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. 2020 Jul 21;2(8):420-424.
doi: 10.1253/circrep.CR-20-0062.

Initial Experience With Tafamidis Treatment for Transthyretin Amyloid Cardiomyopathy

Makiko Nakamura  1 , Teruhiko Imamura  1 , Masakazu Hori  1 , Ryuichi Ushijima  1 , Shuji Joho  1 , Koichiro Kinugawa  1
Affiliations

Initial Experience With Tafamidis Treatment for Transthyretin Amyloid Cardiomyopathy

Makiko Nakamura et al. Circ Rep. .

Abstract

Background: Transthyretin amyloid cardiomyopathy is a progressive disease with a poor prognosis. There had been no specific treatment for transthyretin amyloid cardiomyopathy until tafamidis received expanded approval in March 2019 in Japan. However, the clinical efficacy of tafamidis remains unknown. Methods and Results: We initiated tafamidis treatment in 9 patients (median age 78 years; 89% male) from May 2019 to April 2020. Within 6 months after initiation, 1 patient discontinued prematurely and 2 patients were hospitalized due to worsening heart failure, with 1 of these patients discontinuing therapy. There were no significant changes in plasma B-type natriuretic peptide and serum troponin I concentrations over the 6-month treatment period, but interventricular septum thickness increased in 3 of 6 patients. Conclusions: Further evaluation of tafamidis therapy in a larger patient cohort with transthyretin amyloid cardiomyopathy is warranted to determine the optimal therapeutic strategy.

Keywords: Atrial fibrillation; Cardiac hypertrophy; Congestive heart failure.

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Conflict of interest statement

K.K. is a member of Circulation Reports ’ Editorial Team.

Figures

Figure 1.
Figure 1.
Trends in (A) B-type natriuretic peptide (BNP), (B) N-terminal pro B-type natriuretic peptide (NT-proBNP), and (C) troponin I concentrations, as well as (D) QRS duration before and 6 months after the initiation of tafamidis in 6 patients who continued on tafamidis for more than 6 months. Median (interquartile rage) values for each of the parameters before and at 6 months are given at the top of each graph.
Figure 2.
Figure 2.
Trends in (A) interventricular septum thickness (IVST), (B) left ventricular mass index (LVMI), (C) the ratio of early transmitral flow velocity to early mitral annular velocity (E/e′), and (D) left ventricular stroke volume index before and 6 months after the initiation of tafamidis in 6 patients who continued on tafamidis for more than 6 months. Median (interquartile rage) values for each of the parameters before and at 6 months are given at the top of each graph.
See this image and copyright information in PMC

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