Myocardial factor revisited: The importance of myocardial fibrosis in adults with congenital heart disease.

Myocardial factor revisited: The importance of myocardial fibrosis in adults with congenital heart disease.
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DOI:
10.1016/j.ijcard.2015.04.064
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发表时间:
2015-06-15
影响因子:
3.5
通讯作者:
Burchill LJ
Burchill LJ
中科院分区:
医学2区
文献类型:
--
作者:
Broberg CS;Burchill LJ

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先天性心脏病手术的先驱们观察到,尽管手术修复成功,运动能力并没有恢复到正常水平,这导致一些人引用了“心肌因素”的作用。他们强调,心肌功能的残余改变对患者的长期前景具有重要意义。在实现他们的早期观察,今天的成人先天性心脏病(ACHD)的人口显示出公认的心脏衰竭的特点,即使在没有明确的残留解剖或血流动力学异常的患者,证明心肌在他们的发病率和死亡率的重要作用。而“心肌因素”是一个难以捉摸的概念,在早期的历史,先天性心脏病护理,我们现在有成像技术来检测和量化这样一个因素-心肌纤维化。了解心肌纤维化作为各种先天性病变的最终共同途径的重要性,为临床心力衰竭的研究和治疗提供了一个框架。虽然典型的心力衰竭药理学应该减少或减弱纤维化,但在ACHD中使用标准药物治疗显示有意义的改善的努力一再不足。本文讨论了心肌纤维化和功能的重要性,目前ACHD心肌纤维化的证据及其对研究和治疗的意义。
Pioneers in congenital heart surgery observed that exercise capacity did not return to normal levels despite successful surgical repair, leading some to cite a “myocardial factor” playing a role. They conjectured that residual alterations in myocardial function would be significant for patients’ long-term outlook. In fulfillment of their early observations, today’s adult congenital heart disease (ACHD) population shows well-recognized features of heart failure, even among patients without clear residual anatomic or hemodynamic abnormalities, demonstrating the vital role of the myocardium in their morbidity and mortality. Whereas the ‘myocardial factor’ was an elusive concept in the early history of congenital heart care, we now have imaging techniques to detect and quantify one such factor – myocardial fibrosis. Understanding the importance of myocardial fibrosis as a final common pathway in a variety of congenital lesions provides a framework for both the study and treatment of clinical heart failure in this context. While typical heart failure pharmacology should reduce or attenuate fibrogenesis, efforts to show meaningful improvements with standard pharmacotherapy in ACHD repeatedly fall short. This paper considers the importance of myocardial fibrosis and function, the current body of evidence for myocardial fibrosis in ACHD, and its implications for research and treatment.
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