Cardiac amyloidosis: From diagnosis to therapeutics breakthroughs

Figure 1 Strain apical sparing[24]. Citation: Gil J, Abreu L, Antunes H, Gonçalves ML, Pires MI, Moreira D, Correia E, Santos LS, Cabral JC. Apical sparing of longitudinal strain in speckle-tracking echocardiography: A sensitive and specific finding in cardiac amyloidosis. Neth Heart J 2018; 26: 635. Copyright© The Authors 2018. Published by Springer Nature. The article is open access (Supplementary material).

Figure 2 Patient diagnosed with endomyocardial biopsy. A: T1 native mapping demonstrates abnormally high values in all entire left ventricle (LV) walls in cardiac amyloidosis; B: Diffuse transmural LV and subendocardial right ventricle (RV) late gadolinium enhancement (LGE) in two-chamber short; C: Bulls-eye map of T1 native; D: Diffuse transmural LV and subendocardial RV LGE in long axes[26]. Citation: Maggialetti N, Torrente A, Lorusso G, Villanova I, Ficco M, Gravina M, Ferrari C, Giordano L, Granata V, Rubini D, Lucarelli NM, Stabile Ianora AA, Scardapane A. Role of Cardiovascular Magnetic Resonance in Cardiac Amyloidosis: A Narrative Review. J Pers Med 2024; 14: 407. Copyright© The Authors 2024. Published by MDPI. The article is open access (Supplementary material).

Figure 3 Planar 99mTc-DPD scintigraphies of four different patients. A: No cardiac uptake (Perugini score 0); B: Light cardiac uptake with preserved delineation of bone tissue (Perugini score 1); C: Strong cardiac uptake above that of bone tissue and increased soft-tissue uptake, particularly in the shoulder, abdominal wall, and gluteal region (Perugini score 2); D: Strong cardiac and soft-tissue uptake with no discernable bone-tissue uptake, suggesting difuse amyloid soft-tissue infltration (Perugini score 3); E: Single-photon emission computed tomography imaging: Short axis, vertical long axis, and horizontal long axis slices at the top and the corresponding polar plot at the bottom; white/yellow indicates high uptake; blue/black indicates little or no uptake[38]. Citation: Yilmaz A, Bauersachs J, Bengel F, Büchel R, Kindermann I, Klingel K, Knebel F, Meder B, Morbach C, Nagel E, Schulze-Bahr E, Aus dem Siepen F, Frey N. Diagnosis and treatment of cardiac amyloidosis: Position statement of the German Cardiac Society (DGK). Clin Res Cardiol 2021; 110: 479-506. Copyright© The Authors 2021. Published by Springer Nature. The article is open access (Supplementary material).

Figure 4 Simplified diagnostic algorithm for suspected cardiac amyloidosis. ATTR: Amyloidosis, transthyretin-related; ECG: Electrocardiogram; CMR: Cardiac magnetic resonance; AL: Amyloid light-chain; CA: Cardiac amyloidosis; SPECT: Single-photon emission computed tomography; ATTRv: Hereditary variant amyloidosis, transthyretin-related; ATTRwt: Amyloidosis, transthyretin-related wild-type.