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Editorial
. 2019 Jul 2;140(1):27-30.
doi: 10.1161/CIRCULATIONAHA.119.041015. Epub 2019 Jul 1.

The Truth Is Unfolding About Transthyretin Cardiac Amyloidosis

Justin L Grodin  1 , Mathew S Maurer  2
Affiliations
Editorial

The Truth Is Unfolding About Transthyretin Cardiac Amyloidosis

Justin L Grodin et al. Circulation. .
No abstract available

Keywords: Editorials; amyloidosis, hereditary; cardiomyopathies; quality of life.

PubMed Disclaimer

Conflict of interest statement

CONFLICT OF INTEREST DISCLOSURES

J.L.G. has consulting income from Pfizer.

M.S.M. has consulting income from Pfizer, GSK, EIdos, Prothena, Akcea and Alnylam, and institution received clinical trial funding from Pfizer, Prothena, Eidos and Alnylam

Figures

Figure 1.
Figure 1.. Conceptual model of ATTR cardiac amyloidosis progression over time
Changes in various parameters are shown. The relative scale specific to each factor and time course are not proportional. Myocardial amyloid infiltration occurs before clinically manifest changes in ejection fraction, cardiac biomarkers and renal function. Thus, most patients with ATTR-CA likely have a long latency period prior to declines in functional capacity, which can occur rapidly in the context of multiple hospitalizations for acute decompensated heart failure and arrhythmias. The ideal emerging therapeutic window for novel therapies is hypothesized to be before significant organ dysfunction has occurred and prior to rapid and potentially irreversible declines in functional capacity. * Biomarker Stage defined by the Mayo Clinic for ATTRwt as cardiac troponin T (< 0.05 ng/mL) and NTproBNP (< 3,000 pg/mL) with Stages I, II, and III are defined as having both, one, or neither of the markers below the threshold, with a median survival of 66, 40 and 20 months, respectively or by the UK-NAC for both ATTRwt and ATTRh with estimated glomerular filtration rate (eGFR) of 45 mL/min instead of troponin. Median survival was 69, 47, and 24 months in Stages I, II, and III, respectively, with longer survival in ATTRwt compared to ATTRh-CA.
See this image and copyright information in PMC

Comment on

  • Natural History, Quality of Life, and Outcome in Cardiac Transthyretin Amyloidosis.
    Lane T, Fontana M, Martinez-Naharro A, Quarta CC, Whelan CJ, Petrie A, Rowczenio DM, Gilbertson JA, Hutt DF, Rezk T, Strehina SG, Caringal-Galima J, Manwani R, Sharpley FA, Wechalekar AD, Lachmann HJ, Mahmood S, Sachchithanantham S, Drage EPS, Jenner HD, McDonald R, Bertolli O, Calleja A, Hawkins PN, Gillmore JD. Lane T, et al. Circulation. 2019 Jul 2;140(1):16-26. doi: 10.1161/CIRCULATIONAHA.118.038169. Epub 2019 May 21. Circulation. 2019. PMID: 31109193

References

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Supplementary concepts