Medicine · Heart Failure and Cardiomyopathies

A 68-year-old man presents with progressive exertional dyspnea and bilateral ankle edema. Echo shows concentric LV wall thickening (IVS 16 mm) with a preserved EF of 55% and a restrictive filling pattern. ECG shows low voltage limb leads. He underwent bilateral carpal tunnel release five years ago. Which investigation is most likely to establish the diagnosis?

  • A 99mTc-pyrophosphate (PYP) scintigraphy
  • B Coronary angiography
  • C Transoesophageal echocardiography
  • D Cardiac MRI with late gadolinium enhancement only
Correct answer: A. 99mTc-pyrophosphate (PYP) scintigraphy

Explanation

The combination of concentric LV hypertrophy with discordantly low ECG voltage, diastolic dysfunction, and prior carpal tunnel syndrome strongly suggests cardiac amyloidosis, particularly wild-type transthyretin (ATTR) amyloidosis. Bone tracer scintigraphy with 99mTc-PYP avidly binds transthyretin deposits, and a grade 2-3 uptake with negative serum free light chains establishes ATTR without biopsy. Angiography and TOE add nothing diagnostic here, and MRI alone cannot identify the amyloid subtype.

Reference: Harrison's Principles of Internal Medicine, 21st ed.

High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP

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