- 著者
- Hiroki Usuku Eiichiro Yamamoto Masato Nishi Takashi Komorita Masafumi Takae Taiki Nishihara Fumi Oike Masanobu Ishii Koichiro Fujisue Daisuke Sueta Satoshi Araki Seiji Takashio Seitaro Oda Yohei Misumi Mitsuharu Ueda Taishi Nakamura Hiroaki Kawano Hirofumi Soejima Kenji Sakamoto Koichi Kaikita Yukio Ando Hirotaka Matsui Kenichi Tsujita
- 出版者
- The Japanese Circulation Society
- 雑誌
- Circulation Reports (ISSN:24340790)
- 巻号頁・発行日
- vol.2, no.12, pp.730-738, 2020-12-10 (Released:2020-12-10)
- 参考文献数
- 27
Background:Using transthoracic echocardiography, including 2D speckle tracking imaging (STI), this study examined cardiac function after domino liver transplantation (DLT) with liver grafts explanted from patients with hereditary amyloidogenic transthyretin amyloidosis.Methods and Results:In all, 14 patients who underwent DLT at Kumamoto University Hospital and for whom 2D STI information was available were enrolled in the study; time-dependent echocardiographic changes were evaluated in 7. Although left ventricular (LV) systolic and diastolic function did not differ between the pre- and post-DLT periods (mean [±SD] 5.4±1.0 years after DLT), there were significant (P<0.05 for all) increases in the post- vs. pre-DLT period in basal longitudinal strain (LS; −13.4±2.3 vs. −19.3±4.4), relative apical LS index (=apical LS/[basal LS+mid LS]; 0.75±0.20 vs. 0.58±0.08), and LV ejection fraction/global LS (3.91±0.58 vs. 3.06±0.44). Age at the time of DLT was significantly higher in the group with impaired (>−14%) than preserved basal LS (57.2±3.5 vs. 39.6±16.0 years; P<0.05). When control subjects (n=14) were added to the enrolled DLT recipients, multivariable logistic regression analysis revealed that a history of DLT was significantly associated with impaired basal LS (>−14%; odds ratio 28.39, 95% confidence interval 1.89–427.45, P<0.05).Conclusions:LV systolic and diastolic function was preserved in the long term after DLT. However, 2D STI revealed subtle cardiac dysfunction in DLT recipients, which may be an early manifestation of cardiac amyloidosis.
- 著者
- Taku Nakase Taro Yamashita Yoshimasa Matsuo Toshiya Nomura Keiko Sasada Teruaki Masuda Yohei Misumi Kotaro Takamatsu Seitaro Oda Yutaro Furukawa Konen Obayashi Hirotaka Matsui Yukio Ando Mitsuharu Ueda
- 出版者
- The Japanese Society of Internal Medicine
- 雑誌
- Internal Medicine (ISSN:09182918)
- 巻号頁・発行日
- pp.2456-18, (Released:2019-06-07)
- 参考文献数
- 17
- 被引用文献数
- 3
We report the clinical features of a patient with hereditary ATTR amyloidosis associated with a novel mutation (Y114S, p.Y134S). A 65-year-old Japanese man was admitted to our hospital after a 3-year history of progressive dyspnea on exertion. Five years previously, he presented dysesthesia in both hands caused by carpal tunnel syndrome. A genetic analysis revealed a base pair substitution of adenine to cytosine in the second codon of exon 4, residue 114, in the TTR gene (c.401A>C). The clinical characteristics were progressive cardiomyopathy with a poor vital prognosis, late onset, sporadic case, bilateral carpal tunnel syndrome, hypothyroidism, and small fiber neuropathy.