A 68-year-old man has exertional dyspnea with preserved LVEF. Echocardiography shows concentrically thickened ventricular walls with a granular sparkling texture. ECG shows low-voltage complexes in the limb leads. Which diagnosis explains this combination best?
- A Hypertrophic cardiomyopathy
- B Severe aortic stenosis
- C Fabry disease
- D Cardiac amyloidosis ✓
Explanation
The classic clue in cardiac amyloidosis is discordance between thick walls on echocardiography and low voltage on ECG, because infiltrated myocytes are replaced electrically by amyloid fibrils, dampening surface potentials. Hypertrophic cardiomyopathy produces thick walls with normal or tall voltages, which is exactly what kills that distractor. The granular sparkling appearance results from amyloid deposits scattering ultrasound within the myocardium.
Reference: Harrison's Principles of Internal Medicine, 21st ed.
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Written and medically reviewed by the StethoPrep medical team.