Latest Review Date: June 2024

Category: Surgery                                                                 

POLICY:

Native Valve:

Transcatheter pulmonary valve implantation, when using an FDA-approved device according to its specific indications, may be considered medically necessary for individuals with congenital heart disease and current right ventricular outflow tract obstruction or regurgitation including the following indications:

• A dysfunctional , non-compliant right ventricular outflow tract (RVOT) with a clinical indication for intervention , and:
  • Pulmonary regurgitation ≥ moderate; or
  • A mean RVOT gradient ≥35mmHg

Valve-in-Valve:

Transcatheter pulmonary valve implantation, when using an FDA-approved device according to its specific indications (i.e. Melody), may be considered medically necessary for patients with congenital heart disease and current right ventricular outflow tract obstruction or regurgitation including the following indications:

• A surgical bioprosthetic pulmonary valve that has:
  •  ≥ moderate regurgitation; OR
  • A mean RVOT gradient ≥ 35mmHG

Transcatheter pulmonary valve implantation is considered investigational for all other indications.

DESCRIPTION OF PROCEDURE OR SERVICE:

Transcatheter pulmonary valve implantation (TVPI) has been proposed as a less invasive alternative to open surgical pulmonary valve replacement or reconstruction for right ventricular outflow tract (RVOT) obstruction. Percutaneous pulmonary valve replacement may be indicated for congenital pulmonary stenosis. Pulmonary stenosis or regurgitation in a patient with congenital heart disease (CHD) who has previously undergone RVOT surgery are additional indications. Patients with prior CHD repair are at risk of needing repeated reconstruction procedures.

Congenital Heart Disease

Congenital heart disease, including tetralogy of Fallot, pulmonary atresia, and transposition of the great arteries, is generally treated by surgical repair at an early age. This involves reconstruction of the right ventricular outflow tract (RVOT) and pulmonary valve by means of a surgical homograft or a bovine-derived valved conduit. These repairs are prone to development of pulmonary stenosis or regurgitation over long periods of follow-up.

Because individuals with surgically corrected congenital heart disease repair are living into adulthood, RVOT dysfunction following initial repair has become more common. Calcification of the RVOT conduit can lead to pulmonary stenosis, while aneurysmal dilatation can result in pulmonary regurgitation. RVOT dysfunction can lead to decreased exercise tolerance, potentially fatal arrhythmias, and/or irreversible right ventricular dysfunction.

Treatment

Treatment options for pulmonary stenosis are open surgery with valve replacement, balloon dilatation, or percutaneous stenting. Interventions for pulmonary regurgitation are primarily surgical, either reconstruction of the RVOT conduit or replacement of the pulmonary valve. The optimal timing of these interventions is not well understood.

KEY POINTS:

The most recent update with literature review is through April 18, 2024.

Summary of Evidence

For individuals with a history of congenital heart disease and current RVOT obstruction who receive TPVI with an FDA-approved device according to FDA indications, the evidence includes a systematic review of retrospective comparative studies, prospective, interventional, noncomparative studies, and a multicenter registry of 2476 individuals who underwent TPV replacement with a Melody (82%) or Sapien (18%) valve between July 2005 and March 2020. Relevant outcomes are overall survival, symptoms, functional outcomes, quality of life, hospitalizations, and treatment-related morbidity and mortality. Overall, the evidence suggests that TPVI is associated with high rates of short-term technical success and improvements in heart failure-related symptoms and hemodynamic parameters. Most valves demonstrate competent functioning by Doppler echocardiography at 6- to 12-month follow-up. Publications with longer follow-up have reported stent fractures in up to 26% of patients; however, most stent fractures did not required reintervention. Studies with follow-up extending to a maximum of 8 years postprocedure suggest that the functional and hemodynamic improvements are durable. The evidence is sufficient to determine that the technology results in an improvement in the net health outcome.

For individuals with a history of congenital heart disease and current RVOT obstruction who receive TPVI with a non-FDA-approved indication or device, the evidence includes case series. Relevant outcomes are overall survival, symptoms, functional outcomes, quality of life, hospitalizations, and treatment-related morbidity and mortality. There is limited evidence on the off-label use of TPVI, including the use of a non-FDA approved valve or use of an approved valve for a non-FDA-approved indication. The published case series enrolled relatively few patients and are heterogeneous in terms of the device used and the indications for TPVI. The evidence is insufficient to determine that the technology results in an improvement in the net health outcome.

Practice Guidelines and Position Statements

Society for Cardiovascular Angiography and Interventions and the Adult Congenital Heart Association

In 2020, the Society for Cardiovascular Angiography and Interventions and the Adult Congenital Heart Association published a position statement on operator and institutional recommendations for transcatheter pulmonary valve implantation (TPVI). Included were recommendations for interventional training, practicing physician competency, ongoing education and training, and institutional and team requirements.

American College of Cardiology, American Heart Association et al

In 2018, the American College of Cardiology and American Heart Association and 6 other societies published comprehensive guidelines on the management of patients with congenital heart disease. Included are recommendations for treatment of pulmonary stenosis, pulmonary regurgitation and tetralogy of Fallot (Table 1).

Table 1. ACC/AHA Guidelines on the Management of Patients with Tetralogy of Fallot

ACC/AHA: American College of Cardiology/American Heart Association; B-NR: Non-randomized (moderate quality evidence); C-EO: consensus of expert opinion; LOE: level of evidence, SOR: strength of recommendation; TOF: tetrology of Fallot; PR: pulmonary regurgitation

U.S. Preventive Services Task Force Recommendations

Not applicable.

KEY WORDS:

Pulmonary valve prosthesis, endovascular implantation, transcatheter pulmonary valve, TPVI, Melody, Ensemble Transcatheter Valve Delivery System, Edwards SAPIEN® Pulmonic Valve, right ventricular outflow tract, RVOT, percutaneous pulmonary valve implantation, PPVI

APPROVED BY GOVERNING BODIES:

Devices for transcatheter pulmonary valve implantation were initially available through a humanitarian device exemption (HDE) or used off-label until full premarket approval (see Table 2).

Table 2. Regulatory Status of Transcatheter Pulmonary Valve Implantation Devices

HDE: humanitarian device exemption; PMA: premarket approval; RVOT: right ventricular outflow tract.

In January 2010, the Melody® Transcatheter Pulmonary Valve (TPV) and the Ensemble® Transcatheter Valve Delivery System (Medtronic, Minneapolis, MN) were approved by the U.S. Food and Drug Administration (FDA) under the HDE program for use as an adjunct to surgery in the management of pediatric and adult patients with the following clinical conditions:

• Existence of a full (circumferential) right ventricular outflow tract (RVOT) conduit that is 16 mm or greater in diameter when originally implanted, and
• Dysfunctional RVOT conduits with clinical indication for intervention, and either:
  • regurgitation: moderate-to-severe regurgitation, or
  • stenosis: mean RVOT gradient ≥35 mm Hg.

On January 27, 2015, approval of the Melody® system was amended to a PMA because FDA determined that the device represented a breakthrough technology. The PMA was based, in part, on 2 prospective clinical studies, the Melody® TPV Long-term Follow-up Post Approval Study (PAS) and the Melody TPV New Enrollment PAS.

On February 24, 2017, approval of the Melody® system was expanded to include patients with a dysfunctional surgical bioprosthetic valve (valve in valve).

The Edwards Sapien XT™ Transcatheter Heart Valve (Pulmonic) (Edwards Lifesciences. It was approved by the FDA in 2016 “for use in pediatric and adult patients with a dysfunctional, noncompliant Right Ventricular Outflow Tract (RVOT) conduit with a clinical indication for intervention and:

• pulmonary regurgitation ≥ moderate and/or
• mean RVOT gradient ≥ 35 mmHg.”

The approval for the pulmonic valve indication is a supplement to the 2014 premarket approval for use of the Edwards SAPIEN XT™ Transcatheter Heart Valve (THV) System for relief of aortic stenosis in patients with symptomatic heart disease due to severe native calcific aortic stenosis and who are judged by a heart team, including a cardiac surgeon, to be at high or greater risk for open surgical therapy (i.e., Society of Thoracic Surgeons operative risk score ≥8% or at a ≥15% risk of mortality at 30 days).

The Harmony™ Transcatheter Pulmonary Valve (Medtronic) received breakthrough technology status in 2019 and PMA in 2021. This device is indicated "for use in pediatric and adult patients with severe pulmonary regurgitation (determined by echocardiography and/or pulmonary regurgitant fraction ≥ 30% by cardiac magnetic resonance imaging) who have a native or surgically-repaired right ventricular outflow tract and are clinically indicated for surgical pulmonary valve replacement."

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

CURRENT CODING:

CPT codes:

REFERENCES:

1. Aboulhosn JA, Hijazi ZM, Kavinsky CJ, et al. SCAI position statement on adult congenital cardiac interventional training, competencies and organizational recommendations. Catheter Cardiovasc Interv. Sep 01 2020; 96(3): 643-650.
2. Armstrong AK, Balzer DT, Cabalka AK, et al. One-year follow-up of the Melody transcatheter pulmonary valve multicenter post-approval study. JACC Cardiovasc Interv. Nov 2014;7(11):1254-1262.
3. Batra AS, McElhinney DB, Wang W et al.  Cardiopulmonary exercise function among patients undergoing transcatheter pulmonary valve implantation in the US Melody valve investigational trial.  Am Heart J.  2012; 163(2):280-7).
4. Boshoff D, Cools B, Heying R et al.  Off-label use of percutaneous pulmonary valved stents in the right ventricular outflow tract:  Time to rewrite the label?  Catheter. Cardiovasc. Interv. 2012.
5. Boudjemline Y, Malekzadeh-Milani S, Patel M, et al. Predictors and outcomes of right ventricular outflow tract conduit rupture during percutaneous pulmonary valve implantation: a multicentre study. EuroIntervention. Sep 22 2014.
6. Cheatham JP, Hellenbrand WE, Zahn EM, et al. Clinical and hemodynamic outcomes up to 7 years after transcatheter pulmonary valve replacement in the US melody valve investigational device exemption trial. Circulation. Jun 2 2015; 131(22):1960-1970.
7. Cheatham SL, Holzer RJ, Chisolm JL et al. The Medtronic Melody® Transcatheter pulmonary valve implanted at 24-mm diameter-it works. Catheter Cardiovasc Interv 2013.
8. Demkow M, Ruzyllo W, Biernacka EK, et al. Percutaneous edwards SAPIEN valve implantation for significant pulmonary regurgitation after previous surgical repair with a right ventricular outflow patch. Catheter Cardiovasc Interv. Feb 15 2014; 83(3):474-481.
9. FDA Summary of Safety and Probable Benefit. Melody® Transcatheter Pulmonary Valve and Ensemble® Transcatheter Valve Delivery System. Available online at www.accessdata.fda.gov/cdrh_docs/pdf8/H080002b.pdf.
10. FDA. Summary of Safety and Effectiveness Data: Melody™ Transcatheter Pulmonary Valve. 2015; //www.accessdata.fda.gov/cdrh_docs/pdf14/p140017b.pdf.
11. FDA. Summary of Safety and Effectiveness Data: Melody™ Transcatheter Pulmonary Valve, models PB1016 and PB1018; Ensemble™ Transcatheter Valve Delivery System 2017; https://www.accessdata.fda.gov/cdrh_docs/pdf14/p140017s005b.pdf.
12. FDA. Summary of Safety and Effectiveness Data: Edwards SAPIEN XT™ Transcatheter Heart Valve. 2016; https://www.accessdata.fda.gov/cdrh_docs/pdf13/p130009s037b.pdf. Accessed May 31, 2017.
13. FDA. Summary of Safety and Effectiveness: Harmony Transcatheter Pulmonary Valve.2021. https://www.accessdata.fda.gov/cdrh_docs/pdf20/P200046B.pdf.
14. FDA. Conditions for Approval for an HDE: Medtronic Melody® Transcatheter Pulmonary Valve (Model PB 10) and Medtronic Ensemble® Transcatheter Valve Delivery System (NU10) (H080002). 2010; https://www.accessdata.fda.gov/cdrh_docs/pdf8/H080002A.pdf.
15. Fraisse A, Assaidi A, Mauri L, et al. Coronary artery compression during intention to treat right ventricle outflow with percutaneous pulmonary valve implantation: incidence, diagnosis, and outcome. Catheter Cardiovasc Interv. Jun 1 2014; 83(7):E260-268.
16. Georgiev S, Ewert P, Eicken A, et al. Munich Comparative Study: Prospective Long-Term Outcome of the Transcatheter Melody Valve Versus Surgical Pulmonary Bioprosthesis With Up to 12 Years of Follow-Up. Circ Cardiovasc Interv. Jul 2020; 13(7): e008963
17. Gillespie MJ, Bergersen L, Benson LN, et al. 5-Year outcomes from the harmony native outflow tract early feasibility study. JACC Cardiovasc Interv. Apr 12 2021; 14(7): 816-817.
18. Gillespie MJ, McElhinney DB, Jones TK, et al. 1-Year Outcomes in a Pooled Cohort of Harmony Transcatheter Pulmonary Valve Clinical Trial Participants. JACC Cardiovasc Interv. Aug 14 2023; 16(15): 1917-1928.
19. Gillespie MJ, McElhinney DB, Kreutzer J, et al. Transcatheter Pulmonary Valve Replacement for Right Ventricular Outflow Tract Conduit Dysfunction after the Ross Procedure. Ann Thorac Surg. Sep 2015; 100(3):996-1003.
20. IOM (Institute of Medicine). 2011. Clinical Practice Guidelines We Can Trust. Washington, DC: The National Academies Press.
21. Jones TK, McElhinney DB, Vincent JA, et al. Long-Term Outcomes After Melody Transcatheter Pulmonary Valve Replacement in the US Investigational Device Exemption Trial. Circ Cardiovasc Interv. Jan 2022; 15(1): e010852.
22. Khambadkone S, Nordmeyer J, Bonhoeffer P. Percutaneous implantation of the pulmonary and aortic valves: indications and limitations. J Cardiovasc Med 2007; 8(1):57-61.
23. Lopez KN, Baker-Smith C, Flores G, et al. Addressing Social Determinants of Health and Mitigating Health DisparitiesAcross the Lifespan in Congenital Heart Disease: A Scientific Statement From the American Heart Association. J AmHeart Assoc. Apr 19 2022; 11(8): e025358.
24. Malekzadeh-Milani S, Ladouceur M, Iserin L, et al. Incidence and outcomes of right-sided endocarditis in patients with congenital heart disease after surgical or transcatheter pulmonary valve implantation. J Thorac Cardiovasc Surg. Aug 9 2014.
25. Malekzadeh-Milani S, Ladouceur M, Patel M, et al. Incidence and predictors of Melody(R) valve endocarditis: a prospective study. Arch Cardiovasc Dis. Feb 2015;108(2):97-106.
26. Malekzadeh-Milani S, Ladouceur M, Cohen S, et al. Results of transcatheter pulmonary valvulation in native or patched right ventricular outflow tracts. Arch Cardiovasc Dis. Sep 10 2014.
27. Markham R, Challa A, Kyranis S, et al. Outcomes following Melody transcatheter pulmonary valve implantation for right ventricular outflow tract dysfunction in repaired congenital heart disease: first reported Australian single centre experience. Heart Lung Circ. Jan 24 2017.
28. McElhinney DB, Cabalka AK, Aboulhosn JA, et al. Transcatheter tricuspid valve-in-valve implantation for the treatment of dysfunctional surgical bioprosthetic valves: an international multicenter registry study. Circulation. Mar 18 2016.
29. McElhinney DB, Cheatham JP, Jones TK et al.  Stent fracture, valve dysfunction, and right ventricular outflow tract reintervention after transcatheter pulmonary valve implantation:  patient-related and procedural risk factors in the US Melody Valve Trial.  Circ. Cardiovasc. Interv. 2011; 4(6):602-14.
30. McElhinney DB, Hellenbrand WE, Zahn EM et al. Short- and medium-term outcomes after transcatheter pulmonary valve placement in the expanded multicenter US Melody Valve Trial. Circulation 2010; 122(5):507-16.
31. McElhinney DB, Zhang Y, Levi DS, et al. Reintervention and survival after transcatheter Pulmonary valve replacement. J Am Coll Cardiol. Jan 04 2022; 79(1): 18-32.
32. McElhinney DB, Zhang Y, Aboulhosn JA, et al. Multicenter study of endocarditis after transcatheter pulmonary valve Replacement. J Am Coll Cardiol. Aug 10 2021; 78(6): 575-589.
33. Meadows JJ, Moore PM, Berman DP, et al. Use and performance of the Melody Transcatheter Pulmonary Valve in native and postsurgical, nonconduit right ventricular outflow tracts. Circ Cardiovasc Interv. Jun 2014; 7(3):374-380.
34. Morray BH, McElhinney DB, Cheatham JP, et al. Risk of coronary artery compression among patients referred for transcatheter pulmonary valve implantation: a multicenter experience. Circ Cardiovasc Interv. Oct 1 2013; 6(5):535-542.
35. Muller J, Engelhardt A, Fratz S, et al. Improved exercise performance and quality of life after percutaneous pulmonary valve implantation. Int J Cardiol. May 15 2014; 173(3):388-392.
36. National Institute for Health and Care Excellence. Percutaneous pulmonary valve implantation for right ventricular outflow tract dysfunction. January 2013. Available at: https://www.nice.org.uk/guidance/ipg436.
37. Nishimura RA, Otto CM, Bonow RO, et al. 2017 AHA/ACC Focused Update of the 2014 AHA/ACC Guideline for the Management of Patients With Valvular Heart Disease: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines. J Am Coll Cardiol. Mar 10 2017.
38. Odemis E, Guzeltas A, Saygi M et al. Percutaneous Pulmonary Valve Implantation Using Edwards SAPIEN Transcatheter Heart Valve in Different Types of Conduits: Initial Results of a Single Center Experience. Congenit Heart Dis Sep-Oct 2013; 8(5):411-7.
39. O'Donnell C, Holloway R, Tilton E, et al. Infective endocarditis following Melody valve implantation: comparison with a surgical cohort. Cardiol Young. Mar 2017;27(2):294-301.
40. Ribeiro JM, Teixeira R, Lopes J, et al. Transcatheter Versus Surgical Pulmonary Valve Replacement: A Systemic Review and Meta-Analysis. Ann Thorac Surg. Nov 2020; 110(5): 1751-1761.
41. Stout KK, Daniels CJ, Aboulhosn JA, et al. 2018 AHA/ACC Guideline for the Management of Adults WithCongenital Heart Disease: Executive Summary: A Report of the American College of Cardiology/American HeartAssociation Task Force on Clinical Practice Guidelines. J Am Coll Cardiol. Apr 02 2019; 73(12): 1494-1563.
42. Van Dijck I, Budts W, Cools B, et al. Infective endocarditis of a transcatheter pulmonary valve in comparison with surgical implants. Heart. May 15 2015; 101(10):788-793.
43. Vezmar M, Chaturvedi R, Lee KJ et al. Percutaneous pulmonary valve implantation in the young 2-year follow-up. JACC Cardiovasc Interv 2010; 3(4):439-48.
44. Zahn EM, Hellenbrand WE, Lock JE et al. Implantation of the Melody transcatheter pulmonary valve in patients with a dysfunctional right ventricular outflow tract conduit. J Am Coll Cardiol 2009; 54(18):1722-9.

POLICY HISTORY:

Medical Policy Group (2): November 2011

Medical Policy Administration Committee, November 2011

Available for comment December 19, 2011 through February 1, 2012

Medical Policy Panel, November 2012

Medical Policy Group (2): November 2012 Medically necessary statement amended to include “when performed according to FDA-approved indications”.  Key Points, Key Words, and References updated to support policy statement change. 

Medical Policy Administration Committee, December 2012

Available for comments December 12, 2012 through January 26, 2013

Medical Policy Panel, November 2013

Medical Policy Group (4): Updated Key Points and References. No changes to the policy at this time.

Medical Policy Panel, November 2014

Medical Policy Group, November 2014 (3): Update to Description, Key Points, Approved Governing Bodies, and References. No change to policy statement.

Medical Policy Group, November 2015: 2016 Annual Coding Update. Added new CPT code 33477 to current coding.  Created a previous coding section and moved deleted CPT code 0262T from current coding to previous coding.

Medical Policy Panel, November 2015

Medical Policy Group, December 2015 (4): Updates to Description, Key Points, Approved Governing Bodies, and References.  No change to policy statement.

Medical Policy Panel, June 2016

Medical Policy Group, July 2016 (4): Updates to Key Points, Key Words and References. Updated policy statement, but no change to intent of statement.

Medical Policy Panel, May 2018

Medical Policy Group, May 2018 (4): Updates to Description, Policy, Key Points, Approved by Governing Bodies, and References. Updated policy statements to allow coverage per FDA indications of device.

Medical Policy Administration Committee: June 2018

Medical Policy Panel, June 2019

Medical Policy Group, July 2019 (4): Updates to Key Points.  No change to policy statements.

Medical Policy Panel, June 2020

Medical Policy Group, June 2020 (4): Updates to Key Points and Previous Coding.  Removed deleted code (12/31/15) 0262T from Previous Coding. Clarification made to Native policy statement by removing “conduit” from 1st bullet point.

Medical Policy Panel, June 2021

Medical Policy Group, June 2021 (4): Updates to Description, Key Points, Practice Guidelines, Governing Bodies and References. Policy statement updated to remove “not medically necessary,” no change to policy intent.

Medical Policy Panel, June 2022

Medical Policy Group, June 2022 (4): Updates to Key Points and References.  No change to policy statement.

Medical Policy Panel, June 2023

Medical Policy Group, June 2023 (4): Updates to Key Points, Benefit Application, and References. No change to policy statement.

Medical Policy Panel, June 2024

Medical Policy Group, June 2024 (4): Updates to Description, Key Points and References.  No change to policy statements.

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.