An uncommon case of complete AV block

Area of Science:

• Cardiology
• Rheumatology
• Systemic Inflammatory Disorders

Background:

• Granulomatosis with polyangiitis (GPA) is a rare systemic vasculitis affecting the respiratory tract and kidneys.
• Cardiac involvement in GPA is uncommon, often presenting late and potentially life-threatening.
• Conduction system abnormalities are a rare but severe complication of GPA.

Observation:

• A 45-year-old male with GPA presented with complete atrioventricular (AV) block at disease onset.
• Echocardiogram showed no abnormalities, but cardiac magnetic resonance (CMR) revealed ventricular wall inflammation.
• The patient required a permanent pacemaker due to recurrent complete AV block despite immunosuppressive therapy.

Findings:

• GPA can manifest with significant cardiac conduction abnormalities, including complete AV block.
• Inflammation of the cardiac conduction system, potentially due to granulomatous lesions or arteritis, underlies these abnormalities.
• Cardiac magnetic resonance (CMR) is valuable in detecting subclinical cardiac inflammation in GPA.

Implications:

• Regular cardiac screening (ECG, echocardiogram, CMR) is essential for early detection of cardiac involvement in GPA patients.
• Prompt immunosuppressive therapy may improve or reverse conduction abnormalities.
• Pacemaker implantation may be necessary for managing persistent or recurrent AV block in GPA.