医学
肥厚性心肌病
心源性猝死
危险分层
疾病
重症监护医学
心脏病学
内科学
猝死
作者
Sneha Annie Sebastian,Venkatesh Panthangi,K.K. Singh,Swetha Rayaroth,Aditi Gupta,Darshan Shantharam,Banan Qasim Rasool,Inderbir Padda,Edzel Lorraine Co,Gurpreet Johal
标识
DOI:10.1016/j.cpcardiol.2022.101552
摘要
Hypertrophic cardiomyopathy (HCM) is a disease involving the cardiac sarcomere. It is associated with various disease-causing gene mutations and phenotypic expressions, managed with different therapies with variable prognoses. The heterogeneity of the disease is evident in the fact that it burdens patients of all ages. HCM is the most prevalent cause of sudden death in athletes. However, several technological advancements and therapeutic options have reduced mortality in patients with HCM to 0.5% per year. In addition, rapid advances in our knowledge of the molecular defects accountable for HCM have strengthened our awareness of the disorder and recommended new approaches to the assessment of prognosis. Despite all these evolutions, a small subgroup of patients with HCM will experience sudden cardiac death, and risk stratification remains a critical challenge. This review provides a practical guide to the updated recommendations for patients with HCM, including clinical updates for diagnosis, family screening, clinical imaging, risk stratification, and management.
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