Q87.40

Billable

Marfan syndrome, unspecified

HCC Category Mapping

RxHCCHCC 84 — Vasculitis and Other Autoimmune Disorders

0.000

What This Code Means

Marfan syndrome is a genetic disorder affecting connective tissue that causes tall stature, long limbs, and potential heart and eye problems, without specification of which body systems are involved.

Coding Tips

•Use this code only when the specific manifestations of Marfan syndrome are not documented; otherwise use more specific codes Q87.41-Q87.43
•Confirm genetic testing or clinical diagnostic criteria are documented to support Marfan syndrome diagnosis

Clinical Significance

Marfan syndrome is a serious connective tissue disorder that requires lifelong monitoring and management due to potential life-threatening cardiovascular complications. This unspecified code indicates the presence of the syndrome but lacks specificity about which organ systems are affected, limiting optimal risk stratification.

Documentation Requirements

✓Genetic testing results or clinical diagnosis of Marfan syndrome
✓Family history of Marfan syndrome or related connective tissue disorders
✓Physical examination findings consistent with Marfan features
✓Ophthalmologic evaluation results
✓Cardiovascular assessment including echocardiogram findings
✓Skeletal measurements and deformities
✓Documentation of affected organ systems
✓Provider's clinical assessment and diagnostic reasoning

Commonly Confused Codes

Q87.410 — Use when aortic dilation is specifically documented Q87.418 — Use when other cardiovascular manifestations are present Q87.42 — Use when ocular manifestations are documented Q87.43 — Use when skeletal manifestations are present M35.9 — Systemic connective tissue disorder, unspecified (acquired vs congenital)

Code Hierarchy

└Q87Other specified congenital malformation syndromes affecting multiple systems └Q87.4Marfan syndrome └Q87.40Marfan syndrome, unspecified

└Q87.40Marfan syndrome, unspecified