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Cardiac amyloidosis — RCPSC IM MCQ

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HardCardiologyCardiac amyloidosisRCPSC IM

A 73-year-old man has progressive exertional dyspnea, bilateral carpal tunnel syndrome and orthostatic dizziness. ECG shows low limb-lead voltage; echocardiography shows concentric left ventricular thickening with preserved ejection fraction and apical sparing strain. Serum free light chains are normal and immunofixation is negative. What is the most likely diagnosis?

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Correct answer: E — Transthyretin cardiac amyloidosis

Heart failure with preserved ejection fraction, increased wall thickness, low ECG voltage, apical sparing strain and carpal tunnel syndrome suggests cardiac amyloidosis. Negative monoclonal protein studies make transthyretin amyloidosis more likely than AL amyloidosis.

Reference: Canadian Cardiovascular Society cardiac amyloidosis position statement