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v1.0.0
ICD-10 Guide
ICD-10 CodesQ87.41

Q87.41

Billable

Marfan syndrome with cardiovascular manifestations

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

Code Description

ICD-10 Q87.41 is a billable code used to indicate a diagnosis of marfan syndrome with cardiovascular manifestations.

Key Diagnostic Point:

Marfan syndrome is a genetic disorder that affects the body's connective tissue, leading to a variety of symptoms and complications. The cardiovascular manifestations of Marfan syndrome are particularly significant and can include aortic dilation, aortic regurgitation, mitral valve prolapse, and other structural heart defects. These cardiovascular issues arise due to the weakening of the connective tissue that supports the heart and blood vessels. Patients may present with symptoms such as chest pain, palpitations, or even syncope due to arrhythmias or aortic dissection. Early diagnosis and management are crucial to prevent life-threatening complications. Regular monitoring through echocardiograms and appropriate medical or surgical interventions are essential components of care for individuals with Marfan syndrome. The condition is inherited in an autosomal dominant pattern, and genetic counseling is often recommended for affected individuals and their families.

Code Complexity Analysis

Complexity Rating: Medium

Medium Complexity

Complexity Factors

  • Variability in clinical presentation and severity of symptoms
  • Need for comprehensive documentation of cardiovascular assessments
  • Potential for co-existing congenital conditions
  • Requirement for genetic testing results to support diagnosis

Audit Risk Factors

  • Inadequate documentation of cardiovascular manifestations
  • Failure to include genetic testing results
  • Misclassification of related congenital conditions
  • Inconsistent follow-up documentation

Specialty Focus

Medical Specialties

Pediatrics

Documentation Requirements

Pediatric documentation should include growth parameters, developmental milestones, and specific cardiovascular assessments.

Common Clinical Scenarios

Pediatric patients presenting with chest pain or family history of Marfan syndrome requiring evaluation.

Billing Considerations

Consideration of age-related manifestations and the impact of growth on cardiovascular health.

Genetics

Documentation Requirements

Documentation must include family history, genetic testing results, and counseling notes.

Common Clinical Scenarios

Patients undergoing genetic counseling for Marfan syndrome and related cardiovascular risks.

Billing Considerations

Understanding the implications of genetic inheritance and the need for family screening.

Coding Guidelines

Inclusion Criteria

Use Q87.41 When
  • Follow the official ICD
  • CM guidelines for coding congenital conditions, ensuring accurate documentation of all manifestations and related conditions
  • Use additional codes as necessary to capture the full clinical picture

Exclusion Criteria

Do NOT use Q87.41 When
No specific exclusions found.

Related ICD-10 Codes

Related CPT Codes

93306CPT Code

Echocardiography, transthoracic, real-time with interpretation and report

Clinical Scenario

Used for monitoring cardiovascular manifestations in patients with Marfan syndrome.

Documentation Requirements

Document the reason for the echocardiogram, including symptoms and family history.

Specialty Considerations

Pediatric cardiology may require additional details on growth and development.

ICD-10 Impact

Diagnostic & Documentation Impact

Enhanced Specificity

ICD-10 Improvements

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

Reimbursement & Billing Impact

reimbursement processes.

Resources

Clinical References

  • •
    Marfan Foundation

Coding & Billing References

  • •
    Marfan Foundation

Frequently Asked Questions

What are the key symptoms of Marfan syndrome that should be documented?

Key symptoms include tall stature, long limbs, arachnodactyly, cardiovascular issues such as aortic dilation, and ocular problems like lens dislocation. Comprehensive documentation of these symptoms is essential for accurate coding.