) results in fatigue, palpitation, dyspnea, or anginal pain.
Patients with cardiac disease resulting in marked limitation of physical activity. They are comfortable at rest. Less than ordinary activity (i.e., mild exertion) causes fatigue, palpitation, dyspnea, or anginal pain.
Patients with cardiac disease resulting in inability to carry on any physical activity without discomfort. Symptoms of cardiac insufficiency or of the anginal syndrome may be present even at rest. If any physical activity is undertaken, discomfort is increased.
CPT Codes / HCPCS Codes / ICD-9 Codes
CPT codes covered if selection criteria are met::
33930 - 33945
HCPCS codes covered if selection criteria are met::
Transplantation of multivisceral organs
Harvesting of donor multivisceral organs, with preparation and maintenance of allografts; from cadaver donor
Lobar lung transplantation
Donor lobectomy (lung) for transplantation, living donor
ICD-9 codes covered if selection criteria are met::
277.00 - 277.09
414.00 - 414.07
416.0 - 416..9
Chronic pulmonary heart disease
425.0 - 425.9
428.0 - 428.9
490 - 496
Chronic obstructive pulmonary disease and allied conditions
Post-inflammatory pulmonary fibrosis
745.0 - 746.9
Bulbus cordis anomalies and anomalies of cardiac septal closure and other congenital anomalies of heart
Complications of transplanted organ, heart
Complications of transplanted organ, lung
Other ICD-9 codes related to the CPB::
001.0 - 139.8
Infectious and parasitic diseases
140.0 - 208.91
250.00 - 250.93
Other deficiencies of circulating enzymes (e.g., alpha1-antitrypsin deficiency, hereditary angioedema)
Other specified disorders of metabolism (e.g., eosinophil granuloma)
290.0 - 316
Psychoses, other psychoses, and neurotic disorders, personality disorders, and other nonpsychotic mental disorders
358.0 - 359.9
Myoneural disorders, muscular dystrophies and other myopathies
401.0 - 405.99
Endocarditis, valve unspecified
430 - 438.9
Idiopathic fibrosing alveolitis
531.00 - 537.9
Diseases of stomach and duodenum
570 - 573.9
Acute and subacute necrosis of liver, chronic liver disease and cirrhosis, and liver abscess and sequelae of chronic liver disease, and other disorders of liver
Anomalies of pulmonary artery (e.g., agenesis, atresia)
Congenital cystic lung
Agenesis, hypoplasia, and dysplasia of lung
Chronic respiratory disease arising in the perinatal period (e.g., bronchopulmonary dysplasia)
V10.0 - V10.9
Personal history of malignant neoplasm
History of tobacco use
Organ or tissue replaced by transplant, heart
Organ or tissue replaced by transplant, lung
V46.0 - V46.9
Other dependence on machines [ambulatory ECMO is not a contraindication to heart-lung transplantation]
The above policy is based on the following references:
Pigula FA, Gandhi SK, Ristich J, et al. Cardiopulmonary transplantation for congenital heart disease in the adult. J Heart Lung Transplant. 2001;20(3):297-303.
Green I. Institutional and patient criteria for heart-lung transplantation. Health Technology Assessment No. 1. AHCPR Pub. No. 94-0042. Rockville, MD: Agency for Health Care Policy and Research (AHCPR); May 1994.
Steinman TI, Becker BN, Frost AE, et al. Guidelines for the referral and management of patients eligible for solid organ transplantation. Transplantation. 2001;71(9):1189-1204.
Barlow CW, Robbins RC, Moon MR, et al. Heart-lung versus double-lung transplantation for suppurative lung disease. J Thorac Cardiovasc Surg. 2000;119(3):466-476.
Ro PS, Spray TL, Bridges ND. Outcome of infants listed for lung or heart/lung transplantation. J Heart Lung Transplant. 1999;18(12):1232-1237.
Whyte RI, Robbins RC, Altinger J, et al. Heart-lung transplantation for primary pulmonary hypertension. Ann Thorac Surg. 1999;67(4):937-941; discussion 941-942.
Conte JV Jr. Thoracic transplantation in 1998. Md Med J. 1998;47(5):235-240.
McLaughlin VV, Rich S. Pulmonary hypertension -- advances in medical and surgical interventions. J Heart Lung Transplant. 1998;17(8):739-743.
Noyes BE, Kurland G, Orenstein DM. Lung and heart-lung transplantation in children. Pediatr Pulmonol. 1997;23(1):39-48.
Bando K, Armitage JM, Paradis IL, et al. Indications for and results of single, bilateral, and heart-lung transplantation for pulmonary hypertension. J Thorac Cardiovasc Surg. 1994;108(6):1056-1065.
Hosenpud JD, Novick RJ, Breen TJ, et al. The registry of the International Society for Heart and Lung Transplantation: Eleventh official report -- 1994. J Heart Lung Transplant. 1994;13(4):561-570.
Adams DH, Cochrane AD, Khaghani A, et al. Retransplantation in heart-lung recipients with obliterative bronchiolitis. J Thorac Cardiovasc Surg. 1994;107(2):450-459.
Scott JP, Dennis C, Mullins P. Heart-lung transplantation for end-stage respiratory disease in cystic fibrosis patients. J R Soc Med. 1993;86(Suppl 20):19-22.
McCarthy PM, Kirby TJ, White RD, et al. Lung and heart-lung transplantation: The state of the art. Cleve Clin J Med. 1992;59(3):307-316.
Kendall SW, Wallwork J. Heart-lung transplantation: Indications and technique. Semin Thorac Cardiovasc Surg. 1992;4(2):101-106.
Madden BP, Hodson ME, Tsang V, et al. Intermediate-term results of heart-lung transplantation for cystic fibrosis. Lancet 1992;339(8809):1583-1587.
Pierce JA, Sobel BE. Frontiers in surgery. A perspective. JAMA 1991;266(14):1990-1992.
LeGal YM. Lung and heart-lung transplantation. Ann Thorac Surg. 1990;49(5):840-844.
Reichart B, Vosloo S, Holl J. Surgical management of heart-lung transplantation. Ann Thorac Surg. 1990;49(2):333-340.
Burch M, Aurora P. Current status of paediatric heart, lung, and heart-lung transplantation. Arch Dis Child. 2004;89(4):386-389.
Doyle RL, McCrory D, Channick RN, et al.; American College of Chest Physicians. Surgical treatments/interventions for pulmonary arterial hypertension: ACCP evidence-based clinical practice guidelines. Chest. 2004;126(1 Suppl):63S-71S.
Webber SA, McCurry K, Zeevi A. Heart and lung transplantation in children. Lancet. 2006;368(9529):53-69.
Zheng C, Kane TD, Kurland G, et al. Feasibility of laparoscopic Nissen fundoplication after pediatric lung or heart-lung transplantation: Should this be the standard? Surg Endosc. 2011;25(1):249-254.
Scouras NE, Matsusaki T, Boucek CD, et al. Portopulmonary hypertension as an indication for combined heart, lung, and liver or lung and liver transplantation: Literature review and case presentation. Liver Transpl. 2011;17(2):137-143.
Hayes D Jr, Galantowicz M, Hoffman TM. Combined heart-lung transplantation: A perspective on the past and the future. Pediatr Cardiol. 2013;34(2):207-212.
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