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v1.0.0
ICD-10 Guide
ICD-10 CodesQ26.2

Q26.2

Billable

Total anomalous pulmonary venous connection

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

Code Description

ICD-10 Q26.2 is a billable code used to indicate a diagnosis of total anomalous pulmonary venous connection.

Key Diagnostic Point:

Total anomalous pulmonary venous connection (TAPVC) is a congenital heart defect characterized by the abnormal drainage of all four pulmonary veins into the right atrium instead of the left atrium. This condition leads to a lack of oxygenated blood entering the systemic circulation, resulting in cyanosis and respiratory distress in affected infants. TAPVC can be classified into four types: supracardiac, cardiac, infracardiac, and mixed, based on the anatomical location of the anomalous veins. Clinical presentation typically includes tachypnea, retractions, and a heart murmur. Diagnosis is confirmed through echocardiography, which visualizes the abnormal venous connections. Surgical intervention is often required to correct the defect, and early diagnosis is crucial for improving outcomes. The condition is often associated with other congenital heart defects, such as atrial septal defects or ventricular septal defects, necessitating comprehensive evaluation and management.

Code Complexity Analysis

Complexity Rating: High

High Complexity

Complexity Factors

  • Variability in anatomical types of TAPVC
  • Potential for associated congenital heart defects
  • Need for precise documentation of surgical interventions
  • Complexity of diagnostic imaging interpretations

Audit Risk Factors

  • Inaccurate documentation of associated congenital defects
  • Failure to specify the type of TAPVC
  • Misclassification of severity or urgency of the condition
  • Inadequate linkage between diagnosis and surgical procedures

Specialty Focus

Medical Specialties

Pediatrics

Documentation Requirements

Detailed clinical notes on symptoms, diagnostic imaging results, and treatment plans.

Common Clinical Scenarios

Infants presenting with respiratory distress and cyanosis, requiring urgent evaluation.

Billing Considerations

Consideration of age and weight for surgical risk assessment and management.

Genetics

Documentation Requirements

Family history of congenital heart defects and genetic counseling notes.

Common Clinical Scenarios

Assessment of genetic syndromes associated with congenital heart defects.

Billing Considerations

Evaluation for chromosomal abnormalities that may accompany TAPVC.

Coding Guidelines

Inclusion Criteria

Use Q26.2 When
  • Follow official ICD
  • CM guidelines for coding congenital malformations, ensuring accurate documentation of the condition's specifics and any associated anomalies

Exclusion Criteria

Do NOT use Q26.2 When
No specific exclusions found.

Related ICD-10 Codes

Related CPT Codes

33533CPT Code

Repair of total anomalous pulmonary venous connection

Clinical Scenario

Used during surgical intervention for TAPVC correction.

Documentation Requirements

Operative report detailing the procedure and any complications.

Specialty Considerations

Pediatric cardiology and cardiothoracic surgery documentation standards.

ICD-10 Impact

Diagnostic & Documentation Impact

Enhanced Specificity

ICD-10 Improvements

The transition to ICD-10 has allowed for more specific coding of TAPVC, improving the accuracy of data collection and reimbursement processes.

ICD-9 vs ICD-10

The transition to ICD-10 has allowed for more specific coding of TAPVC, improving the accuracy of data collection and reimbursement processes.

Reimbursement & Billing Impact

reimbursement processes.

Resources

Clinical References

  • •
    American Academy of Pediatrics - Congenital Heart Disease

Coding & Billing References

  • •
    American Academy of Pediatrics - Congenital Heart Disease

Frequently Asked Questions

What is the significance of coding the specific type of TAPVC?

Coding the specific type of TAPVC is crucial for accurate treatment planning, surgical intervention, and understanding the prognosis, as each type may have different implications for management and outcomes.