Applicable procedure codes listed — reference only

The following CPT/PLA procedure codes are provided for reference; listing does not guarantee coverage and inclusion does not imply reimbursement eligibility.

• 0237U
• 0401U
• 0439U
• 0440U
• 0466U
• 0617U
• 81410
• 81411
• 81413
• 81414
• 81439
• 81479
• 81493

Prior authorization: favor targeted and variant-specific testing

Genetic testing should be focused on definitive/strong disease-associated genes for index patients and variant-specific testing should be used for relatives once a pathogenic familial variant is identified.

• Target testing is most useful in patients with high-probability clinical diagnosis.
• Variant-specific cascade testing recommended for family members after identification of disease-causing variant.

Phenotype-driven testing and cascade testing

Order testing only when clinical phenotype and pre-test probability support it; perform variant-specific (cascade) testing for relatives only after a P/LP variant is identified in the proband.

• Testing without a diagnostic ECG or appropriate phenotype reduces clinical utility and may lead to denial.
• First-line cascade screening in relatives should prioritize ECG and echocardiography; genetic cascade testing reserved when a familial pathogenic variant is known.

Testing scope: comprehensive ACM panels; HCM initial-tier genes

For arrhythmogenic cardiomyopathy (ACM) perform comprehensive genetic testing including first‑tier genes; for HCM, include genes with definitive/strong evidence in the initial tier.

• ACM first‑tier genes: PKP2, DSP, DSG2, DSC2, JUP, TMEM43, PLN, FLNC, DES, LMNA.
• HCM initial tier should include genes with definitive/strong evidence (e.g., MYH7, MYBPC3, TNNI3, TPM1, MYL2, MYL3, ACTC1, TNNT2).

Genetic testing for cardiomyopathy probands and cascade testing for relatives

Genetic testing is recommended for probands with cardiomyopathy and the most clearly affected family member should be tested first; cascade testing is appropriate for relatives when a pathogenic/likely pathogenic variant is identified.

• Device and medical therapy decisions should be guided by phenotype and clinical guidelines.
• Variant-specific testing recommended for at-risk first‑degree relatives after identification of a familial P/LP variant.

Idaho-specific applicability — verify local authorization rules

This policy applies to UnitedHealthcare Idaho community plan members; check the policy and applicable plan/state requirements for local authorization and coverage rules.

• Refer to federal, state, or contractual requirements as they may supersede this standard policy.
• Check Idaho-specific provider handbook for non-covered or excluded services.

Coverage note — FDA/CLIA (informational)

FDA clearance/approval is provided for informational purposes only; FDA approval alone is not a basis for coverage, and laboratories performing genetic tests are regulated under CLIA.

• FDA approval alone does not guarantee coverage decisions.
• Labs performing genetic tests must comply with CLIA regulations.

Check plan/state coverage for listed CPT/PLA codes (incl. 0617U)

Verify plan and state coverage before billing for the listed CPT/PLA codes; several codes (including newly added 0617U) are noted as not on the Idaho Medicaid Fee Schedule and may be denied by Idaho Medicaid.

• Codes called out as potentially not on Idaho Medicaid Fee Schedule include: 0237U, 0401U, 0439U, 0440U, 0466U, 0617U, 81410.
• Refer to the Idaho Medicaid Provider Handbook for non-covered and excluded services.

Maintain complete medical record documentation to support medical necessity

Ensure the patient's medical record contains legible documentation that fully supports medical necessity for genetic testing requests.

• Include relevant medical history, physical exam, and results of pertinent diagnostic tests or procedures.
• Medical records must be made available upon request for review.

Preferred: targeted gene testing before very large panels

Prefer targeted, gene-specific testing for syndromes with well-established causal genes before ordering broad/comprehensive panels to limit VUS burden and improve utility.

• Example: test KCNQ1, KCNH2, SCN5A for high‑probability LQTS before broad panels.
• Larger panels can increase VUS frequency and may reduce clinical utility.

Gene-targeted sequencing guidance — order genes tied to the clinical diagnosis

When a specific syndromic diagnosis is suspected, order gene-targeted sequencing for the implicated genes (e.g., SCN5A for BrS; KCNH2/KCNQ1 for SQTS) rather than non-specific broad panels.

• Perform gene-specific analysis for syndromic presentations (e.g., KCNQ1/KCNE1 for Jervell and Lange‑Nielsen, CACNA1C for Timothy syndrome).
• Variant-specific testing recommended for relatives after identification of causative variant.

Cascade testing: variant-specific testing for relatives and initial clinical screening

When a pathogenic/likely pathogenic variant is found in a proband, perform cascade (variant‑specific) testing for first‑degree relatives and prioritize initial clinical screening (ECG, echocardiography) as first-line cascade evaluation.

• Cascade genetic testing appropriate for asymptomatic at‑risk first‑ or second‑degree relatives when familial mutation identified.
• Initial cascade screening in relatives may prioritize ECG and echocardiography prior to or alongside genetic testing.

When multiple affected relatives exist, test the most affected/earliest case first

In families with multiple affected members, consider testing the individual with the most severe or earliest phenotype first (the genetic proband) because complex genotypes may be present.

• Testing the most affected case first helps clarify variant segregation and identify the causal variant for cascade testing.

Refer to research when HTAD testing is negative in families without systemic features

If HTAD genetic testing is negative in families without systemic features, consider referral to research studies as additional genes may not yet be identified.

• Approximately 70% of families with HTAD without systemic features have negative genetic testing; research referral may be appropriate.

Document clinical rationale and diagnostic evaluations in the medical record

Obtain and retain in the medical record disease-appropriate clinical documentation that supports the indication for testing, including diagnostic evaluations and rationale.

• Documentation should support medical necessity and be legible and available upon request.
• Include the rationale for the chosen test and any prior testing or results relevant to the decision.

Clinical documentation: include history, family history, and ECG features (Schwartz score when applicable)

Include clinical history, family history, and ECG characteristics (baseline, Holter, exercise testing) and, when applicable, diagnostic scores (e.g., Schwartz Score) to justify molecular genetic testing for index patients.

• Provide patient's clinical history and family history.
• Include ECG findings (baseline, Holter, exercise) and any diagnostic scoring used to establish high pre-test probability.

Document phenotype and diagnostic testing to justify gene selection

Ensure documentation supports the phenotype and pre-test probability (clinical history, family history, ECG including baseline, Holter and exercise testing) when ordering molecular genetic testing for index patients.

• Support the diagnostic probability (e.g., LQTS Schwartz Score ≥3.5, CPVT diagnostic score thresholds, SQTS diagnostic criteria).
• Document why targeted genes or panels were selected based on phenotype.

Family history documentation: obtain a three‑generation pedigree for HCM patients

For individuals with HCM, include a three-generation family history in the evaluation and record it in the medical record as part of the initial assessment.

• Three-generation pedigree is recommended per ACC/AHA guidance as part of the initial HCM assessment.
• Include relatives' ages, diagnoses, and any sudden/unexplained cardiac events.

Phenotyping requirement before testing — include three‑generation family history

Disease-appropriate phenotyping including a three-generation family history should be completed prior to ordering genetic testing.

• Perform focused cardiovascular phenotyping and create a pedigree before testing.
• Phenotyping should inform choice between targeted genes, panels, or broader testing.

Obtain multigenerational family history (≥3 generations) for primary cardiomyopathy patients

Obtain and document a family history of at least three generations, including a pedigree, for all patients with primary cardiomyopathy before testing.

• Pedigree should capture affected/unaffected relatives and ages at diagnosis or sudden death.
• Use family history to guide test selection and cascade planning.

Document multigenerational family history for thoracic aortic disease cases

Obtain and document a multigenerational family history of thoracic aortic disease, unexplained sudden deaths, and peripheral or intracranial aneurysms for individuals with aortic root/ascending aortic aneurysms or dissection.

• Multigenerational history guides HTAD testing decisions and panel gene selection (e.g., FBN1, TGFBR1/2, ACTA2).
• Document associated systemic features if present.

Use CLIA‑certified laboratories for genetic testing

Laboratories performing genetic tests are regulated under CLIA; providers should use CLIA‑certified laboratories for testing and may reference FDA listings for cleared/approved nucleic acid‑based tests (informational).

• CLIA regulation of clinical genetic labs is required.
• FDA approvals are informational only and not a basis for coverage.

Reference federal/state/contractual benefit rules when determining coverage

When deciding coverage, reference applicable federal, state, or contractual benefit requirements; these govern in case of conflict with this policy and determine prior authorization and coverage rules.

• This policy assists interpretation of UnitedHealthcare standard plans but does not replace plan terms.
• Check Idaho Medicaid Provider Handbook for non‑covered/excluded services when billing Idaho Medicaid members.

Denial risk: CAD genetic/gene‑expression tests are not medically necessary

Genetic testing for coronary artery disease (CAD), including gene expression tests and microarray/genetic profiles, is considered unproven and not medically necessary and may be denied.

• Examples include Cardiac DNA Insight, Cardio IQ gene tests, and mRNA gene expression profiling (81493).
• Do not expect coverage for CAD genetic or gene-expression risk tests under this policy.

Denial risk: large panels increase VUS frequency — justify broad panels

Avoid ordering very large/comprehensive gene panels unless clearly justified; large panels increase variants of unknown significance (VUS) frequency and may reduce clinical utility, risking unfavorable coverage decisions.

• Van Lint et al. showed larger panels increase VUS frequency and LP/P detection but may increase uncertain results.
• Document rationale if using broad panels and why targeted testing is not appropriate.

Denial risk: testing without diagnostic phenotype or ECG may be denied

Genetic testing without a diagnostic ECG or appropriate phenotype is not supported and may lead to denial; testing should be driven by phenotype and pre‑test probability.

• Pre‑test probability determines utility; identification of a variant alone rarely meets diagnostic criteria without phenotype.
• Ensure diagnostic ECG or other phenotype evidence is documented prior to testing.

Denial risk: avoid testing for isolated/incidental LVNC without clinical indicators

Do not order genetic testing for isolated (incidental) LVNC when left ventricular function is normal, there are no syndromic features and no family history, as such testing is not recommended and may be denied.

• Genetic testing may be useful only when LVNC diagnosis is established by cardiologist or when syndromic features/family history are present.

Denial risk: FDA approval alone is not a basis for coverage

FDA approval or clearance of a test is informational only and is not sufficient alone to establish coverage; laboratories must be CLIA‑regulated for clinical testing.

• Do not rely solely on FDA approval as the basis for coverage requests.
• Confirm the performing lab is CLIA‑certified.

Idaho Medicaid: listed CPT codes may not be covered — risk of denial

CPT codes 0237U, 0401U, 0439U, 0440U, 0466U, 0617U, and 81410 are not on the State of Idaho Medicaid Fee Schedule and therefore may not be covered by Idaho Medicaid; services billed with these codes may be denied by Idaho Medicaid.

• 0617U was added to the policy's applicable codes and flagged as not on Idaho Medicaid Fee Schedule.
• Consult the Idaho Medicaid Provider Handbook for details on non‑covered and excluded services.