Cardiac amyloidosis originates primarily from the accumulation of insoluble misfolded protein deposits within the myocardial interstitium [1–3]. While >30 proteins are known to be capable of aggregating as amyloid in vivo, nonmutated (ATTRwt) and variant transthyretin (ATTRv) are the most frequent amyloidogenic proteins impacting the heart. Both ATTRwt and ATTRv cardiac amyloidosis can elicit restrictive cardiomyopathy, leading to poor outcomes including heart failure and death [1–3]. While myocardial dysfunction is often cited as the predominant mechanism for dyspnoea and exercise intolerance in patients with cardiac amyloidosis, growing evidence suggests that extracardiac causes, including abnormal lung function, may also be responsible for these clinical symptoms [1–3].

心脏淀粉样变性主要源于心肌间质内不溶性错误折叠蛋白沉积物的积累 [1-3]。虽然已知超过 30 种蛋白质能够在体内聚集为淀粉样蛋白，但非突变 (ATTRwt) 和变体转甲状腺素蛋白 (ATTRv) 是影响心脏的最常见的淀粉样蛋白。ATTRwt 和 ATTRv 心脏淀粉样变性均可引发限制性心肌病，导致不良结局，包括心力衰竭和死亡 [1-3]。虽然心肌功能障碍经常被认为是心脏淀粉样变性患者呼吸困难和运动不耐受的主要机制，但越来越多的证据表明，包括肺功能异常在内的心外原因也可能是导致这些临床症状的原因 [1-3]。