Institute of Life Sciences, Scuola Superiore Sant'Anna, Italy.
University of Ferrara, Italy.
Eur J Prev Cardiol. 2021 Aug 9;28(9):1000-1005. doi: 10.1177/2047487320920756. Epub 2020 Apr 16.
Amyloidosis is a systemic disorder characterized by extracellular deposition of insoluble fibrils. The most common forms are amyloid light chain and amyloid transthyretin (ATTR) amyloidoses. Cardiac involvement may be found in both these forms, and is an important cause of morbidity and mortality. The clinical presentation of cardiac amyloidosis (CA) may be represented by congestive heart failure (HF), possibly progressing to end-stage HF, as well as atrial fibrillation with possible thromboembolic events, and also conduction disturbances related to amyloid infiltration of conduction fibres. Beyond therapies targeting the blood dyscrasia or the ATTR amyloidogenic cascade, a careful choice of drug therapies, need for device implantation, and possibly treatments for advanced HF is then warranted. In the present review, we try to provide a useful guide to clinicians treating patients with CA by enucleating 10 main questions and answering them based on the evidence available as well as expert opinion and our clinical experience.
淀粉样变性是一种全身性疾病,其特征是细胞外不可溶性纤维的沉积。最常见的形式是轻链淀粉样变性和转甲状腺素淀粉样变性(ATTR)。这两种形式均可累及心脏,是发病率和死亡率的重要原因。心脏淀粉样变性(CA)的临床表现可能表现为充血性心力衰竭(HF),可能进展为终末期 HF,以及可能伴有血栓栓塞事件的心房颤动,以及与传导纤维淀粉样浸润相关的传导障碍。除了针对血液异常或 ATTR 淀粉样变性级联的治疗外,还需要仔细选择药物治疗、器械植入的需求,以及可能对晚期 HF 的治疗。在本综述中,我们试图通过提出 10 个主要问题,并根据现有证据以及专家意见和我们的临床经验回答这些问题,为治疗 CA 患者的临床医生提供有用的指南。