A 62-year-old man presents with 6 months of progressive exertional dyspnea and bilateral ankle swelling. Blood pressure is 110/70 mmHg. ECG shows low-voltage complexes in the limb leads. Echocardiogram shows concentric left ventricular wall thickening (interventricular septum 16 mm) with a granular sparkling texture, a small pericardial effusion, and preserved systolic function. What is the most likely diagnosis?
- A Hypertensive heart disease
- B Constrictive pericarditis
- C Cardiac amyloidosis ✓
- D Hypertrophic cardiomyopathy
Explanation
The combination of thickened ventricular walls on echo with disproportionately low ECG voltages is the classic electrocardiographic-echocardiographic discordance of cardiac amyloidosis, where infiltrating amyloid fibrils thicken walls but dampen electrical signals. A granular sparkling texture and small effusion support it. Hypertensive heart disease produces wall thickening but with voltage appropriate to mass. Hypertrophic cardiomyopathy shows asymmetric hypertrophy, not concentric infiltration. Constrictive pericarditis gives normal wall thickness with elevated filling pressures.
Reference: Braunwald's Heart Disease: A Textbook of Cardiovascular Medicine, 12th ed.
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