E85.82

HCC Category Mapping

What This Code Means

A hereditary disease where abnormal transthyretin protein accumulates in the body, particularly affecting the heart and nerves. This is the most common form of hereditary amyloidosis.

Coding Tips

• •Wild-type ATTR (formerly called senile systemic amyloidosis) occurs in elderly patients without genetic mutations
• •Document whether this is hereditary or wild-type variant, as treatment approaches differ significantly

Clinical Significance

Wild-type ATTR amyloidosis (formerly senile cardiac amyloidosis) is an increasingly recognized cause of heart failure in elderly patients, caused by age-related misfolding of normal transthyretin protein. The availability of disease-modifying therapy (tafamidis) has made accurate diagnosis and coding essential for treatment access.

Documentation Requirements

• ✓Confirmed ATTR amyloidosis diagnosis (cardiac MRI, technetium pyrophosphate scan, or tissue biopsy)
• ✓Documentation that this is WILD-TYPE (not hereditary/mutant) ATTR — negative TTR gene testing
• ✓Cardiac involvement assessment (echocardiography, cardiac biomarkers, NT-proBNP)
• ✓Functional status and NYHA class
• ✓Current treatment (tafamidis, heart failure management)
• ✓Exclusion of hereditary ATTR by genetic testing

Commonly Confused Codes