Skip to main content
HealthPartners

Coverage criteria policies

Genetic Testing for Arrhythmias and Cardiomyopathies

These services may or may not be covered by your HealthPartners plan. Please see your plan documents for your specific coverage information. If there is a difference between this general information and your plan documents, your plan documents will be used to determine your coverage.

Administrative Process

Prior authorization is required for genetic testing related to arrhythmias and cardiomyopathies

For pharmacogenetic testing, please see the Genetic Testing: Pharmacogenetics coverage policy.

Coverage

Indications that are covered

Single-gene and multiple-gene analysis for arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) is covered when criteria 1-4 listed below are met:

  1. The test is ordered by a board-certified cardiologist; electrophysiologist; or geneticist; a licensed, certified genetic counselor; or an advanced-practice nurse or physicians’ assistant in cardiology or genetics.
  2. The test is expected to directly impact management of ARVC/D.
  3. The member has received genetic counseling with a board-certified genetic counselor or medical geneticist who is not affiliated with the commercial testing laboratory, if applicable.
  4. The member has definite or possible ARVC/D according to the 2010 International Task Force (ITF) criteria (certification of this must be present in the clinical notes) or the member has a first- or second-degree relative diagnosed with ARVC/D.

Single-gene and multiple-gene analysis for Brugada syndrome (BrS) is covered when criteria 1-4 listed below are met:

  1. The test is ordered by a board-certified cardiologist; electrophysiologist; or geneticist; a licensed, certified genetic counselor; or an advanced-practice nurse or physicians’ assistant in cardiology or genetics.
  2. The test is expected to directly impact management of BrS.
  3. The member has received genetic counseling with a board-certified genetic counselor or medical geneticist who is not affiliated with the commercial testing laboratory, if applicable.
  4. The member has any of the following:
    1. A type 1 Brugada syndrome electrocardiographic phenotype (resting 12-lead ECGs and/or provocative drug challenge testing
    2. A first- or second-degree relative with sudden death suspected to be related to BrS
    3. A first- or second-degree relative diagnosed with BrS

Single-gene and multiple-gene analysis for catecholeminergic polymorphic ventricular tachycardia (CPVT) is covered when criteria 1-4 listed below are met:

  1. The test is ordered by a board-certified cardiologist; electrophysiologist; or geneticist; a licensed, certified genetic counselor; or an advanced-practice nurse or physicians’ assistant in cardiology or genetics.
  2. The test is expected to directly impact management of CPVT.
  3. The member has received genetic counseling with a board-certified genetic counselor or medical geneticist who is not affiliated with the commercial testing laboratory, if applicable.
  4. The member has any of the following:
    1. Suspected CPVT after provocative stress testing with exercise or catecholamine infusion
    2. A first- or second-degree relative with sudden death suspected to be related to CPVT
    3. A first- or second-degree relative diagnosed with CPVT

Single-gene and multiple-gene analysis for dilated cardiomyopathy (DCM) is covered when criteria 1-4 listed below are met:

  1. The test is ordered by a board-certified cardiologist; electrophysiologist; or geneticist; a licensed, certified genetic counselor; or an advanced-practice nurse or physicians’ assistant in cardiology or genetics.
  2. The test is expected to directly impact management of DCM.
  3. The member has received genetic counseling with a board-certified genetic counselor or medical geneticist who is not affiliated with the commercial testing laboratory, if applicable.
  4. The member has any of the following:
    1. A clinical diagnosis of DCM and significant cardiac conduction disorder, and the member is a candidate for an implantable or wearable cardioverter defibrillator
    2. A clinical diagnosis of DCM and first- or second-degree relative with sudden death of suspected cardiac etiology at age 50 or younger
    3. A first- or second-degree relative diagnosed with DCM

Single-gene and multiple-gene analysis for hypertrophic cardiomyopathy (HCM) is covered when criteria 1-4 listed below are met:

  1. The test is ordered by a board-certified cardiologist; electrophysiologist; or geneticist; a licensed, certified genetic counselor; or an advanced-practice nurse or physicians’ assistant in cardiology or genetics.
  2. The test is expected to directly impact management of HCM.
  3. The member has received genetic counseling with a board-certified genetic counselor or medical geneticist who is not affiliated with the commercial testing laboratory, if applicable.
  4. The member has a clinical diagnosis of HCM or the member has a first- or second-degree relative diagnosed with HCM.

Single-gene and multiple-gene analysis for left ventricular non-compaction (LVNC) is covered when criteria 1-4 listed below are met:

  1. The test is ordered by a board-certified cardiologist; electrophysiologist; or geneticist; a licensed, certified genetic counselor; or an advanced-practice nurse or physicians’ assistant in cardiology or genetics.
  2. The test is expected to directly impact management of LVNC.
  3. The member has received genetic counseling with a board-certified genetic counselor or medical geneticist who is not affiliated with the commercial testing laboratory, if applicable.
  4. The member has suspected LVNC based on clinical and family history and electrocardiographic/echocardiographic phenotype or the member has a first- or second-degree relative diagnosed with LVNC.

Single-gene and multiple-gene analysis for long QT syndrome (LQTS) is covered when criteria 1-4 listed below are met:

  1. The test is ordered by a board-certified cardiologist; electrophysiologist; or geneticist; a licensed, certified genetic counselor; or an advanced-practice nurse or physicians’ assistant in cardiology or genetics.
  2. The test is expected to directly impact management of LQTS.
  3. The member has received genetic counseling with a board-certified genetic counselor or medical geneticist who is not affiliated with the commercial testing laboratory, if applicable.
  4. The member has any of the following:
    1. A confirmed prolonged QT interval on resting electrocardiogram (ECG) or Holter monitor and acquired/external causes have been ruled out.
    2. A first-degree relative with a history of a prolonged QT interval on ECG or Holter monitor and acquired/external causes were ruled out.
    3. A first- or second- degree relative with sudden death from suspected LQTS at age 40 or younger
    4. A first- or second-degree relative diagnosed with LQTS

Single-gene and multiple-gene analysis for restrictive cardiomyopathy (RCM) is covered when criteria 1-4 listed below are met:

  1. The test is ordered by a board-certified cardiologist; electrophysiologist; or geneticist; a licensed, certified genetic counselor; or an advanced-practice nurse or physicians’ assistant in cardiology or genetics.
  2. The test is expected to directly impact management of RCM.
  3. The member has received genetic counseling with a board-certified genetic counselor or medical geneticist who is not affiliated with the commercial testing laboratory, if applicable.
  4. The member has suspected RCM based on electrocardiographic and/or echocardiographic phenotype or the member has a first- or second-degree relative diagnosed with RCM.

Single-gene and multiple-gene analysis for short QT syndrome (SQTS) is covered when criteria 1-4 listed below are met:

  1. The test is ordered by a board-certified cardiologist; electrophysiologist; or geneticist; a licensed, certified genetic counselor; or an advanced-practice nurse or physicians’ assistant in cardiology or genetics.
  2. The test is expected to directly impact management of SQTS.
  3. The member has received genetic counseling with a board-certified genetic counselor or medical geneticist who is not affiliated with the commercial testing laboratory, if applicable.
  4. The member has any of the following:
    1. Suspected diagnosis of SQTS and an electrophysiologist has recommended genetic testing.
    2. A first- or second-degree relative diagnosed with SQTS

Genetic testing for arrhythmias and cardiomyopathies other than those listed above is subject to a review for medical necessity, based on current clinical literature and expert recommendations, unless listed below as an indication that is not covered.

Indications that are not covered

  1. Genetic testing for arrhythmias and cardiomyopathies is not covered and is considered not medically necessary when test results will not directly impact the treatment or management of a condition because the testing is not expected to restore or maintain the member’s health, prevent deterioration of the member’s condition, nor prevent the reasonably likely onset of a health problem or detect an incipient problem.
  2. Repeat testing of a unique analyte using the identical method of analysis is not covered and is considered not medically necessary because it is not considered an appropriate frequency of care.
  3. Multiple-gene panels which include genes not associated with the specific condition under evaluation, and panels which include evaluation for conditions not within the member’s differential diagnosis, are not covered and are considered not medically necessary because they are not considered an appropriate type of service for the member’s condition.
  4. Single-gene and multiple-gene assays for the following conditions are considered experimental/investigational, because reliable evidence does not permit conclusions concerning safety, effectiveness, or effect on health outcomes:
  • Atrial fibrillation, including SNP genotyping and miRNA microarray
  • Brugada syndrome presenting with a Brugada type 2 or type 3 ECG pattern

Definitions

Arrhythmia is an abnormal heart rhythm or any change from the normal sequence of electrical impulses.

Cardiomyopathy is disease of the heart muscle.

First-degree relative is an individual’s parent, sibling, or child

Genetic refers to inherited traits or disorders, or those that result from acquired changes in genetic makeup.

Genetic testing involves analysis of human genetic material (such as DNA or chromosomes) to identify whether a person has a particular inherited trait or disorder, or an acquired genetic change, or whether he or she carries a gene that could lead to a specific disease or condition.

Multiple-gene panels (often referred to as next-generation sequencing panels) analyze several genes simultaneously.

Second-degree relative is an individual’s grandparent, grandchild, aunt, uncle, nephew, niece, or half-sibling.

Single-gene analysis analyzes a single, unique gene.

Third-degree relative is an individual’s first cousin, great-grandparent, great-grandchild, great-aunt, great-uncle, grandniece, grandnephew, half-aunt, half-uncle, half-nephew, or half-niece.

If available, codes are listed below for informational purposes only, and do not guarantee member coverage or provider reimbursement. The list may not be all-inclusive.

Code

Description

S3861

Genetic testing, sodium channel, voltage-gated, type V, alpha subunit (SCN5A) and variants for suspected Brugada Syndrome

S3865

Comprehensive gene sequence analysis for hypertrophic cardiomyopathy

S3866

Genetic analysis for a specific gene mutation for hypertrophic cardiomyopathy (HCM) in an individual with a known HCM mutation in the family

81413

Cardiac ion channelopathies (eg, Brugada syndrome, long QT syndrome, short QT syndrome, catecholaminergic polymorphic ventricular tachycardia); genomic sequence analysis panel, must include sequencing of at least 10 genes, including ANK2, CASQ2, CAV3, KCNE1, KCNE2, KCNH2, KCNJ2, KCNQ1, RYR2, and SCN5A

81414

Cardiac ion channelopathies (eg, Brugada syndrome, long QT syndrome, short QT syndrome, catecholaminergic polymorphic ventricular tachycardia); duplication/deletion gene analysis panel, must include analysis of at least 2 genes, including KCNH2 and KCNQ1

81439

Hereditary cardiomyopathy (eg, hypertrophic cardiomyopathy, dilated cardiomyopathy, arrhythmogenic right ventricular cardiomyopathy) genomic sequence analysis panel, must include sequencing of at least 5 genes (eg. DSG2, MYBPC3, MYH7, PKP2, TTN)

CPT Copyright American Medical Association. All rights reserved. CPT is a registered trademark of the American Medical Association

Products

This information is for most, but not all, HealthPartners plans. Please read your plan documents to see if your plan has limits or will not cover some items. If there is a difference between this general information and your plan documents, your plan documents will be used to determine your coverage. These coverage criteria may not apply to Medicare Products if Medicare requires different coverage. For more information regarding Medicare coverage criteria or for a copy of a Medicare coverage policy, contact Member Services at 952-883-7979 or 1-800-233-9645.

References

  1. Ackerman, M. J., Priori, S. G., Willems, S., Berul, C., Brugada, R., Calkins, H., … Zipes, D. P. (2011). HRS/EHRA consensus statement on the state of genetic testing for the channelopathies and cardiomyopathies. Heart Rhythm, 8, 1308-1339.
  2. Antzelevitch, C., & Cordeiro, J. M. (2016). Short QT syndrome. In: M. S. Link & B. C. Downey (Eds.). UpToDate. Waltham, MA: UpToDate.
  3. Arbelo, E., & Brugada, J. (2014). Risk stratification and treatment of Brugada syndrome. Current Cardiology Reports, 16, 508-517.
  4. Arscott, P., Caleshu, C., Kotzer, K., Kreykes, S., Kruisselbrink, T., Orland, K., . . . Cherny, S. (2016). A case for inclusion of genetic counselors in cardiac care. Cardiology Reviews, 24, 49-55.
  5. Connolly, H. M., & Attenhofer-Jost, C. H. (2016). Isolated left ventricular noncompaction. In: W. J. McKenna & S. B. Yeon (Eds.). UpToDate. Waltham, MA: UpToDate.
  6. D’Argenio, V., Frisso, G., Precone, V., Boccia, A., Fienga, A., Pacileo, G., . . . Salvatore, F. (2014). DNA sequence capture and next-generation sequencing for the molecular diagnosis of genetic cardiomyopathies. Journal of Molecular Diagnostics, 16, 32-44.
  7. Gersh, B. J., Maron, B. J., Bonow, R. O., Dearani, J. A., Fifer, M. A., Link, M. S., . . . Yancy, C. W. (2011). 2011 ACCF/AHA guideline for the diagnosis and treatment of hypertrophic cardiomyopathy: A report of the American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines. Journal of the American College of Cardiology, 58, e212-e260.
  8. Glotov, A. S., Kazakov, S. V., Zhukova, E. A., Alexandrov, A. V., Glotov, O. S., Pakin, V. S., . . . Baranov, V. S. (2015). Targeted next-generation sequencing (NGS) of nine candidate genes with custom AmpliSeq in patients and a cardiomyopathy risk group. Clinica Chimica Acta, 446, 132-140.
  9. Golbus, J. R., Puckelwartz, M. J., Dellefave-Castillo, L., Fahrenbach, J. P., Nelakudti, V., Pesce, L. L., . . . McNally, E. M. (2014). Targeted analysis of whole genome sequence data to diagnose genetic cardiomyopathy. Circulation: Cardiovascular Genetics, 7, 751-759.
  10. Gollob, M. H., Redpath, C. J., & Roberts, J. D. (2011). The short QT syndrome: Proposed diagnostic criteria. Journal of the American College of Cardiology, 57, 802-812.
  11. Groeneweg, J. A., Bhonsale, A., James, C. A., te Riele, A. S., Dooijes, D., Tichnell, C., . . . Calkins, H. (2015). Clinical presentation, long-tern follow-up, and outcomes of 1001 arrhythmogenic right ventricular dysplasia/cardiomyopathy patients and family members. Circulation: Cardiovascular Genetics, 8, 437-446.
  12. Groeneweg, J. A., van der Zwaag, P. A., Olde Nordkamp, L. R., Bikker, H., Jongbloed, J. D., Wiesfeld, A. C., . . . Hauer, R. N. (2013). Arrythmogenic right ventricular dysplasia/cardiomyopathy according to revised 2010 task force criteria with inclusion of non-desmosomal phospholamban mutation carriers. American Journal of Cardiology, 112, 1197-1206.
  13. Heart Failure Society of America. (2010). HFSA 2010 comprehensive heart failure practice guideline. Journal of Cardiac Failure, 16, 475-539.
  14. Hershberger, R. E. (2016). Familial dilated cardiomyopathy: Prevalence, diagnosis, and treatment. In: W. J. McKenna, B. A. Raby, & S. B. Yeon (Eds.). UpToDate. Location: Waltham, MA.
  15. Hershberger, R. E., & Siegfried, J. D. (2011). Update 2011: Clinical and genetic issues in familial dilated cardiomyopathy. Journal of the American College of Cardiology, 57, 1641-1649.
  16. Hershberger, R. E., Lindenfeld, J., Mestroni, L., Seidman, C. E., Taylor, M. R., & Towbin, J. A. (2009). Genetic evaluation of cardiomyopathy: A Heart Failure Society of America practice guideline. Journal of Cardiac Failure, 15, 83-97.
  17. Hu, D., Barajas-Martinez, H., Pfeiffer, R., Dezi, F., Pfeiffer, J., Buch, T., . . . Antzelevitch, C. (2014). Mutations in SCN10A are responsible for a large fraction of cases of Brugada syndrome. Journal of the American College of Cardiology, 64, 66-79.
  18. Le Scouarnec, S., Karakachoff, M., Gourraud, J. B., Lindenbaum, P., Bonnaud, S., Porero, V., . . . Redon, R. (2015). Testing the burden of rare variation in arrhythmia-susceptibility genes provides new insights into molecular diagnosis for Brugada syndrome. Human Molecular Genetics, 24, 2757-2763.
  19. Li, Q., Gruner, C., Chan, R. H., Care, M., Siminovitch, K., Williams, L., . . . Rakowski, H. (2014). Genotype-positive status in patients with hypertrophic cardiomyopathy is associated with higher rates of heart failure events. Circulation: Cardiovascular Genetics, 7, 416-422.
  20. Marcus, F. I., McKenna, W. J., Sherrill, D., Basso, C., Bauce, B., Bluemke, D. A., . . . Zareba, W. (2010). Diagnosis of arrhythmogenic right ventricular cardiomyopathy/dysplasia: Proposed modification of the task force criteria. Circulation, 121, 1533-1541.
  21. Maron, M. S. (2016). Hypertrophic cardiomyopathy: Gene mutations and clinical genetic testing. In: B. A. Raby, W. J. McKenna, & B. C. Downey (Eds.). UpToDate. Waltham, MA: UpToDate.
  22. Maron, M. S. (2017). Hypertrophic cardiomyopathy: Clinical manifestations, diagnosis, and evaluation. In: W. J. McKenna, & B. C. Downey (Eds.). UpToDate. Waltham, MA: UpToDate.
  23. McKenna, W. J. (2016). Arrhythmogenic right ventricular cardiomyopathy: Diagnostic evaluation and diagnosis. In: H. Calkins & B. C. Downey (Eds.). UpToDate. Waltham, MA: UpToDate.
  24. Napolitano, C., Novelli, V., Francis, M. D., & Priori, S. G. (2015). Genetic modulators of the phenotype in the long QT syndrome: State of the art and clinical impact. Current Opinion in Genetics and Development, 33, 17-24.
  25. Oliveira, T. G., Mitne-Neto, M., Cerdeira, L. T., Marsiglia, J. D., Arteaga-Fernandez, E., Krieger, J. E., & Pereira, A. C. (2015). A variant detection pipeline for inherited cardiomyopathy-associated genes using next-generation sequencing. Journal of Molecular Diagnostics, 17, 420-430.
  26. Philips, B., & Cheng, A. (2016). 2015 update on the diagnosis and management of arrhythmogenic right ventricular cardiomyopathy. Current Opinion in Cardiology, 31, 46-56.
  27. Priori, S. G., Wilde, A. A., Horie, M., Cho, Y., Behr, E. R., Berul, C., . . . Tracy, C. (2013). HRS/EHRA/APHRS expert consensus statement on the diagnosis and management of patients with inherited primary arrhythmia syndromes. Heart Rhythm, 10, 1932-1963.
  28. Pugh, T. J., Kelly, M. A., Gowrisankar, S., Hynes, E., Seidman, M. A., Baxter, S. M., . . . Funke, B. H. (2014). The landscape of genetic variation in dilated cardiomyopathy as surveyed by clinical DNA sequencing. Genetics in Medicine, 16, 601-608.
  29. Riuro, H., Campuzano, O., Arbelo, E., Iglesias, A., Batlle, M., Perez-Villa, F., . . . Brugada, R. (2014). A missense mutation in the sodium channel β1b subunit reveals SCN1B as a susceptibility gene underlying long QT syndrome. Heart Rhythm, 11, 1202-1209.
  30. Roux-Buisson, N., Gandjbakhch, E., Donal, E., Probst, V., Deharo, J. C., Chevalier, P., . . . Charron, P. (2014). Prevalence and significance of rare RYR2 variants in arrhythmogenic right ventricular cardiomyopathy/dysplasia: results of a systematic screening. Heart Rhythm, 11, 1999-2009.
  31. Sarquella-Brugada, G., Campuzano, O., Arbelo, E., Brugada, J., & Brugada, R. (2016). Brugada syndrome: Clinical and genetic findings. Genetics in Medicine, 18, 3-12.
  32. Seslar, S. P., Ziimetbaum, P. J., & Berul, C. I. (2014). Diagnosis of congenital long QT syndrome. In: J. K. Triedman, S. Asirvatham, & B. C. Downey (Eds.). UpToDate. Waltham, MA: UpToDate.
  33. Stacey, R. B., Caine, A. J., & Hundley, W. G. (2015). Evaluation and management of left ventricular noncompaction cardiomyopathy. Current Heart Failure Reports, 12, 61-67.
  34. Van Driest, S. L., Wells, Q. S., Stallings, S., Bush, W. S., Gordon, A., Nickerson, D. A., . . . Roden, D. M. (2016). Association of arrhythmia-related genetic variants with phenotypes documented in electronic medical records. JAMA, 315, 47-57.
  35. Wu, W., Lu, C. X., Wang, Y. N., Liu, F., Chen, W., Liu, Y. T., . . . Zhang, X. (2015). Novel phenotype-genotype correlations of restrictive cardiomyopathy with myosin-binding protein C (MYBPC3) gene mutations tested by next-generation sequencing. Journal of the American Heart Association, 4, 1-7.
  36. Wylie, J. V., & Garlitski, A. C. (2017). Brugada syndrome: Clinical presentation, diagnosis, and evaluation. In: S. Manaker, S. Asirvatham, & B. C. Downey (Eds.). UpToDate. Waltham, MA: UpToDate.
  37. Zimetbaum, P. J. (2016). Genetics of congenital and acquired long QT syndrome. In: J. K. Triedman, S. Asirvatham, & B. C. Downey (Eds.). UpToDate. Waltham, MA: UpToDate.

Go to

Policy activity

  • 06/21/2016 - Date of origin
  • 09/01/2017 - Effective date
Review date
  • 06/2017
Revision date
  • 09/01/2017

Related content