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淀粉样心肌病和心力衰竭患者的心脏收缩力调制治疗、病例报告、CCM功能生物物理学综述及AMY-CCM注册研究介绍

Cardiac Contractility Modulation Therapy in Patients with Amyloid Cardiomyopathy and Heart Failure, Case Report, Review of the Biophysics of CCM Function, and AMY-CCM Registry Presentation.

作者信息

Marchese Procolo, Gennaro Francesca, Mazzotta Giovanni, Acciarri Claudia, Amabili Stenio, Bonanni Carlo, D'Antonio Antonella, Delfino Domenico, Di Vito Luca, Partemi Manrico, Pascucci Riccardo, Romandini Andrea, Scalone Giancarla, Silenzi Simona, Grossi Pierfrancesco

机构信息

Cardiology Unit, Mazzoni Civil Hospital, 63100 Ascoli Piceno, Italy.

出版信息

J Clin Med. 2023 Feb 2;12(3):1184. doi: 10.3390/jcm12031184.

Abstract

Cardiac amyloidosis may result in an aggressive form of heart failure (HF). Cardiac contractility modulation (CCM) has been shown to be a concrete therapeutic option in patients with symptomatic HF, but there is no evidence of its application in patients with cardiac amyloidosis. We present the case of TTR amyloidosis, where CCM therapy proved to be effective. The patient had a history of multiple HF hospitalizations due to an established diagnosis of wild type TTR-Amyloidosis with significant cardiac involvement. Since he was highly symptomatic, except during continuous dobutamine and diuretic infusion, it was opted to pursue CCM therapy device implantation. At follow up, a significant improvement in clinical status was reported with an increase of EF, functional status (6 min walk test improved from zero meters at baseline, to 270 m at 1 month and to 460 m at 12 months), and a reduction in pulmonary pressures. One year after device implantation, no other HF hospital admission was needed. CCM therapy may be effective in this difficult clinical setting. The AMY-CCM Registry, which has just begun, will evaluate the efficacy of CCM in patients with HF and diagnosed TTR amyloidosis to bring new evidence on its potential impact as a therapeutic option.

摘要

心脏淀粉样变性可能导致一种侵袭性心力衰竭(HF)。心脏收缩力调制(CCM)已被证明是有症状HF患者的一种切实可行的治疗选择,但尚无其应用于心脏淀粉样变性患者的证据。我们报告了1例转甲状腺素蛋白淀粉样变性病例,CCM治疗在该病例中被证明是有效的。该患者因确诊为野生型转甲状腺素蛋白淀粉样变性且有显著心脏受累,有多次HF住院史。由于他症状严重,除持续静脉输注多巴酚丁胺和利尿剂期间外,决定植入CCM治疗设备。随访时,报告临床状况有显著改善,射血分数增加,功能状态(6分钟步行试验从基线时的0米改善到1个月时的270米和12个月时的460米),肺压力降低。设备植入一年后,无需再次因HF住院。CCM治疗在这种困难的临床情况下可能有效。刚刚启动的AMY - CCM注册研究将评估CCM对HF合并确诊转甲状腺素蛋白淀粉样变性患者的疗效,以提供其作为一种治疗选择的潜在影响的新证据。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f830/9917884/50ae297efa59/jcm-12-01184-g001.jpg

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