ICD-10 Code A50.54 – Late congenital cardiovascular syphilis (2026): Diagnosis, Symptoms & Billing Guide

The ICD-10 code for Late congenital cardiovascular syphilis is A50.54.
2026 ICD-10-CM Diagnosis Code A50.54 – Late congenital cardiovascular syphilis

What it is

A50.54 identifies late congenital syphilis affecting the cardiovascular system. Use it when congenital infection has caused documented heart or great-vessel involvement later in life.

Clinical signs

Clinical features vary; refer to documentation. Findings may include congenital syphilitic cardiovascular disease, such as aortic involvement or other structural cardiac abnormalities attributed to congenital syphilis.

When to use this code

Use this code when the provider documents late congenital syphilis with cardiovascular manifestations. It is appropriate for records linking the heart or great vessels to congenital treponemal infection. If the note only says congenital syphilis without cardiac involvement, check documentation.

Do not use for

Do not use this code for acquired syphilis or for congenital syphilis without cardiovascular disease. If the cardiovascular problem is unrelated to congenital syphilis, code the documented condition instead.

Coding tip

Confirm both “late congenital syphilis” and cardiovascular involvement are explicitly documented before assigning A50.54.

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