Cardiovascular Syphilis: Natural History, Manifestations, and Management

Cardiovascular syphilis (CVS) is a late manifestation of untreated Treponema pallidum infection, occurring in ~10% of untreated secondary syphilis cases, 10–30 years after primary infection.

Pathogenesis

• Treponemes invade aortic vasa vasorum → endarteritis obliterans → ischemia of aortic media → elastic fiber destruction, medial necrosis.
• Ascending aorta most affected (proximal vasa vasorum).
• Inflammatory infiltrate: Plasma cells, lymphocytes, obliterative endarteritis.

Clinical Stages

Early CVS (Syphilitic Aortitis)

• Often asymptomatic.
• May cause: Aortic root dilation, aortic regurgitation (AR), coronary ostial stenosis (angina/MI).
• ECG: PR prolongation, AV block (if extends to conduction system).

Late CVS (Destructive Phase)

• Aortic aneurysm: Ascending > arch > descending. Risk of rupture, dissection.
• Aortic regurgitation: Root dilation → leaflet malcoaptation → volume overload → LV dilation/failure. “Corrigan’s pulse,” “de Musset’s sign.”
• Coronary ostial stenosis: Fibrotic narrowing → angina, MI, sudden death.
• Heart failure: From AR, coronary stenosis, or myocardial gummas (rare).

Diagnosis

• Serology: RPR/VDRL (high titer) + confirmatory treponemal (TPPA, FTA-ABS). CSF VDRL if neurosyphilis suspected.
• Imaging: TTE/TEE (aortic root, AR, coronary ostia), CT/MRI aortography (aneurysm, dissection).
• Histology: Rarely needed; shows obliterative endarteritis, plasma cells.

Treatment

• Penicillin G benzathine 2.4 MU IM weekly × 3 weeks (late latent/tertiary).
• If penicillin allergic: Doxycycline 100 mg BID × 28 days OR desensitization.
• Jarisch-Herxheimer reaction common (fever, chills, hypotension) – premedicate with antipyretics.
• Surgery (valve replacement, aortic graft) for hemodynamic compromise – ideally after antimicrobial course.
• Follow-up: Clinical + serologic (RPR titer ↓ 4-fold at 12–24 mo).

Prevention

• Early detection/treatment of primary/secondary syphilis.
• Partner notification and treatment.
• Congenital syphilis prevention (prenatal screening).

CV syphilis is preventable and treatable. Late complications are largely irreversible; early penicillin halts progression.