医学
转甲状腺素
心力衰竭
心肌病
疾病
内科学
淀粉样蛋白(真菌学)
心脏病学
限制性心肌病
重症监护医学
病理
作者
Hritvik Jain,Murali Mohan Rama Krishna Reddy,Rohit Chandra Dey,Jyoti Jain,Zaid Shakhatreh,Sarbagya Manandhar,Purushottam Neupane,Madeeha Subhan Waleed,Rukesh Yadav,Biki Kumar Sah,Rukam Mahawa
标识
DOI:10.1016/j.cpcardiol.2023.102057
摘要
Transthyretin amyloid cardiomyopathy (ATTR-CM) is a mutation-based genetic disorder due to the accumulation of unstable transthyretin protein and presents with symptoms of congestive heart failure (CHF) and numerous extracardiac symptoms like carpal tunnel syndrome and neuropathy. Two subtypes of ATTR-CM are hereditary and wild-type, both of which have different risk factors, gender prevalence and major clinical symptoms. Timely usage of imaging modalities like echocardiography, cardiac magnetic imaging resonance, and cardiac scintigraphy has made it possible to suspect ATTR-CM in patients presenting with CHF. Management of ATTR-CM includes appropriate treatment for heart failure for symptomatic relief, prevention of arrhythmias and heart transplantation for nonresponders. With the recent approval of tafamidis in the successful management of ATTR-CM, numerous potential therapeutic points have been identified to stop or delay the progression of ATTR-CM. This article aims to provide a comprehensive review of ATTR-CM and insights into its novel therapeutics and upcoming treatments.
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