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1. Maurer MS, Elliott P, Merlini G, Shah SJ, Cruz MW, Flynn A, et al; ATTR-ACT Study Investigators. Design and rationale of the phase 3 ATTR-ACT Clinical Trial (Tafamidis in Transthyretin Cardiomyopathy Clinical Trial). Circ Heart Fail 2017; 10: e003815. T ransthyretin (TTR) amyloidosis, caused by the deposition of wild-type TTR (ATTRwt) in the heart, is an underdiagnosed cause of heart failure with preserved ejection fraction (HFpEF). The relative apical sparing pattern on 2-D speckle-tracking echocardiography (2D-STE) is frequently seen in cardiac amyloidosis. Tafamidis is a kinetic stabilizer of TTR that inhibits misfolding and amyloidogenesis. We present a case of biopsy-proven WT-TTR, in which the cardiac apical sparing pattern improved with tafamidis treatment. A 78-year-old man, misdiagnosed with hypertensive cardiomyopathy, was hospitalized with HFpEF. He also had orthostatic hypotension and plantar numbness. Both endomyocardial and intestinal biopsy confirmed the presence of TTR amyloid deposits (Figure A), accompanied by positive late gadolinium enhancement on cardiac magnetic resonance imaging (Figure B). 2D-STE on admission showed a relative apical sparing pattern with global longitudinal strain −5.7%. Heart failure medication was optimized, and treatment with 20 mg tafamidis daily was initiated. The area where the myocardial strain was preserved, expanded in the course of treatment (Figure C). Received May 24, 2018; revised manuscript received July 21, 2018; accepted July 28, 2018; released online September 5, 2018 Time for primary review: 21 days Department of Medicine, Division of Cardiology, Showa University Koto Toyosu Hospital, Tokyo (T.S.K., N.I., K.T.); Department of Cardiovascular Medicine, Juntendo University Faculty of Medicine, Tokyo (T.S.K.), Japan; Department of Medicine, Division of Cardiology, Columbia University Medical Center, New York, NY (M.S.M.), USA; and Department of Neurology, Graduate School of Medical Sciences, Kumamoto University, Kumamoto (Y.A.), Japan Mailing address: Tomoko S. Kato, MD, PhD, Department of Medicine, Division of Cardiology, Showa University Koto Toyosu Hospital, 5-1-38 Toyosu, Koto-ku, Tokyo 135-8577, Japan. E-mail: rinnko@sannet.ne.jp ISSN-1346-9843 All rights are reserved to the Japanese Circulation Society. For permissions, please e-mail: cj@j-circ.or.jp Transthyretin Stabilizer Is Associated With Expanding Apical Sparing Area and Improving Global Cardiac Function in a Patient With Wild-Type Cardiac Amyloidosis

circulation journal

Transthyretin Stabilizer Is Associated With Expanding Apical Sparing Area and Improving Global Cardiac Function in a Patient With Wild-Type Cardiac Amyloidosis