Cardiac amyloidosis: Topical Collection

Cardiac amyloidosis imageCardiac amyloidosis is a form of restrictive infiltrative cardiomyopathy that confers significant mortality. Due to the relative rarity of cardiac amyloidosis, clinical and diagnostic expertise in the recognition and evaluation of individuals with suspected amyloidosis is mostly limited to a few expert centers.

This collection of recent JNC publications aims to present a comprehensive overview of the many different facets related to diagnosis and treatment.

ASNC/AHA/ASE/EANM/HFSA/ISA/SCMR/SNMMI expert consensus recommendations for multimodality imaging in cardiac amyloidosis: Part 1 of 2—evidence base and standardized methods of imaging. Dorbala S, Ando Y, Bokhari S, et al.

ASNC/AHA/ASE/EANM/HFSA/ISA/SCMR/SNMMI expert consensus recommendations for multimodality imaging in cardiac amyloidosis: Part 2 of 2—Diagnostic criteria and appropriate utilization. Dorbala S, Ando Y, Bokhari S, et al.

Positron emission tomography (PET) utilizing Pittsburgh compound B (PIB) for detection of amyloid heart deposits in hereditary transthyretin amyloidosis (ATTR). Pilebro B, Arvidsson S, Lindqvist P, et al.

Standardization of (99m)Technetium pyrophosphate imaging methodology to diagnose TTR cardiac amyloidosis. Bokhari S, Morgenstern R, Weinberg R, et al.

(18)Fluorine sodium fluoride positron emission tomography, a potential biomarker of transthyretin cardiac amyloidosis. Morgenstern R, Yeh R, Castano A, et al.

Imaging cardiac innervation in amyloidosis. Slart RHJA, Glaudemans AWJM, Hazenberg BPC, Noordzij W.

Technetium pyrophosphate uptake in transthyretin cardiac amyloidosis: Associations with echocardiographic disease severity and outcomes. Vranian MN, Sperry BW, Hanna M, et al.

State-of-the-art radionuclide imaging in cardiac transthyretin amyloidosis. Singh V, Falk R, Di Carli MF, et al.

Apical sparing pattern of left ventricular myocardial Tc-99m-HMDP uptake in patients with transthyretin cardiac amyloidosis. Van Der Gucht A, Cottereau A-S, Abulizi M, et al.

Setting the stage for the next step in cardiac amyloidosis imaging: Serial quantitative studies to assess disease activity. Soman P & Masri A.


Excerpts and images reprinted from Dorbala S, Ando Y, Bokhari S, et al. (2019) ASNC/AHA/ASE/EANM/HFSA/ISA/SCMR/SNMMI expert consensus recommendations for multimodality imaging in cardiac amyloidosis: Part 1 of 2—evidence base and standardized methods of imaging. J Nucl Cardiol. https://doi.org/10.1007/s12350-019-01760-6.

Figure 2 caption: Characteristic appearance of cardiac amyloidosis on echocardiography. (a)-(d) 2D echocardiography. (a) (parasternal long axis) and (b) (parasternal short axis) demonstrate increased LV wall thickness with a sparkling texture of the myocardium (yellow arrows) in a patient with primary (AL) cardiac amyloidosis. Also, note the small pericardial effusion (white arrows), which is often seen in patients with cardiac amyloidosis. (c) (apical 4-chamber view) demonstrates increased biventricular wall thickness, biatrial enlargement, and increased thickening of the interatrial septum (yellow arrow) and mitral valve leaflets (white arrow) in a patient with wild-type transthyretin cardiac amyloidosis. (d) Tissue Doppler imaging (TDI) tracing taken at the septal mitral annulus in a patient with ATTR cardiac amyloidosis. The TDI tracings shows the “5-5-5” sign (s’ [systolic], e’ [early diastolic], and a’ [late (atrial) diastolic] tissue velocities are all < 5 cm/s), which is seen in patients with more advanced cardiac amyloidosis. The dotted lines denote the 5 cm/s cut-off for systolic and diastolic tissue velocities. In addition to the decreased tissue velocities, isovolumic contraction and relaxation times (IVCT and IVRT, respectively) are increased and ejection time (ET) is decreased, findings also seen in patients with cardiac amyloidosis especially as the disease becomes more advanced