Department of Cardiology, Azienda Sanitaria Universitaria Integrata Giuliano Isontina (ASUGI), University of Trieste, Trieste, Italy.
Department of Cardiovascular Science, Faculty of Life Science and Medicine, King's College London, London, UK.
Minerva Cardiol Angiol. 2022 Apr;70(2):171-188. doi: 10.23736/S2724-5683.21.05736-7. Epub 2021 Aug 2.
Non-ischemic dilated cardiomyopathy (NI-DCM) represents a specific etiology of systolic heart failure that usually affect young individuals with a genetic background in up to 40% of cases. Behind the term NI-DCM there is a spectrum of different diseases, and an accurate etiological classification appears pivotal for the clinical management and prognostic stratification of these patients. In the last years the prognosis of NI-DCM patients dramatically improved thanks to the progresses in medical treatment/ device therapy and earlier diagnosis especially in familial context. In this review we summarize the actual state of art in the management of these patients. In the era of precision medicine, a lot of progresses have been made to expand our knowledge on the management of NI-DCM patients. A complex interaction between genotype and external triggers is the main determinant of the clinical phenotype in NI-DCM, and a lot of efforts must be done by clinicians to systematically rule out all the possible causes involved in the pathogenesis. Progresses in cardiac imaging and familial screening led us to detect subtle abnormalities in the initial phase of the disease and also helped us to furtherly stratify the prognosis and arrhythmic risk of these patients. It is plausible that a more precise etiological classification will be needed in the near future. NI-DCM contains a spectrum of different diseases. Proper etiological classification, early diagnosis and strict follow-up are essential to tailor care of these patients.
非缺血性扩张型心肌病(NI-DCM)是一种以收缩性心力衰竭为特征的特定病因,通常影响有遗传背景的年轻人,其中多达 40%的病例有遗传背景。在 NI-DCM 这一术语背后,存在着一系列不同的疾病,准确的病因分类对于这些患者的临床管理和预后分层至关重要。近年来,由于医疗/器械治疗的进步和早期诊断(尤其是在家族性背景下),NI-DCM 患者的预后有了显著改善。在精准医学时代,我们在 NI-DCM 患者的管理方面取得了许多进展。基因型和外部触发因素之间的复杂相互作用是 NI-DCM 临床表型的主要决定因素,临床医生必须做出大量努力,系统排除发病机制中涉及的所有可能原因。心脏成像和家族筛查方面的进展使我们能够在疾病的初始阶段检测到细微的异常,并进一步对这些患者的预后和心律失常风险进行分层。在不久的将来,可能需要更精确的病因分类。NI-DCM 包含了一系列不同的疾病。适当的病因分类、早期诊断和严格随访对于这些患者的护理至关重要。