心房淀粉样变性的当前观点:一篇叙述性综述。
Current Perspectives on Atrial Amyloidosis: A Narrative Review.
作者信息
Tana Marco, Tana Claudio, Guglielmi Maria Domenica, Stefanelli Arianna, Mantini Cesare, Porreca Ettore
机构信息
Internal Medicine and Cardiovascular Ultrasound Unit, Medical Department, St Annunziata Hospital, 66100 Chieti, Italy.
Department of Innovative Technologies in Medicine and Dentistry, G. D'Annunzio University of Chieti-Pescara, 66100 Chieti, Italy.
出版信息
Rev Cardiovasc Med. 2024 Feb 20;25(2):73. doi: 10.31083/j.rcm2502073. eCollection 2024 Feb.
Amyloidosis is a systemic disease caused by low molecular weight protein accumulation in the extracellular space, which can lead to different degrees of damage, depending of the organ or tissue involved. The condition is defined cardiac amyloidosis (CA) when heart is affected, and it is associated with an unfavorable outcome. Different types of CA have been recognized, the most common (98%) are those associated with deposition of light chain (AL-CA), and the form secondary to transthyretin deposit. The latter can be classified into two types, a wild type (transthyretin amyloidosis wild type (ATTRwt)-CA), which mainly affects older adults, and the hereditary or variant type (ATTRh-CA or ATTRv-CA), which instead affects more often young people and is associated with genetic alterations. The atrial involvement can be isolated or linked to CA with a nonspecific clinical presentation represented by new onset atrial fibrillation (AF), diastolic dysfunction and heart failure with preserved ejection fraction, or thromboembolism and stroke. Untreated patients have a median survival rate of 9 years for AL-CA and 7 years for ATTR-CA. By contrast, AL-CA and ATTR-CA treated patients have a median survival rate of 24 and 10 years, respectively. Atrial involvement in CA is a common but poor studied event, and alterations of performance can anticipate the anatomical damage. Recently, numerous advances have been made in the diagnostic field with improvements in the available techniques. An early diagnosis therefore allows a more effective therapeutic strategy with a positive impact on prognosis and mortality rate. A multimodality approach to the diagnosis of atrial involvement from CA is therefore recommended, and standard echocardiography, advanced Doppler-echocardiography (DE) and cardiac magnetic resonance (CMR) can be useful to detect early signs of CA and to estabilish an appropriate treatment.
淀粉样变性是一种由低分子量蛋白质在细胞外间隙积聚引起的全身性疾病,根据受累的器官或组织不同,可导致不同程度的损害。当心脏受累时,这种情况被定义为心脏淀粉样变性(CA),并且与不良预后相关。已识别出不同类型的CA,最常见的(98%)是与轻链沉积相关的类型(AL-CA),以及继发于转甲状腺素蛋白沉积的类型。后者可分为两种类型,一种是野生型(转甲状腺素蛋白淀粉样变性野生型(ATTRwt)-CA),主要影响老年人,另一种是遗传性或变异型(ATTRh-CA或ATTRv-CA),更常影响年轻人且与基因改变有关。心房受累可以是孤立的,也可以与CA相关,其非特异性临床表现为新发房颤(AF)、舒张功能障碍和射血分数保留的心力衰竭,或血栓栓塞和中风。未经治疗的AL-CA患者中位生存期为9年,ATTR-CA患者为7年。相比之下,接受治疗的AL-CA和ATTR-CA患者中位生存期分别为24年和10年。CA中的心房受累是一个常见但研究较少的情况,功能改变可先于解剖学损害出现。最近,随着现有技术的改进,诊断领域取得了许多进展。因此,早期诊断可采用更有效的治疗策略,对预后和死亡率产生积极影响。因此,建议采用多模态方法诊断CA引起的心房受累,标准超声心动图、先进的多普勒超声心动图(DE)和心脏磁共振(CMR)有助于检测CA的早期迹象并确定合适的治疗方案。
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