Cardiac amyloidosis and red flags: natural history and its impact in morbimortality.

IF 2.6 4区 医学 Q1 MEDICINE, GENERAL & INTERNAL
Emilio Blanco-López, Jorge Martínez-Del Río, Alba López-Calles, Martín Negreira-Caamaño, Daniel Águila-Gordo, Pablo Soto-Martín, Maria Maeve Soto-Pérez, Andrez Felipe Cubides-Novoa, Maria Gonzalez-Barderas, Ignacio Sánchez-Pérez, Jesús Piqueras-Flores
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引用次数: 0

Abstract

Introduction and objectives: Red flags (RF) are typical cardiac and extracardiac manifestations that may precede the definitive diagnosis of cardiac amiloidosis (CA) by several years, playing a pivotal role in the early diagnosis of the disease. The principal aim of the research was to determine the chronology of onset of RF throughout the natural history of CA as well as its prognostic influence.

Patients and methods: Observational, retrospective inquiry of consecutive patients with a definitive diagnosis of CA in a terciary hospital centre in Ciudad Real (Spain) between February 2016 and December 2023. We defined 21 RF and 3 adverse clinical events, establishing the date of occurrence of each of them.

Results: 102 patients (81.6±7.7 years; 84,3% males) with a diagnosis of CA (89.2% TTR; 10,8% AL) were included. The prevalence of RF was very high (8.4±2.3). In the natural history, the first to appear were integumentary, with the most specific cardiological ones being the closest to diagnosis. The 2-year mortality was 49%, with biomarker RFs and the presence of ≥10 RFs being associated with higher mortality.

Conclusions: RFs proved highly prevalent among patients with CA and substantially preceded disease diagnosis. RF burden was associated with prognosis in the follow-up of ATTR patients.

心脏淀粉样变性和红旗:自然史及其对死亡率的影响。
导言和目的:红旗(RF)是典型的心脏和心外表现,可能比心脏蛛网膜病(CA)的明确诊断早数年,在疾病的早期诊断中起着关键作用。研究的主要目的是确定 RF 在整个 CA 自然史中的发病时间顺序及其对预后的影响:对2016年2月至2023年12月期间在雷阿尔城(西班牙)一家三甲医院中心明确诊断为CA的连续患者进行观察性、回顾性调查。我们定义了 21 个 RF 和 3 个不良临床事件,并确定了每个事件的发生日期:共纳入 102 名诊断为 CA(89.2% TTR;10.8% AL)的患者(81.6±7.7 岁;84.3% 男性)。RF的发病率非常高(8.4±2.3)。在自然病史中,最先出现的是全身性疾病,而最接近诊断的是最特殊的心脏病。2年死亡率为49%,生物标志物RFs和RFs≥10个与死亡率较高有关:结论:RFs在CA患者中非常普遍,而且在疾病诊断前就已存在。在对ATTR患者的随访中,RF负荷与预后相关。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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来源期刊
Medicina Clinica
Medicina Clinica 医学-医学:内科
CiteScore
3.10
自引率
5.10%
发文量
295
审稿时长
22 days
期刊介绍: Medicina Clínica, fundada en 1943, es una publicación quincenal dedicada a la promoción de la investigación y de la práctica clínica entre los especialistas de la medicina interna, así como otras especialidades. Son características fundamentales de esta publicación el rigor científico y metodológico de sus artículos, la actualidad de los temas y, sobre todo, su sentido práctico, buscando siempre que la información sea de la mayor utilidad en la práctica clínica.
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