Merlo Marco, Gigli Marta, Poli Stefano, Stolfo Davide, Brun Francesca, Lardieri Gerardina, Pinamonti Bruno, Zecchin Massimo, Pivetta Alberto, Vitrella Giancarlo, Di Lenarda Andrea, Sinagra Gianfranco
Dipartimento Cardiovascolare e Scuola di Specializzazione in Malattie dell'Apparato Cardiovascolare, A.O.U. "Ospedali Riuniti" e Università degli Studi, Trieste.
G Ital Cardiol (Rome). 2016 Jan;17(1):15-23. doi: 10.1714/2140.23184.
Dilated cardiomyopathy (DCM) is a relatively rare primary heart muscle disease with genetic or post-inflammatory etiology. In the last decade, the incidence and prevalence of the disease have significantly increased as a consequence of an earlier diagnosis supported by extensive familial screening programs and by the improvement in diagnostic techniques. Moreover, current therapeutic strategies have deeply modified the prognosis of DCM with a dramatic reduction in mortality. A significant number of patients with DCM present an impressive response to pharmacological and non-pharmacological therapy in terms of left ventricular reverse remodeling (reduction in ventricular size with improvement of systolic function), which confers a more favorable prognosis in the long term. However, the identification of patients with an increased likelihood of improvement after therapeutic optimization remains a challenging issue; in particular the assessment of arrhythmic risk carries important implications. Finally, the long-term follow-up of patients showing a significant left ventricular functional recovery under optimal treatment is still poorly known. Hence, the aim of the present review is to provide an insight into the clinical evolution/long-term follow-up of DCM, which should be actually considered a dynamic process rather than a static and chronic disease. Left ventricular reverse remodeling should be considered a key therapeutic goal, mostly associated with a long-standing recovery, but cannot be considered the expression of permanent "healing", confirming the need for a systematic and careful follow-up over time in this setting.
扩张型心肌病（DCM）是一种相对罕见的原发性心肌疾病，病因包括遗传或炎症后因素。在过去十年中，由于广泛的家族筛查计划以及诊断技术的改进使得早期诊断得以实现，该疾病的发病率和患病率显著上升。此外，当前的治疗策略已深刻改变了DCM的预后，死亡率大幅降低。相当数量的DCM患者在左心室逆向重构（心室大小减小且收缩功能改善）方面对药物和非药物治疗表现出显著反应，这在长期来看赋予了更有利的预后。然而，识别治疗优化后改善可能性增加的患者仍然是一个具有挑战性的问题；特别是心律失常风险的评估具有重要意义。最后，对于在最佳治疗下左心室功能显著恢复的患者的长期随访情况仍知之甚少。因此，本综述的目的是深入了解DCM的临床演变/长期随访情况，实际上应将其视为一个动态过程，而非一种静态的慢性疾病。左心室逆向重构应被视为一个关键的治疗目标，大多与长期恢复相关，但不能被视为永久性“治愈”的表现，这证实了在此情况下需要随着时间进行系统且仔细的随访。
