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无症状转甲状腺素蛋白淀粉样变突变携带者的亚临床心脏受累:来自心脏磁共振成像、心肌应变和标测技术的见解

Subclinical Cardiac Involvement in Asymptomatic ATTR Mutation Carriers: Insights from Cardiac MRI, Myocardial Strain, and Mapping Techniques.

作者信息

Conia Luca, Filatova Daria, Pambianchi Giacomo, Marchitelli Livia, Cundari Giulia, Stancanelli Giuseppe, Alfarano Maria, Marchionni Giulia, Chimenti Cristina, Catalano Carlo, Galea Nicola

机构信息

Department of Radiological, Oncological and Pathological Sciences, Sapienza University of Rome, 00161 Rome, Italy.

Department of Radiation Diagnostics and Therapy, Lomonosov Moscow State University, Moscow 119991, Russia.

出版信息

J Cardiovasc Dev Dis. 2025 May 1;12(5):172. doi: 10.3390/jcdd12050172.

Abstract

Transthyretin cardiac amyloidosis (ATTR-CA) leads to myocardial infiltration, affecting prognosis and survival. Diagnosing early-stage ATTR-CA remains challenging due to its subtle manifestations. This study investigates subclinical myocardial alterations in asymptomatic ATTR mutation carriers (ATTR-MC) using advanced cardiac magnetic resonance (CMR) techniques, including T1 mapping and myocardial strain analysis. A retrospective cohort of 60 subjects was analyzed, comprising 20 ATTR-CA patients, 20 asymptomatic ATTR-MC, and 20 controls. Standard CMR parameters were compared alongside myocardial strain analysis. Results indicated that despite preserved ejection fraction and myocardial morphology, ATTR-MC exhibited significantly impaired left ventricular global longitudinal strain (LV GLS), left atrial reservoir, conduit, and booster pump strain (LA RS, CS, and BPS) compared to controls. However, native T1 and extracellular volume (ECV) values remained within normal ranges, distinguishing early dysfunction from overt amyloid deposition seen in ATTR-CA. These findings suggest that myocardial strain analysis could serve as an early biomarker for subclinical ATTR-CA, offering a potential target for selecting patients who may benefit from early intervention. Implementing CMR-derived strain parameters in clinical practice may improve risk stratification and timely therapeutic decisions in ATTR-MC.

摘要

转甲状腺素蛋白心脏淀粉样变性(ATTR-CA)会导致心肌浸润,影响预后和生存率。由于其临床表现不明显,早期ATTR-CA的诊断仍然具有挑战性。本研究使用先进的心脏磁共振(CMR)技术,包括T1 mapping和心肌应变分析,调查无症状ATTR突变携带者(ATTR-MC)的亚临床心肌改变。分析了一个包含60名受试者的回顾性队列,其中包括20名ATTR-CA患者、20名无症状ATTR-MC和20名对照。将标准CMR参数与心肌应变分析进行了比较。结果表明,尽管射血分数和心肌形态保持正常,但与对照组相比,ATTR-MC的左心室整体纵向应变(LV GLS)、左心房储器、管道和增压泵应变(LA RS、CS和BPS)显著受损。然而,天然T1和细胞外容积(ECV)值仍在正常范围内,这将早期功能障碍与ATTR-CA中明显的淀粉样蛋白沉积区分开来。这些发现表明,心肌应变分析可作为亚临床ATTR-CA的早期生物标志物,为选择可能从早期干预中获益的患者提供潜在靶点。在临床实践中应用CMR衍生的应变参数可能会改善ATTR-MC的风险分层和及时的治疗决策。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/18a4/12112504/34f6b36ce491/jcdd-12-00172-g001.jpg

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