J Korean Med Sci.  2024 Apr;39(13):e126. 10.3346/jkms.2024.39.e125.

Case 20: A 69-Year-Old Man With Exertional Dyspnea

• Mok JS
• Kim J
• Park CS

• KMID: 2554580
• DOI: http://doi.org/10.3346/jkms.2024.39.e125

Figure

• 

  Fig. 1 Transthoracic echocardiography. (A, B) The parasternal long-axis view revealed an interventricular septum thickness of 17 mm and a left ventricular posterior wall thickness of 17 mm. (C) Compared with the mid or basal regions, the left ventricular apical region exhibits a distinctive pattern with even more normal strain values (apical sparing or bull’s eye pattern).

• 

  Fig. 2 99mTc-PYP scintigraphy (HDP bone scan). Grade 3 tracer uptake on planar HDP bone scan. Cardiac uptake is greater than rib uptake, with mild/absent rib uptake.99mTc = 99m-technetium, PYP = pyrophosphate, HDP = hydroxymethylene diphosphonate.

• 

  Fig. 3 Myocardial biopsy. (A) Endomyocardial biopsy shows amorphous and homogeneous amyloid deposition between the muscle fibers (hematoxylin-eosin 400×). (B) Congo red staining shows positive staining (orange-red) of amorphous material (Congo red 400×). (C) Congo red staining shows apple-green birefringence under polarized light, diagnostic of amyloid (Congo red 400×).

Reference

1. Kittleson MM, Maurer MS, Ambardekar AV, Bullock-Palmer RP, Chang PP, Eisen HJ, et al. Cardiac amyloidosis: evolving diagnosis and management: a scientific statement from the American Heart Association. Circulation. 2020; 142(1):e7–22. PMID: 32476490.

2. Ruberg FL, Grogan M, Hanna M, Kelly JW, Maurer MS. Transthyretin amyloid cardiomyopathy: JACC State-of-the-Art Review. J Am Coll Cardiol. 2019; 73(22):2872–2891. PMID: 31171094.

3. Kharoubi M, Bézard M, Galat A, Le Bras F, Poullot E, Molinier-Frenkel V, et al. History of extracardiac/cardiac events in cardiac amyloidosis: prevalence and time from initial onset to diagnosis. ESC Heart Fail. 2021; 8(6):5501–5512. PMID: 34714605.

4. Griffin JM, Rosenthal JL, Grodin JL, Maurer MS, Grogan M, Cheng RK. ATTR amyloidosis: current and emerging management strategies: JACC: CardioOncology State-of-the-Art Review. JACC CardioOncol. 2021; 3(4):488–505. PMID: 34729521.