Kasiakogias Alexandros, Ragavan Aaraby, Halliday Brian P
Inherited Cardiac Conditions Care Group, Royal Brompton and Harefield Hospitals, Part of Guy's and St Thomas' NHS Foundation Trust, London, UK.
National Heart and Lung Institute, Imperial College London, London, UK.
Curr Heart Fail Rep. 2023 Dec;20(6):542-554. doi: 10.1007/s11897-023-00636-8. Epub 2023 Nov 24.
With the widespread implementation of contemporary disease-modifying heart failure therapy, the rates of normalization of ejection fraction are continuously increasing. The TRED-HF trial confirmed that heart failure remission rather than complete recovery is typical in patients with dilated cardiomyopathy who respond to therapy. The present review outlines key points related to the management and knowledge gaps of this growing patient group, focusing on patients with non-ischaemic dilated cardiomyopathy.
There is substantial heterogeneity among patients with normalized ejection fraction. The specific etiology is likely to affect the outcome, although a multiple-hit phenotype is frequent and may not be identified without comprehensive characterization. A monogenic or polygenic genetic susceptibility is common. Ongoing pathophysiological processes may be unraveled with advanced cardiac imaging, biomarkers, multi-omics, and machine learning technologies. There are limited studies that have investigated the withdrawal of specific heart failure therapies in these patients. Diuretics may be safely withdrawn if there is no evidence of congestion, while continued therapy with at least some disease-modifying therapy is likely to be required to reduce myocardial workload and sustain remission for the vast majority. Understanding the underlying disease mechanisms of patients with normalized ejection fraction is crucial in identifying markers of myocardial relapse and guiding individualized therapy in the future. Ongoing clinical trials should inform personalized approaches to therapy.
综述目的：随着当代改善病情的心力衰竭治疗方法的广泛应用，射血分数正常化的比例不断增加。TRED-HF试验证实，在对治疗有反应的扩张型心肌病患者中，心力衰竭缓解而非完全恢复是典型情况。本综述概述了与这一不断增加的患者群体的管理相关的要点以及知识空白，重点关注非缺血性扩张型心肌病患者。
最新发现：射血分数正常化的患者之间存在很大的异质性。具体病因可能会影响预后，尽管多重打击表型很常见，若不进行全面表征可能无法识别。单基因或多基因遗传易感性很常见。先进的心脏成像、生物标志物、多组学和机器学习技术可能会揭示持续存在的病理生理过程。在这些患者中，研究特定心力衰竭治疗方法撤药的研究有限。如果没有充血证据，利尿剂可能可以安全撤药，而对于绝大多数患者，可能需要继续至少一些改善病情的治疗以减轻心肌负荷并维持缓解状态。了解射血分数正常化患者的潜在疾病机制对于识别心肌复发标志物和指导未来个体化治疗至关重要。正在进行的临床试验应为个性化治疗方法提供依据。
