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Review
. 2012 Sep 4;126(10):1286-300.
doi: 10.1161/CIRCULATIONAHA.111.078915.

Transthyretin (TTR) cardiac amyloidosis

Frederick L Ruberg  1 , John L Berk
Affiliations
Review

Transthyretin (TTR) cardiac amyloidosis

Frederick L Ruberg et al. Circulation. .
No abstract available

PubMed Disclaimer

Figures

Figure 1
Figure 1
Congo Red staining of myocardial tissue from a patient with amyloid cardiomyopathy. A) Light microscopy, and B) polarized light microscopy, 400X magnification.
Figure 2
Figure 2
TTR amyloid cardiomyopathy by immunohistochemical (IHC) staining. Endomyocardial biopsies were stained with antibodies to a) kappa light-chain, b) lambda lightchain, c) Serum amyloid A, and d) TTR amyloid. Bright light micrographs at 400X magnification.
Figure 3
Figure 3
Isoelectric focusing gel electrophoresis. Sera from patients with V122I, wild-type, and L58H ATTR. Black arrow indicates wild-type TTR migration. Note presence of 2 distinct bands in V122I and L58H lanes.
Figure 4
Figure 4
Diagnostic algorithm for diagnosis of TTR cardiac amyloidosis. CMR: Cardiac Magnetic Resonance Imaging, AL: Light-chain amyloidosis, LC/MS – Laser dissection/liquid chromatography/tandem mass spectrometry, PCR – polymerase chain reaction
Figure 5
Figure 5
Echocardiographic appearance of V122I ATTR cardiac amyloidosis. Parasternal long axis (A) and short axis (B) are illustrated, demonstrating increased ventricular wall thickness, pleural and pericardial effusions. Panel C depicts a restrictive transmitral Doppler pattern. Panel D demonstrates tissue Doppler velocities consistent with reduced longitudinal systolic shortening (reduced S’ velocity) as well as diastolic dysfunction (reduced e’ velocity).
Figure 6
Figure 6
Cardiac Magnetic Resonance (CMR) Imaging of TTR amyloidosis. Late gadolinium enhancement (LGE) images from mid-ventricular short axis slices are depicted illustrating the different LGE patterns observed. In panel A, LGE is evident (arrows) in a characteristic, diffuse sub-endocardial pattern (patient with wtTTR). In panel B, a diffuse transmural low-intensity signal is seen with poor contrast between the blood pool and myocardium (patient with V122I vTTR). In panel C, high signal intensity, patchy LGE is evident involving the subendocardium in the lateral wall, but transmurally involving the septum as well (arrows, patient with wtTTR).
See this image and copyright information in PMC

References

    1. Merlini G, Seldin DC, Gertz MA. Amyloidosis: Pathogenesis and new therapeutic options. Journal of Clinical Oncology. 2011;29:1924–1933. - PMC - PubMed
    1. Sipe JD, Benson MD, Buxbaum JN, Ikeda S, Merlini G, Saraiva MJ, Westermark P. Amyloid fibril protein nomenclature: 2010 recommendations from the nomenclature committee of the International Society of Amyloidosis. Amyloid. 2010;17:101–104. - PubMed
    1. Dubrey SW, Hawkins PN, Falk RH. Amyloid diseases of the heart: Assessment, diagnosis, and referral. Heart. 2011;97:75–84. - PubMed
    1. Picken MM. Amyloidosis-where are we now and where are we heading? Archives of Pathology & Laboratory Medicine. 2010;134:545–551. - PubMed
    1. Lavatelli F, Vrana JA. Proteomic typing of amyloid deposits in systemic amyloidoses. Amyloid. 2011;18:177–182. - PubMed

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