A 62-year-old man presents with progressive dyspnea, bilateral pedal edema, and ascites. Jugular venous pressure is elevated with a rapid y descent. Echocardiogram shows biatrial enlargement, normal LVEF of 60%, normal ventricular wall thickness, and a "sparkling" appearance of the myocardium. Cardiac MRI demonstrates diffuse subendocardial late gadolinium enhancement. Which is the most likely diagnosis?

• A Cardiac amyloidosis (AL type) ✓
• B Constrictive pericarditis
• C Sarcoidosis with pulmonary hypertension
• D Restrictive cardiomyopathy due to hemochromatosis

Explanation

Normal LVEF with diastolic dysfunction, biatrial enlargement, sparkling myocardium on echo, and diffuse subendocardial late gadolinium enhancement on MRI are classic for cardiac amyloidosis. The rapid y descent reflects elevated atrial pressure with impaired ventricular filling. Constrictive pericarditis can mimic this but shows pericardial thickening or calcification on imaging and does not show subendocardial late gadolinium enhancement diffusely. Hemochromatosis causes dilated or restrictive pattern but with different MRI findings (T2* shortening).

Reference: Braunwald's Heart Disease, 12th ed.

High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP

Written and medically reviewed by the StethoPrep medical team.