By Counterforce Health Team in Uptravi (selexipag) —

Coding That Helps Get Uptravi (selexipag) Approved by Aetna (CVS Health) in North Carolina: ICD-10, J-Codes & NDC Requirements

Answer Box

Getting Uptravi (selexipag) covered by Aetna (CVS Health) in North Carolina requires precise coding and comprehensive documentation. Use ICD-10 codes I27.0 (primary PAH) or I27.21 (secondary PAH) with WHO Group I confirmation via right heart catheterization. Bill IV formulations using J3490 + NDC on medical benefit; oral forms use NDC on pharmacy benefit. Step 1: Ensure prescriber is a pulmonologist/cardiologist. Step 2: Document failure of 2+ oral PAH therapies. Step 3: Submit complete prior authorization with hemodynamic data and functional class. If denied, appeal internally within 180 days, then use North Carolina's Smart NC external review within 120 days.

Table of Contents

  1. Coding Basics: Medical vs. Pharmacy Benefit Paths
  2. ICD-10 Mapping for PAH Documentation
  3. Product Coding: HCPCS, J-Codes, and NDC Requirements
  4. Clean Request Anatomy: Building Your Prior Authorization
  5. Frequent Coding Pitfalls to Avoid
  6. Verification Steps with Aetna (CVS Health)
  7. Quick Audit Checklist
  8. Appeals Playbook for North Carolina
  9. FAQ: Common Coding Questions

Coding Basics: Medical vs. Pharmacy Benefit Paths

Uptravi (selexipag) can be billed through different benefit pathways depending on the formulation and administration method. Understanding which path to use is crucial for successful reimbursement.

Medical Benefit (Buy-and-Bill)

  • Injectable/IV Uptravi: Billed using HCPCS code J3490 (unclassified drugs) plus the specific NDC
  • Requires documentation of dose administered and any wastage
  • Subject to JW/JZ modifier requirements for single-dose vials
  • Typically administered in clinic or hospital settings

Pharmacy Benefit

  • Oral Uptravi tablets: Billed directly through CVS Caremark using NDC codes
  • Subject to quantity limits (typically 140 tablets per 28 days for 200 mcg strength)
  • Requires prior authorization through pharmacy benefit management
Note: Most Uptravi prescriptions are for oral tablets dispensed through specialty pharmacies like CVS Specialty, making pharmacy benefit the primary pathway.

ICD-10 Mapping for PAH Documentation

Accurate diagnosis coding is essential for Aetna (CVS Health) approval. The ICD-10 codes must reflect WHO Group I pulmonary arterial hypertension with supporting clinical documentation.

Primary ICD-10 Codes

ICD-10 Code Description When to Use Documentation Required
I27.0 Primary pulmonary hypertension Idiopathic, heritable, or familial PAH Right heart catheterization showing mPAP >20 mmHg, PCWP ≤15 mmHg
I27.21 Secondary pulmonary arterial hypertension PAH due to drugs, HIV, connective tissue disease, congenital heart disease Same hemodynamic criteria plus documentation of underlying cause
I27.20 Pulmonary hypertension, unspecified Only when specific group unknown Avoid if WHO Group I is confirmed

Supporting Documentation Keywords

Aetna (CVS Health) looks for specific clinical language in your documentation. Include these terms when appropriate:

  • "WHO Group I pulmonary arterial hypertension"
  • "Right heart catheterization confirmed"
  • "Functional Class II/III/IV symptoms"
  • "Failed conventional PAH therapies"
  • "Contraindication to standard treatments"

Product Coding: HCPCS, J-Codes, and NDC Requirements

Injectable Uptravi Coding

HCPCS Code: J3490 (Unclassified drugs)

  • No unique J-code exists for Uptravi as of 2025
  • Must include specific NDC and dosage information
  • Bill per vial administered (1800 mcg per single-dose vial)

Required Modifiers:

  • JW: Applied to wasted drug amount (document both administered and wasted portions)
  • JZ: Applied when entire vial administered with zero waste
  • Mandatory since October 2023 - claims without JW/JZ modifiers will be denied

NDC Requirements

All Uptravi formulations require specific NDC codes on claims:

Oral Tablets (Pharmacy Benefit):

  • 200 mcg: NDC varies by manufacturer packaging
  • 400 mcg, 600 mcg, 800 mcg, 1000 mcg, 1200 mcg, 1400 mcg, 1600 mcg strengths available

Injectable (Medical Benefit):

  • Single-dose vial: Include exact NDC with J3490 claims
Tip: Always verify current NDC numbers with your pharmacy or the FDA Orange Book before submitting claims.

Clean Request Anatomy: Building Your Prior Authorization

A successful Aetna (CVS Health) prior authorization for Uptravi requires these essential components:

Required Clinical Elements

  1. Prescriber Qualification
    • Must be pulmonologist or cardiologist
    • Include NPI and specialty designation
  2. Diagnosis Documentation
    • ICD-10 code (I27.0 or I27.21)
    • Right heart catheterization results showing:
      • Mean pulmonary artery pressure >20 mmHg
      • Pulmonary capillary wedge pressure ≤15 mmHg
      • Pulmonary vascular resistance ≥3 Wood units
  3. Functional Class Assessment
    • Current WHO/NYHA functional class (II-IV typically required)
    • 6-minute walk test results if available
    • Documentation of activity limitations
  4. Prior Therapy History
    • Trial of at least 2 different oral PAH therapy classes for ≥60 days each:
      • PDE-5 inhibitors (sildenafil, tadalafil)
      • Endothelin receptor antagonists (ambrisentan, bosentan, macitentan)
      • Soluble guanylate cyclase stimulators (riociguat)
    • Documentation of failure, intolerance, or contraindications

Sample Documentation Framework

Patient: [Name], DOB [Date]
Diagnosis: Pulmonary arterial hypertension, WHO Group I (ICD-10: I27.0)

Hemodynamic Confirmation:
- Right heart catheterization [Date]: mPAP 45 mmHg, PCWP 12 mmHg, PVR 8 Wood units
- Current functional class: III
- 6-minute walk distance: 280 meters

Prior Therapies:
1. Sildenafil 20mg TID x 90 days - discontinued due to inadequate response
2. Ambrisentan 5mg daily x 120 days - discontinued due to peripheral edema
3. Current: Bosentan 125mg BID - stable but limited improvement

Request: Uptravi (selexipag) 200 mcg BID with titration per FDA labeling
Rationale: Add-on prostacyclin pathway therapy per current PAH guidelines

Frequent Coding Pitfalls to Avoid

Common Mistakes That Trigger Denials

  1. Wrong ICD-10 Selection
    • Using I27.20 (unspecified) instead of I27.0 or I27.21
    • Missing secondary diagnosis codes for underlying conditions
  2. Incomplete Prior Authorization
    • Missing hemodynamic data
    • Inadequate documentation of prior therapy failures
    • Wrong prescriber specialty
  3. Billing Errors
    • Missing NDC on J3490 claims
    • Incorrect unit calculations
    • Omitting mandatory JW/JZ modifiers
  4. Documentation Gaps
    • No functional class assessment
    • Missing right heart catheterization results
    • Inadequate trial duration for prior therapies

Unit Conversion Issues

Injectable Dosing Math:

  • Each vial contains 1800 mcg
  • Reconstituted concentration: 225 mcg/mL
  • Bill actual dose administered, not vial quantity
  • Document any wastage for JW modifier

Verification Steps with Aetna (CVS Health)

Before submitting your prior authorization, verify these key elements:

Pre-Submission Checklist

  1. Check Current Formulary Status
    • Log into Aetna provider portal
    • Verify Uptravi tier placement and restrictions
    • Confirm quantity limits for prescribed strength
  2. Validate Prior Authorization Form
    • Use current Aetna/CVS Caremark PA form
    • Ensure all required fields completed
    • Include supporting clinical documents
  3. Confirm Submission Method
    • Electronic: Through Aetna provider portal
    • Fax: Verify current PA fax number
    • Phone: Use for urgent/expedited requests
  4. Review Plan-Specific Requirements
    • Some Aetna plans have additional restrictions
    • Medicare Advantage may have different criteria
    • Medicaid managed care plans follow state guidelines

Quick Audit Checklist

Use this checklist before submitting any Uptravi prior authorization:

Clinical Documentation ✓

  • ICD-10 code matches WHO Group I PAH diagnosis
  • Right heart catheterization results included
  • Functional class documented
  • Prior therapy trials documented (≥2 classes, ≥60 days each)
  • Prescriber is pulmonologist or cardiologist

Coding Accuracy ✓

  • Correct NDC for prescribed formulation
  • Appropriate HCPCS code (J3490 for injectable)
  • JW/JZ modifiers applied when required
  • Units match actual dosing

Administrative Details ✓

  • Current PA form used
  • All required fields completed
  • Supporting documents attached
  • Submission method confirmed

Appeals Playbook for North Carolina

When Aetna (CVS Health) denies your Uptravi request, North Carolina offers robust appeal rights through a two-tier system.

Internal Appeals (Aetna)

Timeline: File within 180 days of denial Decision: 30-45 days (standard), 72 hours (expedited) How to File: Aetna member portal, phone, or written request

Required Documents:

  • Original denial letter
  • Medical records supporting necessity
  • Provider letter addressing denial reasons
  • Any new clinical information

External Review (Smart NC)

North Carolina's external review program provides binding decisions when internal appeals fail.

Eligibility:

  • State-regulated plan (not self-insured ERISA plans)
  • Medical necessity denial
  • Cost exceeds $500 (automatically met for Uptravi)
  • Internal appeals exhausted

Timeline: File within 120 days of final internal denial Decision: 45 days (standard), 72 hours (expedited) Cost: Free to patient Contact: Smart NC at 1-855-408-1212

From our advocates: "We've seen several PAH medication denials overturned through North Carolina's external review when comprehensive hemodynamic data and guideline-based medical necessity letters were submitted. The key is demonstrating that Uptravi fills a specific therapeutic gap that other PAH therapies haven't addressed."

When to Request Expedited Review

Request expedited processing if:

  • Patient's condition is rapidly deteriorating
  • Delay would seriously jeopardize health or function
  • Current therapy failing with urgent need for alternative

At Counterforce Health, we specialize in turning insurance denials into successful appeals by analyzing denial letters, plan policies, and clinical notes to create targeted, evidence-backed responses. Our platform identifies the specific denial basis and drafts point-by-point rebuttals aligned to each payer's requirements, pulling the right citations and clinical facts to support your case. Learn more about our approach.

FAQ: Common Coding Questions

Q: How long does Aetna (CVS Health) prior authorization take for Uptravi in North Carolina? A: Standard decisions take 30-45 days. Expedited requests (with physician certification of urgency) receive decisions within 72 hours.

Q: What if Uptravi is non-formulary on my Aetna plan? A: Request a formulary exception with documentation showing medical necessity and failure of formulary alternatives. The same clinical criteria apply.

Q: Can I appeal if I've tried PAH therapies outside North Carolina? A: Yes, prior therapy history from other states counts toward step therapy requirements. Include complete medical records and prescription history.

Q: Does CVS Specialty handle Uptravi differently than other pharmacies? A: CVS Specialty follows the same prior authorization requirements but may offer additional support services for specialty medications like Uptravi.

Q: What happens if my appeal is denied by Smart NC? A: Smart NC decisions are binding on insurance companies. If denied, you've exhausted administrative remedies, though other legal options may exist.

Q: Do I need a specific diagnosis code for functional class? A: No separate ICD-10 code exists for functional class. Document it clearly in clinical notes alongside the primary PAH diagnosis code.

Q: Can nurse practitioners prescribe Uptravi for Aetna coverage? A: Aetna typically requires prescribing by or consultation with a pulmonologist or cardiologist. Check specific plan requirements, as some may accept NP prescribing with specialist oversight.

Q: How do I find my plan's current prior authorization form? A: Log into the Aetna provider portal or call the prior authorization phone line. Forms are updated regularly, so always use the current version.

Sources & Further Reading

Disclaimer: This information is for educational purposes only and does not constitute medical or legal advice. Always consult with qualified healthcare professionals and verify current payer policies before making treatment decisions. Insurance policies and requirements change frequently - confirm all details with your specific plan.

If you need help navigating insurance denials or building stronger appeals, Counterforce Health provides comprehensive support for patients, clinicians, and pharmacies seeking prescription drug approvals through targeted, evidence-backed appeal strategies.

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