Coronary arterial vasculature in the pathophysiology of hypertrophic cardiomyopathy.
Coronary arterial vasculature in the pathophysiology of hypertrophic cardiomyopathy.
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肥厚性心肌病的病理生理学中的冠状动脉血管。
DOI：
10.1007/s00424-018-2224-y
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发表时间：
2019-05
期刊：
影响因子：
--
通讯作者：
Wolska BM
中科院分区：
文献类型：
--
作者：
Marszalek RJ;John Solaro R;Wolska BM
冠状血管系统的改变可能与能量需求和能量供应之间的不匹配有关，并且对于引发导致症状性肥厚性心肌病的级联事件至关重要。针对早期事件，特别是血管重塑，可能是开发有效治疗方法的关键方法。我们对肥厚型心肌病的理解始于早期生物物理学研究的结果，随后进行了查明突变起源的遗传分析，现在涉及此类突变的代谢和血流相关后果的成像。微血管功能障碍一直是遗传性心肌病成像中的一个持续热点话题，其特点是其组织学上显着的重塑，并已被证明是确定这些患者预后的强大资产，并启发科学家了解可能在发育过程早期开始的潜在病理生理级联反应。在这里，我们讨论导致微血管功能障碍的机制过程、其与血管形态变化的相关性以及其对疾病进展的贡献仍然存在的问题。
Alterations in the coronary vascular system are likely associated with a mismatch between energy demand and energy supply and critical in triggering the cascade of events that leads to symptomatic hypertrophic cardiomyopathy. Targeting the early events, particularly vascular remodeling, may be a key approach to developing effective treatments. Improvement in our understanding of hypertrophic cardiomyopathy began with the results of early biophysical studies, proceeded to genetic analyses pinpointing the mutational origin, and now pertains to imaging of the metabolic and flow-related consequences of such mutations. Microvascular dysfunction has been an ongoing hot topic in the imaging of genetic cardiomyopathies marked by its histologically significant remodeling and has proven to be a powerful asset in determining prognosis for these patients as well as enlightening scientists on a potential pathophysiological cascade that may begin early during the developmental process. Here, we discuss questions that continue to remain on the mechanistic processes leading to microvascular dysfunction, its correlation to the morphological changes in the vessels, and its contribution to disease progression.
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