Transthyretin Amyloid Cardiomyopathy: Impact of Transthyretin Amyloid Deposition in Myocardium on Cardiac Morphology and Function

• Published in: Journal of personalized medicine Vol. 12; no. 5; p. 792
• Main Authors: Nakano, Tomoya, Onoue, Kenji, Terada, Chiyoko, Terasaki, Satoshi, Ishihara, Satomi, Hashimoto, Yukihiro, Nakada, Yasuki, Nakagawa, Hitoshi, Ueda, Tomoya, Seno, Ayako, Nishida, Taku, Watanabe, Makoto, Hoshii, Yoshinobu, Hatakeyama, Kinta, Sakaguchi, Yasuhiro, Ohbayashi, Chiho, Saito, Yoshihiko
• Format: Journal Article
• Language: English
• Published: Switzerland MDPI AG 13.05.2022; MDPI
• Subjects: Age; Biopsy; Cardiac arrhythmia; cardiac dysfunction; Cardiomyocytes; Cardiomyopathy; concentric left ventricular hypertrophy; Congestive heart failure; Deoxyguanosine; Ejection fraction; Electrocardiography; endomyocardial biopsy; Geometry; Heart failure; human heart tissue; Hypertrophy; Immunoglobulins; immunostaining; Morphology; Mutation; Myocardium; Patients; Peptides; Precision medicine; Proteins; Reactive oxygen species; Software; Stains & staining; Transthyretin; Ventricle; Japan; human heart tissue; reactive oxygen species; endomyocardial biopsy; cardiac dysfunction; immunostaining; concentric left ventricular hypertrophy; transthyretin amyloid cardiomyopathy
• ISSN: 2075-4426; 2075-4426
• DOI: 10.3390/jpm12050792

Transthyretin (TTR) amyloid cardiomyopathy (ATTR-CM) is increasingly being recognized as a cause of left ventricular (LV) hypertrophy (LVH) and progressive heart failure in elderly patients. However, little is known about the cardiac morphology of ATTR-CM and the association between the degree of TTR amyloid deposition and cardiac dysfunction in these patients. We studied 28 consecutive patients with ATTR-CM and analyzed the relationship between echocardiographic parameters and pathological features using endomyocardial biopsy samples. The cardiac geometries of patients with ATTR-CM were mainly classified as concentric LVH (96.4%). The relative wall thickness, a marker of LVH, tended to be positively correlated with the degree of non-cardiomyocyte area. The extent of TTR deposition was positively correlated with enlargement of the non-cardiomyocyte area, and these were positively correlated with LV diastolic dysfunction. Additionally, the extent of the area containing TTR was positively correlated with the percentage of cardiomyocyte nuclei stained for 8-hydroxy-2'deoxyguanosine, a marker of reactive oxygen species (ROS). ROS accumulation in cardiomyocytes was positively correlated with LV systolic dysfunction. Patients with ATTR-CM mainly displayed concentric LVH geometry. TTR amyloid deposition was associated with cardiac dysfunction via increased non-cardiomyocyte area and ROS accumulation in cardiomyocytes.