ModifiedUnitedHealthcarePolicy 2026T0552CC

Genetic Testing for Cardiac Disease

Policy governing coverage and medical necessity criteria for multi-gene and other genetic testing related to inherited arrhythmias, cardiomyopathies, and thoracic aortic disease for UnitedHealthcare Commercial and Individual Exchange plans.

Policy Summary

PayerUnitedHealthcare

PolicyGenetic Testing for Cardiac Disease

Policy CodePolicy 2026T0552CC

Change TypeCode list update (added 0617U)

Effective DateApr 1, 2026

Next Review Date

Key ActionVerify prior authorization requirements against the member's plan and updated CPT code list (including new code 0617U).

SourceLink

POLICY UPDATE CHANGES

Updated list of applicable CPT codes to reflect quarterly edits; added 0617U

Archived previous policy version 2026T0552BB

1new CPT code added (0617U)

≥5panels addressed by policy

8HCM initial strong genes

3definitive LQTS genes

72-80%LQTS variant detection

Trek Health ingests and normalizes Transparency in Coverage data and payer policy updates to give provider organizations a clear view of how commercial reimbursement behaves across markets, payers, and services. Our platform transforms raw payer disclosures into structured intelligence that supports contract evaluation, payer negotiations, and service line strategy. By combining market benchmarks with ongoing policy visibility, Trek helps teams identify variability, risk, and opportunity in commercial reimbursement. The result is faster insight, stronger negotiating positions, and more informed financial decisions.

Coverage Criteria for Genetic Testing in Cardiac Disease

inv-01: Inherited Arrhythmias - Covered when ANY of the following are met

Multi-gene panel testing is proven and medically necessary in individuals with a confirmed or suspected diagnosis of any of the following conditions:

Inherited arrhythmia indications: Brugada syndrome (BrS); or Catecholaminergic polymorphic ventricular tachycardia (CPVT); or Familial long QT syndrome (LQTS) when acquired causes have been ruled out and one of the following criteria are met: prolonged QTc (>460 ms) on exercise/ambulatory ECG, Holter monitoring, or during pharmacologic provocation testing; or Short QT syndrome (SQTS)

inv-02: Inherited Cardiomyopathies - Covered when ANY of the following are met

Multi-gene panel testing is proven and medically necessary in individuals with a confirmed or suspected diagnosis of any of the following conditions:

Inherited cardiomyopathy indications: Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C); or Dilated cardiomyopathy (DCM) without identifiable cause when one of the following are met: cardiac conduction disease (1st–3rd degree block) OR sudden cardiac death in a first- or second-degree relative at age ≤45; or Hypertrophic cardiomyopathy (HCM) without an identifiable cause (eg valvular disease, hypertension, infiltrative or neuromuscular disorder); or Left ventricular noncompaction cardiomyopathy (LVNC)

inv-03: Inherited Thoracic Aortic Disease - Covered when ANY of the following are met

Multi-gene panel testing is proven and medically necessary for either of the following:

Aortic disease indications: Individual has confirmed thoracic aortic disease; OR thoracic aortic disease is suspected based on family history of thoracic aortic disease in a First- or Second-Degree Relative

inv-04: Testing Based Only On Family History - Covered when ANY of the following are met

Multi-gene panel testing is proven and medically necessary in asymptomatic individuals who have a First-Degree or Second-Degree Relative with one of the following conditions:

Family-history based testing indications: Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C); or Brugada syndrome (BrS); or CPVT; or Congenital long QT syndrome (LQTS); or Familial dilated cardiomyopathy (DCM); or Hypertrophic cardiomyopathy (HCM); or Left ventricular noncompaction cardiomyopathy (LVNC); or Short QT syndrome (SQTS); or a First-Degree Relative experienced sudden cardiac death or near sudden death at age ≤45

inv-05: Not covered / Not medically necessary

Unproven genetic tests: All other genetic testing for cardiomyopathies, arrhythmias, or aortic vascular disease is unproven and not medically necessary due to insufficient evidence of efficacy (does not apply to chromosome microarray for isolated severe congenital heart disease). Genetic testing for coronary artery disease (CAD), including gene expression tests and microarray or other genetic profiles for cardiac disease risk (examples listed), is unproven and not medically necessary.

inv-06: Condition-specific evidence and testing considerations

Condition-specific evidence summaries and guidance included in this policy excerpt:

Long QT Syndrome (LQTS): Genetic testing has diagnostic and prognostic value for LQTS; genetic variants are identified in a substantial proportion of clinically diagnosed individuals. Three genes (KCNQ1, KCNH2, SCN5A) are definitive and some syndromic genes (CALM1-3, TRDN) have strong evidence. Clinical criteria (eg, Schwartz score) have limited sensitivity compared with genetic testing.QTc >470 ms (males) or >480 ms (females)

CPVT presents in childhood/adolescence with exercise- or stress-induced arrhythmia; established genes include RYR2, CASQ2, CALM1-3, TRDN, TECRL. Genetic testing identifies many cases but a proportion remain gene-negative (~25%); family testing has probable utility.

Brugada Syndrome (BrS): BrS is an inherited channelopathy often associated with SCN5A variants (15–30%); testing yields variants in ~25–35% of high-suspicion individuals. Routine reporting of variants in genes with disputed validity is not recommended.

inv-07: LQTS - Covered when ALL of the following are met

Molecular genetic testing is recommended for definitive disease-associated genes in index patients with high-probability LQTS.

LQTS testing criteria: Index patient has a high-probability diagnosis of LQTS based on clinical history, family history, and ECG characteristics (Schwartz Score ≥ 3.5). Molecular testing should target definitive genes (eg KCNQ1, KCNH2, SCN5A, CALM1-3) and gene-specific testing for syndromic forms as indicated. Variant-specific testing is recommended for relatives.Schwartz Score >= 3.5

Predictive testing in related children recommended from birth for pathogenic variants.

inv-08: CPVT - Covered when ALL of the following are met

Genetic testing recommended for patients satisfying diagnostic criteria for CPVT.

CPVT testing criteria: Patient meets diagnostic criteria for CPVT (eg Class 1 clinical diagnosis or CPVT diagnostic score > 3.5); test established CPVT genes (RYR2, CASQ2, CALM1-3, TRDN, TECRL). For modest phenotypes (score ≥2 and <3.5) testing may be considered. Variant-specific testing recommended for relatives.CPVT diagnostic score thresholds

Predictive testing in related children recommended from birth for pathogenic variants.

inv-09: Brugada Syndrome - Covered when ALL of the following are met

Targeted sequencing of SCN5A is recommended for index cases with diagnostic ECG and supporting features.

BrS testing criteria: Index case has BrS with spontaneous or drug-induced type I ECG in standard or high precordial leads with supporting clinical features or family history. Sequence SCN5A; do not routinely report variants in genes with disputed/refuted validity. Variant-specific testing recommended for relatives.Type I ECG

Predictive testing for pathogenic SCN5A variants may be offered from birth.

inv-10: SQTS - Covered when ALL of the following are met

Genetic testing recommended for definitive SQTS genes in patients meeting diagnostic criteria.

SQTS testing criteria: Patient satisfies SQTS diagnostic criteria (eg SQTS diagnostic score ≥ 4); test definitive genes (KCNH2, KCNQ1) and consider KCNJ2, SLC4A3 when cardiologist establishes high probability. Variant-specific testing recommended for relatives.SQTS diagnostic score >= 4

Predictive testing in children may be considered in specific settings.

inv-11: Inherited Atrial Fibrillation - Covered when phenotype is established

Genetic testing may be performed for specific genes when familial/young-onset atrial fibrillation (AF) is established by phenotype.

Inherited AF testing criteria: Index patients with familial or young-onset AF (eg familial AF or age <60, and consider testing for age <45 per ACC/AHA guidance) established by clinical history, family history, and ECG may undergo analysis of SCN5A, KCNQ1, MYL4 and truncating TTN variants. Variant-specific testing may be recommended for relatives.Age <60 (familial AF); consider age <45 for early-onset guidance

Predictive testing in children may be considered in specific settings.

inv-12: Hypertrophic Cardiomyopathy (HCM) - Covered when ALL of the following are met

Genetic testing for HCM is used to identify pathogenic sarcomeric variants in probands and enable cascade screening of relatives.

HCM testing criteria: Clinical diagnosis of HCM based on imaging (non-dilated LV with wall thickness ≥13-15 mm in adults) or a proband with HCM; test core sarcomeric genes (eg MYH7, MYBPC3, TNNI3, TNNT2, TPM1, MYL2, MYL3, ACTC1). Testing is particularly useful with family history or younger onset. Variant-specific cascade testing for relatives is recommended.LV wall thickness >=13-15 mm

Genetic counseling recommended pre- and post-test.

inv-13: Arrhythmogenic Cardiomyopathy (ACM/ARVC) - Covered when ALL of the following are met

Genetic testing is part of diagnostic criteria and can be required for certain ACM subtypes.

ACM testing criteria: Clinical features consistent with ACM/ARVC per diagnostic task force criteria (ECG, imaging, arrhythmia documentation, family history) warrant genetic testing. Identification of a LP/P variant is a major diagnostic criterion and may be required for ALVC diagnosis. First-tier genes include desmosomal and other ACM-associated genes (eg PKP2, DSP, DSG2, DSC2, JUP, TMEM43).ITF/modified 2010 ARVC diagnostic criteria

Variant-specific testing recommended for relatives; consider testing the most severely affected family member first in multiplex families.

inv-14: Genetic testing indications and approach

Covered when criteria below (per professional society guidance) are met:

Proband testing indication: Genetic testing is recommended for probands with a confirmed diagnosis of cardiomyopathy (HCM, DCM, LVNC) or ACM, including post-mortem cases, to identify pathogenic/likely pathogenic variants.

References: ACC/AHA and EHRA/HRS guidance.

Initial gene tier for HCM: Initial testing should include genes with definitive/strong evidence (MYH7, MYBPC3, TNNI3, TNNT2, TPM1, MYL2, MYL3, ACTC1).

Initial gene tier for ACM: First-tier testing for ACM should include definitive disease-associated genes (PKP2, DSP, DSG2, DSC2, JUP, TMEM43, PLN, FLNC, DES, LMNA).

Cascade testing: Variant-specific testing is recommended for relatives when a disease-causative variant has been identified in the proband; cascade testing is not recommended if no P/LP variant was found in the proband.

inv-15: HCM Coverage Criteria

Genetic testing coverage guidance for HCM — recommendations when covered

HCM initial tier genes: Initial testing should include genes with definitive or strong evidence: MYH7, MYBPC3, TNNI3, TPM1, MYL2, MYL3, ACTC1, TNNT2.

HCM moderate evidence genes: Initial tier may include genes with moderate evidence: CSRP3, TNNC1, JPH2.

Predictive testing in children: Predictive genetic testing recommended for related children aged >10-12 years; may be considered below that age when family history of early-onset disease exists.age >10-12 years

Cascade testing guidance: Cascade genetic testing of relatives is recommended for identified P/LP variants; not recommended in families where proband testing found no LP/P variants.

inv-16: ACM Coverage Criteria

Genetic testing coverage guidance for ACM

ACM comprehensive testing: Comprehensive genetic testing is recommended for patients with consistent phenotypic features of ACM, including post-mortem cases.

ACM first-tier genes: Test first-tier definitive disease-associated genes: PKP2, DSP, DSG2, DSC2, JUP, TMEM43, PLN, FLNC, DES, LMNA.

ACM borderline phenotype: Comprehensive testing may be considered in borderline ACM phenotypes; identification of LP/P variant useful to confirm diagnosis.

ACM predictive testing children: Predictive testing recommended for related children >10-12 years; may be considered below that age for early-onset family history.age >10-12 years

inv-17: DCM Coverage Criteria

Genetic testing coverage guidance for DCM

DCM recommended when family history present: Genetic testing is recommended for probands with DCM and family history of DCM; initial tier should include definitive/strong genes (BAG3, DES, FLNC, LMNA, MYH7, PLN, RBM20, SCN5A, TNNC1, TNNT2, TTN, DSP).

DCM useful in sporadic severe cases: Genetic testing can be useful for apparently sporadic DCM with severe systolic dysfunction (LVEF <35%), malignant arrhythmia phenotype, or younger age of onset.LVEF < 35%

DCM moderate evidence genes: Initial tier may include moderate evidence genes: ACTC1, ACTN2, JPH2, NEXN, TNNI3, TPM1, VCL.

DCM predictive testing children: Predictive testing recommended for related children >10-12 years; may be considered below that age if family history of early-onset disease exists.

inv-18: LVNC Coverage Criteria

Genetic testing coverage guidance for LVNC

LVNC testing when clinically diagnosed: Genetic testing may be useful for patients with a clinical diagnosis of LVNC established by cardiologist using history, family history, and ECG/echo/MRI phenotype, or when syndromic features are present.

LVNC not indicated for isolated incidental: Do not perform genetic testing for isolated (incidental) LVNC with normal LV function, no syndromic features, and no family history.

inv-19: HTAD Coverage Criteria

Genetic testing coverage guidance for inherited thoracic aortic disease (HTAD)

HTAD testing when clinical features suggest syndromic disease: Target testing based on clinical features (eg test FBN1 for Marfan phenotype); otherwise consider multi-gene panels (≈15–16 genes) for HTAD when clinical overlap exists.

HTAD core genes: Panels typically include core high-penetrance HTAD genes: FBN1, LOX, COL3A1, TGFBR1, TGFBR2, SMAD3, TGFB2, ACTA2, MYH11, MYLK, PRKG1; ClinGen identified 9 genes with definitive causation and additional genes with strong evidence.

HTAD cascade testing: For individuals with an established P/LP variant predisposing to HTAD, genetic counseling and cascade testing of at-risk biological relatives is recommended; management guided by specific gene/variant.

HTAD testing yield note: Genetic testing is negative in ~70% of families with HTAD without systemic features; consider referral to research if testing is negative.

inv-20: Evidence-based coverage considerations

Coverage considerations informed by evidence and guideline statements in the document:

HTAD genetic testing rationale: Genetic testing is supported to identify causal genes associated with penetrant HTADs because it can inform clinical action regarding presentation, syndromic features, dissection risk, and other vascular diseases.

CAD genomic testing stance: For coronary artery disease (CAD), evidence is insufficient to support genomic risk scores or gene expression testing for routine clinical use; while PRS show associations with risk, clinical actionability and predictive performance vary and are debated.

PRS utility and limitations: PRS may modestly improve risk stratification in some populations (eg top decile GPSCAD associated with higher sudden death risk) but generalizability is limited by ancestry differences and lack of consensus on markers and calculation methods.

Genetic testing for coronary artery disease (CAD), including gene expression assays, microarray profiles, DNA methylation analyses, and polygenic risk score (PRS) algorithms, is considered not medically necessary because current evidence does not demonstrate consistent clinical utility for routine risk prediction or management. Examples of tests cited in this policy include gene-expression/mRNA panels and PRS-based algorithms (see Cardiac DNA Insight®, Cardio IQ® tests and PLA-listed algorithms).

Cardiomyopathies that present primarily as neuromuscular disorders and their related genetic testing are outside the scope of this policy and are handled under the separate UnitedHealthcare Medical Policy titled “Genetic Testing for Neurological Disorders.” Providers should refer to that policy for coverage and testing guidance in those presentations.

Routine reporting or testing of genes with disputed or refuted gene–disease validity (for example, some Brugada syndrome candidate genes) is not recommended. Multi-gene panels should emphasize genes with established clinical validity to avoid increasing rates of variants of uncertain significance (VUS) without improving diagnostic yield.

Cascade (variant-specific) testing of relatives is appropriate when a pathogenic or likely pathogenic (P/LP) variant has been identified in a proband. Conversely, cascade genetic testing is not recommended when testing of the proband found no P/LP variant, because testing relatives in that setting is unlikely to be informative.

Genetic testing should not be performed for isolated/incidental left ventricular noncompaction (LVNC) when there is normal left ventricular function, no associated syndromic features, and no relevant family history; testing in that scenario is unlikely to be clinically useful.

Use of genomic markers, polygenic risk scores, gene-expression profiles, or related genomic algorithms to perform routine, universal DNA-based prediction of coronary artery disease (CAD) is not recommended at this time because of insufficient evidence on standardized marker sets, calculation methods, generalizability across ancestries, and demonstrable clinical actionability.

FDA clearance or approval of a test or platform is provided for informational purposes only. FDA approval alone is not a basis for coverage; coverage determinations require demonstration of medical necessity and alignment with the policy’s evidence-based criteria.

This Medical Policy is intended to assist in interpreting UnitedHealthcare standard benefit plans. The member’s specific benefit plan document governs coverage and may differ from the standard policy; in the event of conflict the member-specific benefit plan governs.

All other genetic testing for cardiomyopathies, arrhythmias, or aortic vascular disease not specifically supported by the policy’s criteria is considered unproven and not medically necessary. Broad commercial panels that include many genes of limited or disputed evidence are discouraged because they increase VUS detection without clear benefit.

Searching for common genetic variants or consumer-targeted genetic testing for atrial fibrillation (including routine use of polygenic risk scores) has not been shown to be clinically useful for management decisions and is of uncertain clinical utility; such approaches are therefore not supported for routine clinical use.

In most situations, ongoing clinical cardiac screening is not indicated for genotype-negative relatives of genotype-positive families with hypertrophic cardiomyopathy (HCM). Decisions about surveillance in relatives should be guided by family history, phenotype, and clinical judgment.

For HCM, cascade genetic testing of family members is recommended when a P/LP variant is identified in the proband; however, cascade testing is not recommended for HCM when proband testing found no likely pathogenic or pathogenic variant.

Genomic risk scores and gene expression testing for CAD have insufficient evidence of clinical utility at present. While some studies show associations between PRS and CAD risk, limitations in marker selection, score calculation, ancestry generalizability, and lack of demonstrated impact on management prevent routine coverage.

Covered Indications and Index Patient Guidance

inv-97: Overall covered populations (arrhythmias, cardiomyopathies, HTAD, and asymptomatic relatives)

Overall covered populations: Inherited arrhythmias, inherited cardiomyopathies, inherited thoracic aortic disease, and asymptomatic individuals with a first- or second-degree relative with a qualifying diagnosis as listed in policy are covered for multi-gene panel testing when criteria are met.

inv-98: Evaluation of suspected inherited arrhythmia syndromes and early-onset AF

Evaluation of suspected inherited arrhythmia syndromes and early-onset AF: Evaluation is supported when clinical features, ECG, exercise testing, family history, or guideline recommendations indicate testing (eg Schwartz Score for LQTS, CPVT exercise-provoked arrhythmia, BrS type I ECG, familial or early-onset AF per ACC/AHA guidance).

Family cascade testing after identification of a pathogenic familial variant is supported; targeted testing for the known familial variant is sufficient.

inv-99: Index patients with phenotype and diagnostic criteria for arrhythmia syndromes and cardiomyopathies; cascade testing when pathogenic variant found

Eligibility Requirements and Scope

Coverage for testing based solely on family history requires documentation that an asymptomatic individual has a first‑degree or second‑degree relative with a qualifying diagnosis listed in the policy (for example HCM, DCM, LVNC, ARVD/C, BrS, CPVT, congenital LQTS, SQTS, or sudden cardiac death at age ≤45). Family-history–based requests must include sufficient clinical detail to confirm the relative’s qualifying diagnosis.

This policy excerpt includes top-level eligibility nodes and guidance; when applying criteria, providers should confirm member eligibility and any plan-specific coverage rules in the member’s benefit document.

Coverage for family-history–only testing is limited to asymptomatic individuals with a documented first‑degree or second‑degree relative who meets one of the listed qualifying conditions; other requests should be evaluated against standard proband-based criteria or alternate policies.

When family-history–based testing is requested, documentation should include the relative’s diagnosis, relationship to the member, and relevant clinical data (e.g., genetic test report showing P/LP variant, ECG/imaging results) to confirm eligibility for coverage under the family-history criteria.

A three-generation family history and disease-appropriate phenotyping are recommended and should be included in documentation supporting eligibility; this assists determination of pre-test probability and appropriate testing strategy.

For certain syndromes (for example LQTS, CPVT, BrS), prior clinical evaluation that documents phenotype (ECG findings, exercise testing, arrhythmia history) and any diagnostic scores should accompany requests for genetic testing based on family history.

Coding and Procedure Codes

Applicable CPT and PLA codes referencedmixed

0237U	Cardiac ion channelopathies (e.g., Brugada syndrome, long QT syndrome, short QT syndrome, catecholaminergic polymorphic ventricular tachycardia), genomic sequence analysis panel including ANK2, CASQ2, CAV3, KCNE1, KCNE2, KCNH2, KCNJ2, KCNQ1, RYR2, and SCN5A, including small sequence changes in exonic and intronic regions, deletions, duplications, mobile element insertions, and variants in non-uniquely mappable regions.
0401U	Cardiology (coronary heart disease [CHD]), 9 genes (12 variants), targeted variant genotyping, blood, saliva, or buccal swab, algorithm reported as a genetic risk score for a coronary event.
0439U	Cardiology (coronary heart disease [CHD]), DNA, analysis of 5 single-nucleotide polymorphisms (SNPs) and 3 DNA methylation markers, qPCR and digital PCR, whole blood, algorithm reported as a 4-tiered risk score for a 3-year risk of symptomatic CHD.
0440U	Cardiology (coronary heart disease [CHD]), DNA, analysis of 10 single-nucleotide polymorphisms (SNPs) and 6 DNA methylation markers, qPCR and digital PCR, whole blood, algorithm reported as detected or not detected for CHD.
0466U	Cardiology (coronary artery disease [CAD]), DNA, genome-wide association studies (564,856 single-nucleotide polymorphisms [SNPs], targeted variant genotyping), patient lifestyle and clinical data, buccal swab, algorithm reported as polygenic risk to acquired heart disease.
0617U	Cardiovascular (atherosclerotic cardiovascular disease [ASCVD]), DNA methylation analysis of more than 20,000 sites, whole blood, algorithm reported as positive or negative risk.
81410	Aortic dysfunction or dilation (e.g., Marfan syndrome, Loeys Dietz syndrome, Ehler Danlos syndrome type IV, arterial tortuosity syndrome); genomic sequence analysis panel, must include sequencing of at least 9 genes, including FBN1, TGFBR1, TGFBR2, COL3A1, MYH11, ACTA2, SLC2A10, SMAD3, and MYLK.
81411	Aortic dysfunction or dilation duplication/deletion analysis panel, must include analyses for TGFBR1, TGFBR2, MYH11, and COL3A1.
81413	Cardiac ion channelopathies 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 duplication/deletion gene analysis panel, must include analysis of at least 2 genes, including KCNH2 and KCNQ1.

1–10 of 13

1/2

Covered CPT Codesmixed

No codes listed

Applicable CPT Codes (updated)CPT

0617U

Cardiovascular (atherosclerotic cardiovascular disease [ASCVD]), DNA methylation analysis of more than 20,000 sites, whole blood, algorithm reported as positive or negative risk.

Covered CPT Codesmixed

No codes listed

Applicable CPT Codes (updated)CPT

0617U

Newly added CPT/PLA code listed in applicable codes; Cardiovascular (atherosclerotic cardiovascular disease [ASCVD]), DNA methylation analysis of more than 20,000 sites, whole blood, algorithm reported as positive or negative risk.

Minimum genes for panels

Aortic dysfunction sequencing (CPT 81410)Panel must include sequencing of at least 9 genes (examples: FBN1, TGFBR1, TGFBR2, COL3A1, MYH11, ACTA2, SLC2A10, SMAD3, MYLK)

Cardiac ion channelopathy sequencing (CPT 81413)Panel must include sequencing of at least 10 genes (examples: ANK2, CASQ2, CAV3, KCNE1, KCNE2, KCNH2, KCNJ2, KCNQ1, RYR2, SCN5A)

Hereditary cardiomyopathy sequencing (CPT 81439)Panel must include sequencing of at least 5 cardiomyopathy-related genes (examples: DSG2, MYBPC3, MYH7, PKP2, TTN)

Diagnostic score thresholds

Schwartz Score threshold for LQTSSchwartz Score ≥ 3.5 indicates high probability of LQTS

Provider Actions, Documentation, and Prior Authorization

Prior Authorization

Prior Authorization and Documentation Required

Prior authorization is required for multi-gene panels (≥5 genes) and other genetic tests addressed by this policy. Documentation submitted with the prior authorization request must include relevant clinical information to demonstrate medical necessity, including phenotype assessment, diagnostic score when available (e.g., Schwartz score for LQTS, CPVT diagnostic score, SQTS diagnostic score), and a detailed family history (preferably a three-generation pedigree). Genetic counseling documentation and evidence of discussion about testing implications should be included.

Prior authorization required for panels with ≥5 genes.
Include clinical phenotype and diagnostic scores (e.g., Schwartz score ≥3.5 for likely LQTS) when applicable.
Provide a three-generation family history and pedigree when available.
Document ECG findings, imaging, and other relevant clinical testing results supporting the suspected diagnosis.
Specify whether testing is for a proband (index case) or for cascade/variant-specific testing in relatives.
For cascade testing, provide the identified proband’s pathogenic/likely pathogenic (P/LP) variant and evidence of the proband’s genotype if testing a relative.
Genetic counseling must be documented prior to testing; include counseling note or plan.

Not Covered / Not Medically Necessary

Genetic testing for CAD risk scores, gene-expression profiling, DNA methylation algorithms, and related polygenic risk assessments are listed as not covered because the evidence does not currently support routine clinical use for risk prediction or to guide management decisions.

Examples include gene-expression/mRNA panels and polygenic algorithms described in the policy’s applicable code list; these tests may be denied when submitted for routine CAD risk stratification.

Broad commercial panels that include many genes with limited or disputed evidence are discouraged and may be processed as not covered when they lack a clear, evidence-based gene list targeted to the phenotype. Such panels increase the likelihood of variants of uncertain significance without improving diagnostic yield.

Routine use of polygenic risk scores or consumer/targeted AF genetic testing for atrial fibrillation is of uncertain utility and is considered not supported for routine clinical use at this time. Clinical management should be driven by phenotype and guideline recommendations rather than PRS alone.

Direct-to-consumer genetic tests for cardiovascular disease lack sufficient evidence for routine clinical use and are discouraged. These tests are often variable in scope and methodology and typically lack standardized counseling or confirmatory clinical evaluation, and therefore are considered not covered for routine clinical decision-making.

Genetic testing for isolated or incidental LVNC in individuals with normal ventricular function, no syndromic features, and no family history is explicitly listed as not covered because it is unlikely to alter clinical management.

Additional Provider Notes and Recommendations

Documentation Required

Pre-test genetic counseling strongly recommended (general)

Pre-test genetic counseling is strongly recommended to inform individuals about advantages, limitations, and potential outcomes of genetic testing and should be documented when possible.

Document that counseling was provided or offered prior to testing.
Counseling should cover test scope, possible results, and implications for family members.

Documentation Required

Consider counseling for early-onset AF and inherited arrhythmia syndromes

Consider genetic counseling for patients with early-onset atrial fibrillation (<45 years) and for inherited arrhythmia syndromes when testing and cascade testing are contemplated; document counseling discussion.

Include age at onset and rationale for counseling referral in the record.
Counseling can support decisions about testing and family screening.

Background and Rationale

Genetic testing technologies — including chromosome microarray analysis (CMA), next‑generation sequencing (NGS), whole-exome sequencing, gene-expression arrays, and DNA methylation assays — can assist with diagnosis, prognosis, and clinical interventions for inherited cardiac syndromes when applied in phenotype-driven contexts. This policy focuses on panels or microarray profiles that evaluate five or more genes for cardiac-related syndromes.

Definitions and Key Terms

First‑Degree Relative

DefinitionFirst-degree relatives include parents, siblings, and offspring

ExamplesParents, brothers and sisters, and children of the index patient

Use in policyFirst-degree relative status qualifies an asymptomatic individual for family-history–based testing when a listed condition is present in the relative

Second‑Degree Relative

DefinitionSecond-degree relatives include half-brothers/sisters, aunts/uncles, grandparents, grandchildren, and nieces/nephews affected on the same side of the family

ExamplesHalf-siblings, aunts, uncles, grandparents, grandchildren, nieces and nephews

Coding Notes and Minimum Panel Sizes

Minimum genes for panels (emphasis)

Minimum genes for panels (repeat)Examples provided by CPT: 81410 (≥9 genes for aortic dysfunction), 81413 (≥10 genes for cardiac ion channelopathies), 81439 (≥5 genes for hereditary cardiomyopathy)

CPT examplesSee CPT descriptors for example gene lists (FBN1, TGFBR1, etc. for 81410; ANK2, KCNQ1, SCN5A, etc. for 81413)

ImplicationPanel size minima are used to map clinical panels to CPT codes for billing/reference

Diagnostic score thresholds (emphasis)

Schwartz Score (repeat)Schwartz Score ≥ 3.5 = high probability of LQTS; scoring details include QTc thresholds and clinical/family criteria

CPVT and SQTS thresholds (repeat)

Policy Revision History and Updates

The previous policy version was archived as part of the current update; operational changes include updates to the applicable procedure code list.

Selected references cited in support of the policy include clinical and guideline publications on inherited cardiomyopathies and arrhythmia syndromes, gene-disease validity evaluations, and population genetics studies (examples: Roselli et al. 2018; Topriceanu et al. 2024; Walsh et al. 2022; van Waning et al.). These sources informed the condition-specific recommendations and gene-tiering referenced throughout the policy.

References and Source Literature

The policy’s clinical recommendations and coverage criteria are informed by evidence and guideline sources addressing inherited cardiomyopathies and arrhythmia syndromes, including consensus and systematic reviews, registry studies, and expert statements cited in the references section (for example, EHRA/HRS consensus statements, ACC/AHA guidance, and specialty systematic reviews).

Policy Summary

PayerUnitedHealthcare

PolicyGenetic Testing for Cardiac Disease

Policy CodePolicy 2026T0552CC

Change TypeCode list update (added 0617U)

Effective DateApr 1, 2026

Next Review Date

Key ActionVerify prior authorization requirements against the member's plan and updated CPT code list (including new code 0617U).

SourceLink

On This Page

Short QT Syndrome (SQTS): SQTS is rare; KCNH2 is classified as definitive and KCNQ1, KCNJ2, SLC4A3 have moderate to strong evidence. Panels including many genes with limited evidence increase VUS without increasing yield.

Inherited Atrial Fibrillation (AF): Genetic testing may be useful in early-onset or familial AF, with disease-associated variants more often in cardiomyopathy genes than pure arrhythmia genes; consider testing in young-onset cases per guideline guidance.Age <45–60 years for consideration depending on guideline

Pediatric predictive testing: Predictive genetic testing in related children is recommended for those aged >10-12 years; testing below that age may be considered in families with early-onset disease.age >10-12 years

Genetic counseling: Pre- and post-test genetic counseling by qualified experts is recommended for all individuals undergoing genetic testing for cardiomyopathies.

Index patients with phenotype and diagnostic criteria: Index patients meeting phenotype/diagnostic criteria for inherited arrhythmia syndromes (LQTS, CPVT, BrS, SQTS, inherited AF) or cardiomyopathies (HCM, ACM/ARVC) are candidates for genetic testing; cascade testing of relatives is recommended when a pathogenic/likely pathogenic variant is identified.

Testing is phenotype-driven and guided by diagnostic scores, ECG findings, imaging, and family history.

inv-100: Probands with confirmed or suspected inherited cardiomyopathy and at-risk relatives when P/LP variant identified

Probands with confirmed or suspected inherited cardiomyopathy and at-risk relatives: Probands with confirmed or suspected inherited cardiomyopathy (HCM, DCM, LVNC) or ACM, including post-mortem cases, should undergo genetic testing to identify P/LP variants; variant-specific cascade testing offered to at-risk first-degree relatives when a P/LP variant is identified.

Includes atypical presentations and suspected phenocopies; comprehensive testing for consistent ACM phenotypes is recommended.

inv-101: Proband with clinical HCM (including post-mortem) for identification of P/LP variants

Proband with clinical HCM (including post-mortem cases) should have initial tier testing that includes definitive/strong genes to identify P/LP variants for diagnosis and cascade testing.

Initial tier genes: MYH7, MYBPC3, TNNI3, TPM1, MYL2, MYL3, ACTC1, TNNT2.

inv-102: Patients with phenotypic features consistent with ACM for comprehensive genetic testing

Patients with phenotypic features consistent with ACM (including post-mortem) should receive comprehensive genetic testing focusing on first-tier definitive genes.

First-tier genes include PKP2, DSP, DSG2, DSC2, JUP, TMEM43, PLN, FLNC, DES, LMNA.

inv-103: Probands with DCM and family history or severe/early phenotype

Probands with DCM indications: Probands with DCM and family history of DCM or suggestive features (premature sudden death, conduction disease, malignant arrhythmia) or sporadic DCM with severe phenotype (eg LVEF <35%) or younger age are candidates for genetic testing using established gene tiers.LVEF <35%

Initial tier genes: BAG3, DES, FLNC, LMNA, MYH7, PLN, RBM20, SCN5A, TNNC1, TNNT2, TTN, DSP.

inv-104: Clinically diagnosed LVNC or LVNC with syndromic features

Clinically diagnosed LVNC or LVNC with syndromic features is an indication for genetic testing when established by cardiologist assessment; do not test isolated incidental LVNC with normal function and no family history.

Variant-specific testing may be considered for relatives when P/LP variant identified.

inv-105: Individuals with thoracic aortic aneurysm/dissection and risk factors for HTAD

Individuals with thoracic aortic aneurysm/dissection and risk factors: Individuals with thoracic aortic aneurysm or dissection and risk factors for HTAD or clinical features suggestive of syndromic aortopathy should undergo targeted gene testing (eg FBN1 for Marfan) or multi-gene panels including core HTAD genes.

Panels typically include ≈11 core HTAD genes with definitive/strong evidence.

inv-106: HTAD evaluation where identification of causal gene impacts management

HTAD testing when management impacted: Evaluation for heritable thoracic aortic aneurysm/dissection (HTAD) where identification of a causal gene will impact clinical management is indicated; testing should include genes with strong/definitive evidence and guide surveillance and management.

11 genes noted with strong/definitive evidence for HTAD.

inv-107: Citations supporting inherited cardiomyopathies and arrhythmia syndrome coverage

Citations supporting inherited cardiomyopathies and arrhythmia syndrome coverage: Multiple cited references and guideline statements (ACC/AHA, EHRA/HRS, AHA scientific statements, and primary literature) support the role of genetic testing in inherited cardiomyopathies, arrhythmogenic cardiomyopathy, long QT syndrome, Brugada syndrome, CPVT, and early-onset atrial fibrillation as described in this policy.

See reference list for details.

When testing is requested for cardiomyopathies based on family history, providers should document that testing in a proband has identified a P/LP variant or provide evidence of a qualifying family diagnosis; without such documentation, coverage under family-history criteria is not supported.

Family-history–based testing is intended to identify asymptomatic at-risk relatives for whom a known familial variant or strong family phenotype exists; requests should state the intended use (predictive testing vs diagnostic evaluation) and whether variant-specific testing is being pursued.

Top-level eligibility nodes for HCM emphasize that predictive testing in related children is generally recommended for those aged > 10–12 years, and earlier testing may be considered when there is a family history of early-onset disease.

Eligibility criteria for ACM note that testing of the most clearly affected family member (the proband) first may optimize diagnostic yield and guide cascade testing decisions for relatives.

For DCM, eligibility for genetic testing is supported when there is a family history of DCM or suggestive features (premature sudden death, conduction disease), or in sporadic DCM with severe phenotype (for example LVEF < 35%) or younger age of onset.

Eligibility for LVNC testing is limited to patients with a clinical diagnosis of LVNC established by a cardiologist or when syndromic features are present; testing is not indicated for isolated incidental LVNC without family history or systolic dysfunction.

Eligibility for HTAD testing is based on clinical features suggestive of syndromic or heritable aortopathy or a family history of thoracic aortic disease; targeted testing may prioritize genes such as FBN1 when the phenotype is consistent with Marfan syndrome.

Family-history–only requests that seek genomic risk scores or CAD gene-expression profiling are not supported because these test types lack consensus on marker selection and clinical utility.

When applying eligibility requirements, providers should confirm the member-specific benefit plan and any applicable federal or state mandates, as plan terms may modify coverage.

CPVT diagnostic score thresholds

Class 1 clinical diagnosis or CPVT diagnostic score > 3.5 = diagnostic; modest phenotype: score ≥ 2 and < 3.5 may be considered for testing

SQTS diagnostic score thresholdSQTS diagnostic score ≥ 4 indicates diagnostic criteria met for SQTS

Policy applicability

Member-specific benefit plan governsCoverage determinations must reference the member specific benefit plan document; in the event of a conflict the member specific benefit plan governs

Check applicable mandatesBefore using this policy, verify the member specific benefit plan and any federal or state mandates

Policy as interpretive guidanceThis Medical Policy provides assistance in interpreting UnitedHealthcare standard benefit plans and does not itself override a member's plan terms

Tests for coronary artery disease genomic risk scores or gene expression profiles are considered not medically necessary and will not be authorized under this policy.

Laboratory performing the test must be CLIA-certified; include lab CLIA status and, if applicable, FDA-cleared/approved assay information (note: FDA approval alone does not determine coverage).

Verify member-specific benefits and any additional prior authorization or coding requirements (including updated CPT codes) before ordering.

Documentation Required

Test Selection and Scope

Genetic testing should be phenotype-driven and guided by established evidence-based gene lists where available. Prefer targeted or evidence-based panels (genes with definitive/strong clinical validity) rather than broad, non-specific large panels. Multi-gene panels with fewer than five genes are outside the scope of this policy and should follow alternative plan processes.

When available, order panels that focus on genes with definitive or strong evidence for the suspected condition (e.g., KCNQ1, KCNH2, SCN5A for LQTS; RYR2, CASQ2, CALM1-3, TRDN, TECRL for CPVT; MYH7, MYBPC3, TNNI3, TNNT2, TPM1, MYL2, MYL3, ACTC1 for HCM).
Avoid reporting or relying on genes with disputed or limited evidence for routine clinical decision-making unless new evidence supports inclusion.
Document rationale for selecting a broader panel if clinically justified (e.g., atypical presentation or syndromic features).

Prior Authorization

Cascade and Family Testing

Cascade (family) testing is supported when a P/LP variant has been identified in an index case (proband). Cascade testing for at-risk relatives should be variant-specific whenever possible. Proband-first testing is recommended; testing relatives before identifying a proband variant is not generally indicated.

Obtain and submit the proband’s genetic test report showing the identified P/LP variant when requesting cascade testing.
If variant-specific testing is requested, include the exact nucleotide/amino acid change and variant classification.
If no P/LP variant has been identified in the family, provide clinical justification for any broader family testing (usually not covered).
Document prior genetic counseling discussions and plans for cascade testing uptake and follow-up.

Documentation Required

Required Clinical Documentation

Clinical documentation must support the medical necessity of testing. For cardiomyopathy and arrhythmia syndromes, include specific phenotype details, diagnostic testing results, and family history to justify testing selection and to facilitate variant interpretation and cascade screening.

Provide clinical diagnostic criteria or scores where applicable (e.g., Schwartz score for LQTS; CPVT diagnostic scores; SQTS criteria).
Include ECG findings (e.g., prolonged QTc, Brugada type I ECG), Holter, exercise testing, imaging (echo/MRI), and other relevant clinical data.
For LVNC, document that testing is not for isolated incidental LVNC with normal LV function unless syndromic features or family history are present.
Attach pertinent specialist consultation notes (e.g., cardiology, electrophysiology, clinical genetics) when available.

Billing Rule

Billing, Coding, and Benefit Verification

Provider must verify member-specific benefits and follow plan pre-authorization processes. Coverage is subject to the member’s benefit plan and applicable laws; inclusion of test in policy does not guarantee coverage. Use updated CPT/HCPCS codes when submitting requests and claims.

Confirm eligibility and medical necessity per the member specific benefit plan prior to testing.
Submit prior authorization through the payer’s designated portal/process and include all requested clinical attachments.
Update billing to reflect quarterly CPT edits and any newly added codes (e.g., codes added in policy history).
If coverage is denied, reference the member specific benefit plan and appeal per plan procedures.

Documentation Required

Laboratory and Regulatory Requirements

Laboratory and regulatory considerations: provide the performing laboratory’s CLIA certification and, if relying on an FDA-cleared/approved assay, note that FDA clearance alone does not determine coverage. Tests must be performed in CLIA-certified labs.

Include the performing laboratory name and CLIA number with the prior authorization request.
If the assay is FDA-cleared or -approved, include assay identification; coverage decisions are based on clinical evidence, not FDA status alone.

Genomic risk scores and gene-expression tests for CAD are considered to have insufficient evidence of clinical utility and are therefore not supported as medically necessary for routine clinical use.

Documentation Required