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v1.0.0
ICD-10 Guide
ICD-10 CodesQ21.3

Q21.3

Billable

Tetralogy of Fallot

BILLABLE STATUSYes
IMPLEMENTATION DATEOctober 1, 2015
LAST UPDATED09/11/2025

Code Description

ICD-10 Q21.3 is a billable code used to indicate a diagnosis of tetralogy of fallot.

Key Diagnostic Point:

Tetralogy of Fallot (ToF) is a complex congenital heart defect characterized by four specific anatomical abnormalities: ventricular septal defect (VSD), pulmonary stenosis, right ventricular hypertrophy, and an overriding aorta. This condition leads to inadequate oxygenation of blood, resulting in cyanosis, particularly during exertion or crying in infants. The severity of symptoms can vary widely, with some infants presenting with significant cyanosis at birth, while others may remain asymptomatic for a period. Diagnosis is typically made through echocardiography, which reveals the structural defects, and may be supplemented by cardiac MRI or catheterization for further assessment. Surgical intervention is often required, usually within the first year of life, to repair the defects and improve blood flow. Long-term follow-up is essential, as patients may develop complications such as arrhythmias, pulmonary regurgitation, or heart failure as they age. Management of Tetralogy of Fallot requires a multidisciplinary approach, including pediatric cardiology, cardiothoracic surgery, and ongoing care into adulthood.

Code Complexity Analysis

Complexity Rating: High

High Complexity

Complexity Factors

  • Multiple anatomical defects requiring detailed documentation
  • Variability in clinical presentation and severity
  • Need for surgical and non-surgical management coding
  • Potential for associated chromosomal abnormalities

Audit Risk Factors

  • Inaccurate documentation of associated anomalies
  • Failure to capture surgical interventions
  • Misclassification of severity or type of defect
  • Inadequate follow-up documentation

Specialty Focus

Medical Specialties

Pediatrics

Documentation Requirements

Detailed clinical notes on symptoms, diagnostic tests, and treatment plans are essential. Documentation should include growth and development assessments, as well as any interventions performed.

Common Clinical Scenarios

Infants presenting with cyanosis, failure to thrive, or heart murmurs; pre-operative assessments; and post-operative follow-ups.

Billing Considerations

Ensure accurate coding of any associated congenital anomalies and the timing of surgical interventions.

Genetics

Documentation Requirements

Genetic counseling notes, family history, and any genetic testing results should be documented to assess for syndromic associations.

Common Clinical Scenarios

Patients with suspected genetic syndromes presenting with ToF; counseling for families with a history of congenital heart defects.

Billing Considerations

Consider the implications of chromosomal abnormalities such as 22q11.2 deletion syndrome, which is commonly associated with ToF.

Coding Guidelines

Inclusion Criteria

Use Q21.3 When
  • Follow the official ICD
  • CM guidelines for coding congenital malformations, ensuring that all associated conditions and complications are accurately captured
  • Documentation must support the diagnosis and any procedures performed

Exclusion Criteria

Do NOT use Q21.3 When
No specific exclusions found.

Related ICD-10 Codes

Related CPT Codes

33641CPT Code

Repair of Tetralogy of Fallot

Clinical Scenario

Used during surgical intervention for ToF repair.

Documentation Requirements

Operative reports detailing the surgical procedure and any complications.

Specialty Considerations

Pediatric cardiothoracic surgery documentation must be thorough to support the procedure.

ICD-10 Impact

Diagnostic & Documentation Impact

Enhanced Specificity

ICD-10 Improvements

The transition to ICD-10 has allowed for more specific coding of congenital heart defects, including Tetralogy of Fallot, which enhances the ability to capture the complexity of these conditions and their management.

ICD-9 vs ICD-10

The transition to ICD-10 has allowed for more specific coding of congenital heart defects, including Tetralogy of Fallot, which enhances the ability to capture the complexity of these conditions and their management.

Reimbursement & Billing Impact

The transition to ICD-10 has allowed for more specific coding of congenital heart defects, including Tetralogy of Fallot, which enhances the ability to capture the complexity of these conditions and their management.

Resources

Clinical References

  • •
    American Academy of Pediatrics - Congenital Heart Disease

Coding & Billing References

  • •
    American Academy of Pediatrics - Congenital Heart Disease

Frequently Asked Questions

What are the common associated conditions with Tetralogy of Fallot?

Common associated conditions include pulmonary atresia, aortic arch anomalies, and chromosomal abnormalities such as 22q11.2 deletion syndrome. Accurate documentation of these associations is crucial for coding.