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    v1.0.28
    OpenPayerPolicy UpdatesCardiologyAmbetter Nevada Cardiovascular Genetic Policy Upda…
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    ModifiedAmbetter NevadaPolicy N/A

    Concert Genetic Testing: Cardiovascular

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    Defines medical necessity and coding guidance for genetic testing (multigene panels and targeted tests) for inherited and sporadic cardiovascular disorders; applies to providers submitting claims for Ambetter Nevada members.

    Policy Summary
    PayerAmbetter Nevada
    PolicyConcert Genetic Testing: Cardiovascular (cardiology — coverage criteria)
    Policy CodePolicy N/A
    Change TypeCriteria revisions and coding updates
    Effective DateN/A
    Next Review DateN/A
    Key ActionObtain prior authorization and document that the member meets the specific numeric and clinical criteria for the selected cardiac genetic panel.
    SourceLink
    POLICY UPDATE CHANGES

    Replaced 'coverage criteria' language with 'criteria' throughout the document and updated multiple panel-specific criteria and wording (e.g., LQTS, cardiomyopathy, Brugada, CPVT, FH, transplant panels).

    For Short QT Syndrome, added explicit SQTS diagnostic threshold (QTc ≤ 330 ms) and recommendation for genetic testing when diagnostic criteria or SQTS diagnostic score ≥ 4 are met.

    For Brugada syndrome, clarified diagnostic ECG pattern language (≥ 2 mm J-wave elevation) and emphasized exclusion of phenocopies and limited gene reporting to validated genes (primarily SCN5A).

    Policy title updated from 'Concert Genetic Testing: Cardiac Disorders' to 'Concert Genetic Testing: Cardiovascular' and numerous criteria, references, and formatting updates were made across cardiology panel sections.

    Brugada, LQTS, CPVT, ARVC/arrhythmogenic cardiomyopathy, HCM, DCM, SQTS panels had multiple clinical criteria and wording edits (including diagnostic thresholds and addition/removal of certain criteria).

    Known Familial Variant Analysis criteria for cardiac disorders were moved out of this policy into the 'Genetic Testing: General Approach to Genetic and Molecular Testing' policy.

    manypanels with medical necessity criteria
    >=15 mmHCM LV wall threshold (adults)
    <=330 msSQTS diagnostic QTc cutoff
    includedfamily-history-based indications
    81439,81413CPT examples referenced
    Wilde et al. 2022guideline/reference updates

    Coverage Criteria for Cardiovascular Genetic Testing

    Comprehensive Cardiomyopathy Panels

    Covered when ANY of the following are met:

    Cardiomyopathy diagnosis or family SCD/SUD: The member/enrollee has a diagnosis of cardiomyopathy; OR the member/enrollee has a first-degree relative with sudden cardiac death (SCD) or sudden unexplained death (SUD) AND (1) the relative's autopsy revealed unspecified cardiomyopathy (e.g., cardiomegaly or cardiomyopathy) OR (2) the relative's autopsy revealed an anatomically normal heart AND the autopsy did not reveal a cause of death.

    Multigene panels targeted to the cardiomyopathy phenotype observed are recommended by professional guidelines.

    Comprehensive Arrhythmia Panels

    Covered when ANY of the following are met:

    Family history of SCD or unexplained SCA: The member/enrollee has a first-degree relative with sudden cardiac death (SCD) or sudden unexplained death (SUD) before age 50, OR has a first-degree relative with SCD at age 50 or older AND (a) the deceased had family history of premature SCD OR (b) the death is suspicious for genetic heart disease; OR the member/enrollee has unexplained sudden cardiac arrest AND clinical tests were non-diagnostic for reversible, ischemic, or structural causes (e.g., ECG, cardiac stress tests, echocardiogram, intravenous pharmacologic provocation testing).
    Policy Summary
    PayerAmbetter Nevada
    PolicyConcert Genetic Testing: Cardiovascular (cardiology — coverage criteria)
    Policy CodePolicy N/A
    Change TypeCriteria revisions and coding updates
    Effective DateN/A
    Next Review DateN/A
    Key ActionObtain prior authorization and document that the member meets the specific numeric and clinical criteria for the selected cardiac genetic panel.
    SourceLink
    On This Page
    Coverage Criteria for Cardiovascular Genetic TestingCoding and Test IdentifiersProvider Actions, Documentation, and Prior AuthorizationBackground and ScopeDefinitions and Key Terms

    Comprehensive Arrhythmia and Cardiomyopathy (Sudden Cardiac or Unexplained Death) Panels

    Covered when ALL of the following are met:

    Combined panel criteria: The member/enrollee meets clinical criteria for Comprehensive Cardiomyopathy Panels AND meets clinical criteria for Comprehensive Arrhythmia Panels.

    Comprehensive panels include genes for both cardiomyopathies and arrhythmias; use appropriate panel code if performed.

    Hypertrophic Cardiomyopathy Panels

    Covered when ALL of the following are met:

    Unexplained LVH threshold: Member/enrollee has unexplained left ventricular hypertrophy (LVH) defined as myocardial wall thickness ≥ 15 mm in adults or z-score ≥ 2 in children on echocardiogram or cardiac MRI.LV wall thickness >=15 mm (adults) or z-score >=2 (children)

    If a panel is performed, the appropriate panel code should be used.

    Comprehensive combined panels

    Comprehensive Arrhythmia and Cardiomyopathy (Sudden Cardiac or Unexplained Death) Panels are covered when ALL of the following are met

    Comprehensive panels: Panel includes genes for both cardiomyopathies and arrhythmias and is considered medically necessary when: (A) the member meets clinical criteria for Comprehensive Cardiomyopathy Panels, AND (B) the member meets clinical criteria for Comprehensive Arrhythmia Panels.

    Current evidence does not support combined panels for other indications.

    Hypertrophic cardiomyopathy

    Hypertrophic Cardiomyopathy Panel covered when ALL of the following are met

    HCM panel: Member/enrollee has unexplained left ventricular hypertrophy (LVH) defined as myocardial wall thickness ≥15 mm in adults or z-score ≥2 in children on echocardiogram or cardiac MRI.LV wall thickness >=15mm or z-score >=2

    If a panel is performed, the appropriate panel code should be used.

    Dilated cardiomyopathy

    Dilated Cardiomyopathy Panel covered when ALL of the following are met

    DCM panel: Member/enrollee has left ventricular enlargement or biventricular dilatation AND systolic dysfunction (e.g., ejection fraction <50%) AND non-genetic causes of DCM have been ruled out (such as prior MI from CAD, valvular or congenital heart disease, toxins, thyroid disease, inflammatory/infectious causes, severe longstanding hypertension, radiation).EF <50%

    If a panel is performed, the appropriate panel code should be used.

    Arrhythmogenic cardiomyopathy (ARVC)

    Arrhythmogenic Cardiomyopathy Panel covered when ANY of the major criteria are met, OR ANY TWO of the minor criteria are met

    Major criteria: Member/enrollee has any one major Task Force criterion from imaging (echo or MRI), RV angiography, endomyocardial biopsy, ECG features (e.g., inverted T waves V1–V3, epsilon wave, characteristic VT), or family history consistent with arrhythmogenic cardiomyopathy.Major imaging/ECG/biopsy thresholds as specified in policy (e.g., Rao RVEDV/BSA >110 mL/m2 male, RVEF <40% for major)

    If panel performed, use appropriate panel code.

    Minor criteria (two required): Member/enrollee has any two minor criteria including less-severe imaging/ECG abnormalities, borderline biopsy findings, arrhythmic findings (e.g., >500 ventricular extrasystoles per 24 hours), or specified family history items.Minor ranges for RVOT, RVEDV/BSA, RVEF, residual myocytes, Holter PVC counts, etc.

    If panel performed, use appropriate panel code.

    Restrictive cardiomyopathy

    Restrictive cardiomyopathy: Current evidence does not support genetic testing for restrictive cardiomyopathy (RCM) via multigene panel for any indication.

    No covered indications provided.

    Long QT syndrome

    Long QT Syndrome Panel covered when EITHER A or B is met

    Asymptomatic with prolonged QTc or family history: Member/enrollee is asymptomatic AND either has confirmed prolonged QTc (>460 ms prepuberty; >480 ms adults) on resting ECG/provocative testing, OR has a close relative with clinical LQTS whose genetic status is unknown.>460 ms prepuberty; >480 ms adults

    If a pathogenic variant is known in an affected relative, follow familial variant criteria.

    Symptomatic: Member/enrollee is symptomatic (e.g., syncope, cardiac arrest, aborted sudden death) AND (a cardiologist has strong clinical suspicion for LQTS based on history/family/ECG phenotype OR Schwartz score ≥3.0) AND non-genetic causes of prolonged QTc have been ruled out.Schwartz score >=3.0

    If a panel is performed, appropriate panel code should be used.

    Short QT syndrome

    Short QT Syndrome Panel covered when ANY of the following are met

    QTc threshold: Member/enrollee has QTc ≤ 330 ms.<=330 ms

    ECG must be recorded without modifiers known to shorten QT; if pathogenic familial variant known, refer to familial testing policy.

    SQTS diagnostic score: Member/enrollee has SQTS diagnostic score ≥ 4 using the specified scoring (ECG QTc points, J-point–T-peak, clinical history, family history, genotype), with at least 1 point from the ECG section.score >=4

    Points and scoring per policy supplementary table.

    Familial indication: Member/enrollee is asymptomatic AND has a first-degree relative with clinical SQTS whose genetic status is unknown.

    If pathogenic variant known in affected relative, follow Known Familial Variant Analysis criteria.

    Brugada syndrome (SCN5A)

    Brugada syndrome (SCN5A): SCN5A variant analysis is medically necessary when the member/enrollee meets Brugada ECG criteria (Type 1, 2, or 3 with J-wave elevation ≥2 mm in right precordial leads, with or without sodium-channel blocker challenge), phenocopies (e.g., ischemia, electrolyte disturbance, drug intoxication) have been excluded, AND the member has recurrent syncope, ventricular fibrillation, self-terminating polymorphic VT, cardiac arrest, or a family history of sudden cardiac death.J-wave elevation >=2 mm

    Genetic testing via genes other than SCN5A, including multigene panels, is not supported.

    CPVT

    Catecholaminergic Polymorphic VT Panel covered when ALL of the following are met

    CPVT panel: No known structural cardiac abnormalities AND the member/enrollee has one of: syncope during physical activity or acute emotion; exercise- or emotion-related palpitations/dizziness; sudden unexpected cardiac death triggered by exercise/emotion; family history of juvenile SCD triggered by exercise/emotion; exercise-induced bidirectional or polymorphic VT; or ventricular fibrillation occurring with acute stress.

    If panel is performed, use appropriate panel code.

    Familial hypercholesterolemia

    Familial Hypercholesterolemia Panel covered when ALL of the following are met

    FH panel: Member/enrollee has ≥2 elevated LDL-C measurements (including assessment after intensive lifestyle modification) AND no apparent secondary cause of hypercholesterolemia AND meets age-specific LDL-C thresholds or family-history criteria: children ≥190 mg/dL, or children ≥160 mg/dL with at least one affected first-degree relative or premature CAD or limited family history; adults ≥250 mg/dL; or adults ≥190 mg/dL with at least one affected first-degree relative or premature CAD.LDL-C numeric thresholds as listed

    Testing should include at minimum LDLR, APOB, PCSK9; consider expanded lipid panels where appropriate.

    Congenital heart malformations

    Congenital Heart Malformation Panel may be considered when ALL of the following are met

    Congenital heart malformations: Member/enrollee has a complex congenital heart malformation (e.g., hypoplastic left heart, transposition of the great vessels, tetralogy of Fallot) AND clinical features do not fit a known genetic disorder for which targeted testing is available AND prenatal teratogen exposure has been considered/ruled out.

    Not supported for simple defects; if panel performed, use appropriate panel code.

    Familial TAAD

    Familial Thoracic Aortic Aneurysm and Dissection (TAAD) Panel covered when ALL of the following are met

    TAAD panel: Member/enrollee has history of aortic root enlargement, thoracic aneurysm, or type A/B aortic dissection AND does not meet diagnostic criteria for another connective tissue disorder AND has family history of dilation or dissection of the aortic root consistent with autosomal dominant inheritance.

    A multigene panel comprising HTAD-associated genes is the recommended approach.

    Hereditary hemorrhagic telangiectasia

    Hereditary Hemorrhagic Telangiectasia Panel covered when ALL of the following are met

    HHT panel: Member/enrollee has clinical features of HHT (spontaneous recurrent epistaxis OR mucocutaneous telangiectases at characteristic sites OR visceral arteriovenous malformation) AND the panel includes at minimum ACVRL1 and ENG genes.

    If ENG and ACVRL1 are negative, consider SMAD4 testing; use appropriate panel code if performed.

    HHT multigene panel — medically necessary

    HHT multigene panel is covered when ALL of the following are met:

    HHT panel criteria: (A) Member/enrollee has at least one clinical feature: spontaneous recurrent epistaxis OR mucocutaneous telangiectases at characteristic sites OR visceral arteriovenous malformation (pulmonary, cerebral, spinal, gastrointestinal, or pancreatic); AND (B) the panel includes at minimum ACVRL1 and ENG.

    From policy section on HHT panels. If ENG/ACVRL1 negative, consider SMAD4.

    Unfocused genetic testing — not routine

    Unfocused testing: Genetic testing using large unfocused panels, whole-exome sequencing, or whole-genome sequencing in the absence of a clinical phenotype is not recommended for routine patient care and counseling.

    May be considered only in research or scientific contexts.

    Long QT syndrome — testing criteria

    LQTS testing indications: Molecular testing for definitive LQTS-associated genes (e.g., KCNQ1, KCNH2, SCN5A, CALM1-3) should be offered to index patients with high probability of LQTS (Schwartz score ≥3.5). Testing of established genes may be considered in intermediate-probability cases; genes with limited/disputed evidence should not be tested in intermediate cases.Schwartz score thresholds

    Per EHRA/HRS/APHRS expert consensus.

    Short QT syndrome — testing criteria

    SQTS testing indications: In patients meeting diagnostic criteria for SQTS (QTc ≤330 ms or SQTS diagnostic score ≥4), molecular testing of definitive genes (currently KCNH2, KCNQ1) is recommended; testing of KCNJ2 and SLC4A3 may be considered when high probability is established.QT <=330 ms or diagnostic score >=4

    Cascade testing for at-risk family members recommended when a pathogenic mutation is identified.

    Cardiomyopathy and arrhythmia panels — indications

    Genetic testing for cardiomyopathies

    HCM testing: Genetic counseling and testing are cornerstones of HCM care; first-degree relative screening can begin at any age and pathogenicity should be reconfirmed every 2–3 years. Imaging diagnostic criteria include maximal LV wall thickness ≥15 mm in adults (13–14 mm may be diagnostic in familial cases or with positive genetic test).LV wall thickness thresholds

    ACC/AHA guidance.

    DCM testing: Genetic testing is useful in DCM patients and recommended particularly in those with high yield; consider testing even without family history. DCM is defined by LV or biventricular dilatation and systolic dysfunction (EF <50%).EF <50%

    EHRA/HRS and Heart Failure Society guidance.

    RCM testing: For restrictive cardiomyopathy, consider HCM or DCM panels and differentiate TTR-related amyloidosis due to management implications; evidence for standalone RCM panels is limited.

    ACMG recommendations.

    ARVC testing: Task Force diagnostic major/minor criteria guide clinical diagnosis; identification of a pathogenic mutation supports diagnosis and cascade testing of relatives.

    ARVC Task Force criteria summarized in policy.

    Short QT Syndrome — Covered when ALL of the following are met

    Short QT syndrome (SQTS) — genetic testing recommended when diagnostic criteria met

    SQTS testing criteria: Testing is recommended for definitive disease-associated genes (currently KCNH2, KCNQ1) when a patient satisfies diagnostic criteria for SQTS (e.g., QTc ≤330 ms) or has an SQTS diagnostic score ≥4.SQTS diagnostic score >=4; QTc <= 330 ms diagnostic cutoff

    Testing of KCNJ2 and SLC4A3 may be considered; cascade testing recommended for at-risk relatives.

    Brugada Syndrome — Covered when ALL of the following are met

    Brugada syndrome — diagnostic and testing guidance

    Brugada diagnostic requirement: Suspect Brugada syndrome when clinical features (recurrent syncope, VF, polymorphic VT, cardiac arrest, family history of SCD) are present AND a diagnostic ECG pattern (Type 1–3) is observed with J-wave elevation ≥2 mm in right precordial leads; phenocopies (e.g., myocardial ischemia, electrolyte disturbances, drug intoxications) must be excluded. Genetic testing should focus on SCN5A; other genes have disputed validity and generally should not be reported diagnostically.J-wave elevation >=2 mm

    Use SCN5A variant analysis when clinically indicated.

    Familial Hypercholesterolemia — Covered when ANY of the following are met

    Familial hypercholesterolemia (FH) — genetic testing indications

    FH testing indications: Offer genetic testing when clinical index of suspicion is strong, including: (1) children with persistent LDL-C ≥160 mg/dL (with family history criteria) or children ≥190 mg/dL; (2) adults with persistent LDL-C ≥250 mg/dL; (3) adults with persistent LDL-C ≥190 mg/dL plus at least one affected first-degree relative or premature CAD; or (4) adults without pre-treatment LDL-C but with personal premature CAD and family history of hypercholesterolemia/premature CAD. Two or more LDL-C measurements (including after lifestyle modification) are expected.LDL-C thresholds as above

    Testing should include at minimum LDLR, APOB, PCSK9; consider expanded panels for phenocopies.

    TAAD — Covered when ALL of the following are met

    TAAD — Covered when ALL of the following are met

    TAAD testing recommendation: Genetic testing is recommended for patients with aortic root/ascending aortic aneurysms or dissection and risk factors for hereditary thoracic aortic disease (e.g., syndromic features, presentation <60 years, family history of TAD or peripheral/intracranial aneurysms, unexplained sudden death in a relative).

    A multigene panel including HTAD-associated genes is the most cost‑effective approach.

    Hereditary Hemorrhagic Telangiectasia — Covered when ANY of the following are met

    Hereditary Hemorrhagic Telangiectasia — Covered when ANY of the following are met

    HHT testing indications: Refer for diagnostic genetic testing to identify causative mutation in families with clinically confirmed HHT; to establish diagnosis in relatives of a person with known causative mutation (including asymptomatic individuals and those desiring prenatal testing); and to assist diagnosis in individuals who do not meet clinical diagnostic criteria. Consider SMAD4 testing if ENG and ACVRL1 are negative.

    HHT panels should include ACVRL1 and ENG at minimum.

    Summary of revised coverage criteria (partial)

    Policy updated: specific panel coverage criteria were revised, reorganized, or relocated; examples below reflect changes noted in this section of the document.

    Relocation of known familial variant criteria: Known Familial Variant Analysis criteria for cardiac disorders were moved to the 'Genetic Testing: General Approach to Genetic and Molecular Testing' policy.

    See general genetic testing policy for single-variant familial testing workflow.

    Post-heart transplant testing: Post Heart Transplant gene expression and donor-derived cfDNA panels updated; routine monitoring frequency defaulted to once every 12 months absent guideline specifying otherwise.once per 12 months default

    Reference table updated (e.g., donor-derived cfDNA added).

    Panel naming and diagnostic threshold updates: Multiple disease-specific panels (Brugada, LQTS, ARVC/arrhythmogenic cardiomyopathy, HCM, DCM, CPVT, SQTS, FH) had wording, diagnostic thresholds, and criteria reorganized; full decision trees and gene lists are in panel-specific sections.see disease-specific criteria

    Examples: ARVC renamed Arrhythmogenic Cardiomyopathy; Brugada ECG thresholds clarified; SQTS diagnostic QTc cutoff ≤330 ms.

    Current evidence does not support the use of comprehensive cardiomyopathy panels for indications other than those explicitly listed in the criteria. Comprehensive cardiomyopathy panels are considered medically necessary only when the member meets the specific cardiomyopathy diagnosis or family SCD/SUD autopsy criteria described in the policy; panels ordered outside those indications are not supported.

    Current evidence does not support the use of comprehensive arrhythmia panels or combined cardiomyopathy/arrhythmia panels for indications beyond the listed criteria. Comprehensive arrhythmia panels and combined panels are medically necessary only when the member meets the specific arrhythmia and/or combined-panel clinical criteria in the policy; ordering these broad panels for other indications is not supported.

    Hypothesis-free or unfocused genetic testing (for example, very large gene panels, whole-exome sequencing, or whole-genome sequencing) performed in the absence of an associated clinical phenotype is discouraged for routine patient care and counseling. Such testing may be considered only in a research or scientific context and is not recommended as standard diagnostic practice.

    Before diagnosing Brugada syndrome or ordering genetic testing, Brugada phenocopies (such as myocardial ischemia, electrolyte disturbances, or drug intoxications) should be excluded. Genetic testing for Brugada is focused primarily on SCN5A and should only be pursued after phenocopies have been ruled out and clinical ECG/feature criteria are met.

    The Left Ventricular Non-Compaction (LVNC) cardiomyopathy panel criteria set has been retired due to the rarity of testing and was removed from active panel-specific criteria.

    Coverage is subject to the member's plan documents, including evidence of coverage, contract terms, and any state Medicaid provisions. Providers must verify member benefits and applicable plan exclusions or limitations prior to ordering testing.

    Current evidence does not support genetic testing for restrictive cardiomyopathy (RCM) via multigene panel for any indication; multigene panel testing for RCM is not supported.

    Genetic testing for Brugada syndrome using genes other than SCN5A, including multigene panels, is not supported for diagnostic purposes. SCN5A variant analysis is the supported genetic testing approach when the Brugada clinical and ECG criteria are met.

    Hereditary Hemorrhagic Telangiectasia (HHT) multigene panel testing is considered medically necessary only for the listed HHT clinical diagnostic features and when the panel includes at minimum ACVRL1 and ENG. HHT panel testing for other indications is not supported, and unfocused hypothesis-free panels without a phenotype are discouraged.

    Testing of genes with disputed ClinGen gene–disease validity for Brugada is discouraged in the diagnostic setting. Aside from SCN5A, many genes implicated in Brugada have disputed ClinGen validity and generally should not be reported diagnostically.

    Language previously characterizing some panel testing as 'investigational' has been revised to the phrasing 'current evidence does not support...' for items where evidence remains insufficient. Policy wording and panel-specific notes were updated to reflect this change.

    Coding and Test Identifiers

    Comprehensive Cardiomyopathy Panels - example codesmixed
    81439Comprehensive cardiomyopathy panel (example billing code listed)
    I42.0Diagnoses associated with cardiomyopathy referenced
    I42.1Hypertrophic cardiomyopathy diagnosis referenced
    I42.2Other specified cardiomyopathies referenced
    I42.5Restrictive cardiomyopathy diagnosis referenced
    I42.8Other cardiomyopathies referenced
    I42.9Cardiomyopathy, unspecified
    Z13.71Encounter for genetic screening
    Z82.41Family history of sudden cardiac death
    Z82.49Family history of other heart disease
    1–10 of 12
    1/2
    Comprehensive Arrhythmia Panels - example codesmixed
    81413Comprehensive arrhythmia panel (example)
    81414Comprehensive arrhythmia panel (example)
    0237USpecific molecular test code listed for arrhythmia panels
    I45.81Long QT syndrome and related conduction disorders
    I49.8Other specified cardiac arrhythmias
    Other cardiac-related panels (FH, congenital, TAAD)mixed
    81401Familial hypercholesterolemia panel example
    E78Disorders of lipoprotein metabolism and other lipidemias
    Q20Congenital malformation of cardiac chambers and connections
    I71.00-I71.9Thoracic aortic aneurysm and dissection codes range
    Q87.5Congenital malformation syndromes predominantly associated with short stature (used in panels listing)
    Hereditary Hemorrhagic Telangiectasia (HHT) panel codesmixed
    R04.0Epistaxis (used in HHT panel listing)
    Q27.30-Q27.39Other congenital malformations of peripheral vascular system range
    Panel CPT codes (panel-specific - use appropriate panel code if performed)mixed
    No codes listed
    Removed CPT Codes / historicalCPT
    81404CPT code previously listed for Restrictive Cardiomyopathy panels (removed in revision).
    Policy reference - example tests updatedHCPCS
    0493UProspera (Natera) (listed in policy reference table)
    Left ventricular (LV) wall thickness — thresholds
    Adult LV wall thickness threshold>= 15 mm
    Pediatric LVH thresholdz-score >= 2
    Modality for measurementEchocardiogram or cardiac MRI
    HCM LV wall thickness — thresholds
    HCM adult diagnostic threshold>= 15 mm myocardial wall thickness
    HCM pediatric diagnostic thresholdz-score >= 2
    Measurement methodsDetermined by echocardiogram or cardiac MRI
    DCM systolic dysfunction — criteria
    DCM systolic dysfunction thresholdLeft ventricular ejection fraction <50%
    Required accompanying findingsLeft ventricular enlargement or biventricular dilatation on echo or cardiac MRI
    Exclusion of non-genetic causesNon-genetic causes (eg, prior MI, valvular disease, toxins, thyroid, inflammatory/infectious, severe hypertension, radiation) should be ruled out
    Arrhythmogenic cardiomyopathy MRI/volumes/EF — thresholds and criteria
    Major MRI RV volume threshold (male)Rao RVEDV/BSA >110 mL/m2 (male)
    Major MRI RV volume threshold (female)Rao RVEDV/BSA >100 mL/m2 (female)
    Major RVEF thresholdRVEF <40%
    Minor criteria ranges (MRI)Rao RVEDV/BSA >100 to <110 mL/m2 (male), >90 to 100 mL/m2 (female); RVEF 40–45%
    Long QT QTc thresholds
    Long QTc threshold (prepuberty)>460 ms
    Long QTc threshold (adults)>480 ms
    Context for measurementConfirmed on resting ECG and/or provocative stress testing (exercise or pharmacologic) per policy
    Short QT QTc threshold
    Short QT diagnostic QTc cutoffQTc <= 330 ms
    SQTS diagnostic score useDiagnostic score >=4 supports testing; at least 1 ECG point required
    Measurement contextECG recorded without modifiers known to shorten QT (resting ECG, Holter, exercise)
    Left ventricular ejection fraction — threshold
    Left ventricular ejection fraction threshold< 50%
    Role in DCM diagnosisEF <50% constitutes systolic dysfunction when coupled with LV enlargement
    Measurement modalitiesEstimated from 2D echocardiogram, cardiac MRI, or left ventricular angiogram
    Schwartz score for LQTS testing — thresholds
    Schwartz score high-probability threshold>= 3.5 points = high probability (policy cites ~3.5 for high probability)
    Intermediate and low ranges1.5–3 points = intermediate; <=1 = low
    Use in testing decisionsSchwartz score guides selection of patients for molecular screening (score >=3.0 recommended for testing per references)
    QT interval cutoff for SQTS — threshold
    SQTS QT interval diagnostic cutoff<= 330 ms
    Associated testing recommendationQTc <=330 ms meets diagnostic threshold for SQTS and supports multigene testing
    Source guidanceHRS/EHRA/APHRS expert consensus (Priori et al) and Wilde et al updates cited
    Diagnostic score thresholds — SQTS and LQTS
    SQTS high-probability score>= 4 points
    SQTS intermediate and low3 points = intermediate; <=2 points = low
    LQTS probability thresholdsLQTS: <=1 low; 1.5–3 intermediate; >=3.5 high
    QTc thresholds for SQTS — point scoring
    QTc point scoring for SQTSQTc <370 ms =1 point; <350 ms =2 points; <330 ms =3 points
    Diagnostic cutoff reiteratedDiagnostic cutoff for SQTS diagnosis: <=330 ms
    Scoring componentsScore also includes J-point–T-peak interval, clinical history, family history, and genotype points
    Brugada ECG threshold — diagnostic criteria
    Brugada diagnostic J-wave thresholdJ-wave elevation >=2 mm in right precordial leads (V1–V3)
    ECG pattern typesType 1–3 ECG patterns described: type 1 coved with negative T wave; type 2 saddleback with >=1 mm ST elevation; type 3 <1 mm ST elevation
    Phenocopy exclusionBrugada phenocopies (ischemia, electrolyte disturbance, drug intoxication) must be excluded before diagnosis/testing
    LDL-C thresholds for FH testing — numeric criteria
    FH pediatric LDL-C thresholdChildren: persistent LDL-C >=160 mg/dL (with family history criteria)
    FH adult LDL-C thresholdAdults: persistent LDL-C >=190 mg/dL (without secondary cause) — higher thresholds (>=250 mg/dL) strengthen indication
    Measurement requirementsTwo or more LDL-C measurements including assessment after intensive lifestyle modification; if LDL-C unavailable, total cholesterol cutoffs may be used

    Provider Actions, Documentation, and Prior Authorization

    Documentation Required

    Phenotype‑focused panel requirement

    Genetic multigene panel testing should be focused to the clinical phenotype being investigated. Order panels that target the suspected condition (for example, HHT panels must include at minimum ACVRL1 and ENG). Hypothesis‑driven, phenotype‑focused panels increase diagnostic yield and reduce uninterpretable findings.

    • Phenotype‑focused panels required to include key genes for the indication (e.g., HHT: ACVRL1, ENG; FH: LDLR, APOB, PCSK9; HTAD panels per GeneReviews list).
    • Unfocused, hypothesis‑free large panels, exome, or genome sequencing without a defined clinical phenotype are discouraged for routine clinical care and may be considered only in a research setting or with explicit multidisciplinary justification.
    Prior Authorization

    Prior authorization for referenced tests

    Prior authorization is required per the payer's administrative policies for covered Concert/registered cardiovascular genetic panels and for specific referenced tests (for example, Donor‑Derived Cell‑Free DNA and updated tests added to the coding table such as 0493U - Prospera). Verify test registration on the Concert Platform and obtain prior authorization where required before ordering.

    • Providers must reference the Concert Platform for registered tests and follow plan-specific prior authorization procedures.
    • Some tests referenced in the policy reference table (e.g., 0493U - Prospera) have been added to coding updates; follow payer guidance and obtain prior authorization as applicable.
    Billing Rule

    Inclusion or exclusion of codes in the policy does not guarantee coverage

    Inclusion or exclusion of codes in this policy is for informational purposes only and does not guarantee coverage. Providers must reference current coding guidance and payer claims rules when submitting reimbursement requests.

    • CPT/HCPCS/ICD codes shown in the policy are examples only and are not exhaustive.
    • Inclusion of a code does not imply coverage; exclusion does not imply non‑coverage. Verify benefit coverage and coding with the payer prior to claim submission.
    Denial Risk

    Comprehensive panel denial trigger

    Comprehensive cardiomyopathy+arrhythmia panels that include both cardiomyopathy and arrhythmia gene sets will be denied if the member does not meet BOTH the clinical criteria for Comprehensive Cardiomyopathy Panels AND the clinical criteria for Comprehensive Arrhythmia Panels. Order comprehensive mixed panels only when both sets of criteria are clearly met.

    • Comprehensive panels combining cardiomyopathy and arrhythmia genes: require meeting ALL criteria for both indications per policy (see respective sections).
    • Ordering these broad combined panels for single‑indication evaluation may trigger denial.
    Denial Risk

    Restrictive cardiomyopathy denial trigger

    Current evidence does not support genetic testing for restrictive cardiomyopathy (RCM) via multigene panel for any indication; ordering an RCM multigene panel may result in denial.

    • Restrictive cardiomyopathy multigene panels are considered investigational/not supported — do not order these panels for diagnostic evaluation under routine clinical criteria.
    Denial Risk

    Unfocused testing discouraged

    Hypothesis‑free, unfocused testing (very large panels, whole‑exome sequencing, whole‑genome sequencing) without a defined clinical phenotype is discouraged for routine clinical care and may be denied or referred for peer review unless justified by a multidisciplinary team or as part of a research protocol.

    • Unfocused testing may be considered only with clear multidisciplinary justification or research context.
    • For investigation of sudden unexplained death or complex cases, testing should remain focused on likely candidate genes when a phenotype is present.
    Denial Risk

    Testing may be denied if criteria revisions are not met

    Testing may be denied if updates to criteria (panel selection, diagnostic thresholds, or required clinical documentation) are not met at the time of review. Ensure documentation supports the specific numeric and clinical criteria cited in the policy (for example LV wall thickness, ejection fraction thresholds, QTc values, LDL‑C levels, imaging measurements, and family history).

    • Clinical documentation must explicitly support policy thresholds and diagnostic criteria (e.g., LV wall thickness ≥15 mm for HCM, ejection fraction <50% for DCM, QTc thresholds for LQTS, LDL‑C thresholds for FH).
    • If required phenotype measurements, imaging, ECG, or family history are missing or insufficient, prior authorization or claim review may result in denial.
    Documentation Required

    Known familial variant routing

    Known familial variant testing should follow the separate General Approach policy for Known Familial Variant Analysis. When a pathogenic or likely pathogenic familial variant has already been identified in an affected family member, order targeted testing (variant analysis) for at‑risk relatives per the General Criteria rather than broad panels.

    • If a familial pathogenic or likely pathogenic variant is known, use the Known Familial Variant Analysis pathway per the general genetic testing policy.
    • Ordering full panels instead of targeted familial variant testing when a known familial variant exists may trigger denial or additional review.
    Prior Authorization

    Post‑transplant monitoring frequency

    For post‑heart transplant monitoring panels (gene expression or donor‑derived cfDNA), in the absence of specific evidence‑based guideline recommendations for a different schedule, routine post‑transplant monitoring is defaulted to once every 12 months for coverage consideration. Individual patient needs may justify different intervals with supporting documentation.

    • Default coverage frequency for routine post‑transplant monitoring panels is once every 12 months unless guideline or chart support specifies otherwise.
    • Prior authorization and documentation of transplant status and clinical indication are required for post‑transplant monitoring tests.
    Documentation Required

    Clinical evaluation and sample documentation

    Clinical evaluation and sample documentation are required. Documentation should include a multidisciplinary clinical evaluation (cardiology/cardiac genetics as appropriate), detailed phenotype (history, family history, ECG measurements, imaging reports), and evidence that an appropriate DNA sample was collected and is available for testing or future confirmatory analysis.

    • Document ECG measurements (e.g., QTc, J‑point/T‑peak intervals), imaging findings (echo/MRI measurements and reports), and relevant numeric thresholds per the policy indication.
    • For SCA/SUD evaluations, document autopsy findings and storage of tissue suitable for DNA testing when applicable. Record that informed pre‑test counseling occurred and plan for results return and family cascade testing is in place.

    Background and Scope

    Genetic testing is used to diagnose and manage inherited and sporadic cardiovascular conditions including structural cardiomyopathies, channelopathies (electrical disorders), vascular disorders such as heritable thoracic aortic disease, and lipid disorders like familial hypercholesterolemia. Because of test complexity and potential for misinterpretation, pre‑test and post‑test genetic counseling and consultation with a cardiac genetics expert are strongly advised to guide test selection, result interpretation, and cascade testing of at‑risk relatives.

    Definitions and Key Terms

    Genetic counseling — recommendation
    Genetic counseling requirementPre-test and post-test genetic counseling that facilitates informed decision-making and addresses possibility of secondary/incidental findings
    Counseling providerTesting decision and interpretation should be performed by, or in consultation with, a cardiac genetics expert
    Purpose of counselingIncludes plan for returning results prior to testing
    Left ventricular hypertrophy (LVH) — definition
    LVH (adult) definitionMyocardial wall thickness >=15 mm on echocardiogram or cardiac MRI
    LVH (children) definitionz-score >=2 on echocardiogram or cardiac MRI
    ContextUsed as diagnostic threshold for hypertrophic cardiomyopathy panel coverage
    Prolonged QTc — definition and thresholds
    Prolonged QTc thresholds>460 ms prepuberty; >480 ms adults on resting ECG or provocative testing
    Measurement contextConfirmed on serial 12-lead ECGs or during exercise/pharmacologic provocation per guideline criteria
    Role in testingMeets criteria for LQTS panel coverage when asymptomatic with confirmed prolongation
    HHT clinical criteria — diagnostic features
    HHT major clinical featuresSpontaneous recurrent epistaxis, mucocutaneous telangiectases at characteristic sites, or visceral arteriovenous malformations
    Panel gene minimumPanel must include at minimum ACVRL1 and ENG to be considered medically necessary
    Use caseTesting used to establish or confirm diagnosis and for cascade testing of relatives
    DCM definition — diagnostic criteria
    DCM definitionLeft ventricular enlargement and systolic dysfunction; an ejection fraction less than 50% is considered systolic dysfunction
    Primary measurementLeft ventricular ejection fraction estimated by echocardiogram or cardiac MRI
    Clinical contextNon-genetic causes should be excluded before genetic testing
    Close relatives — definition
    Definition of close relativesIncludes first-, second-, and third-degree blood relatives; first-degree = parents, siblings, children
    Second- and third-degree examplesSecond-degree: grandparents/aunts/uncles/nieces/nephews/half-siblings; Third-degree: great-grandparents/great-aunts/uncles, first cousins
    Use in policyFamily history of affected close relatives factors into panel coverage decisions
    Phenocopy — definition and examples
    Phenocopy definitionTrait or disease resembling that caused by a genotype but occurring in an individual who is not a carrier of that genotype
    RelevanceBrugada phenocopies must be excluded prior to diagnosis/testing
    Example causesIschemia, electrolyte disturbances, drug intoxication (policy cites these for Brugada)
    Premature coronary artery disease (CAD) — definition
    Premature CAD definitionMales <=55 years and females <=65 years
    Role in FH criteriaPremature CAD in family contributes to FH testing indication when combined with LDL-C thresholds
    SourceAdapted from American Heart Association phenotype definitions cited in policy
    High-probability LQTS — definition and guidance
    High-probability LQTS criteriaClinical history, family history, ECG characteristics including Schwartz Score ~3.5 indicates high probability
    Role in testingMolecular testing for definitive LQTS genes recommended for index patients with high-probability diagnosis
    Reference guidanceWilde et al. 2022 expert consensus and Schwartz scoring cited
    Genetic evaluation/testing for cardiomyopathy and channelopathies — references
    Guideline and consensus sourcesACMG, AHA/ACC, HRS/EHRA/APHRS expert consensus statements and GeneReviews cited
    ApplicationThese references inform testing recommendations for cardiomyopathy and channelopathy panels
    Use by providersClinical documentation should reference relevant guideline-based diagnostic criteria when requesting testing