Discrete Subaortic Stenosis: Perspective Roadmap to a Complex Disease.
Discrete Subaortic Stenosis: Perspective Roadmap to a Complex Disease.
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DOI：
10.3389/fcvm.2018.00122
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发表时间：
2018
影响因子：
3.6
通讯作者：
Sucosky P
中科院分区：
文献类型：
--
作者：
Massé DD;Shar JA;Brown KN;Keswani SG;Grande-Allen KJ;Sucosky P
离散性主动脉瓣下狭窄(DSS)是一种先天性心脏病，导致纤维膜组织的形成，导致左室流出道(LVOT)压力梯度增加。虽然外科手术切除膜在消除梗阻方面取得了一些成功，但它带来了与麻醉、胸骨切开和心脏搭桥相关的重大风险，而且它仍然与高复发率有关。虽然最初提出了遗传病因，但DSS与左心室(LV)几何异常之间的关联为血流动力学病因提供了更多支持，即先天性或手术后左室流出道几何紊乱可在隔壁产生异常剪力，进而触发纤维化反应。验证这一假说的病因并了解剪切力改变导致左心室流出道纤维化的机制生物学过程是主要的知识空白。这篇前瞻性的论文描述了DSS的知识现状，阐明了对一个鲜为人知的重要病理过程的机制洞察的研究需要，并提出了几种旨在阐明DSS发病机制的潜在机械生物学协同作用的策略。拟议的路线图有可能通过确定预防纤维化损害的早期目标来改善DSS的管理，并可能被证明对与血流改变相关的其他纤维性心血管疾病有益。
Discrete subaortic stenosis (DSS) is a congenital heart disease that results in the formation of a fibro-membranous tissue, causing an increased pressure gradient in the left ventricular outflow tract (LVOT). While surgical resection of the membrane has shown some success in eliminating the obstruction, it poses significant risks associated with anesthesia, sternotomy, and heart bypass, and it remains associated with a high rate of recurrence. Although a genetic etiology had been initially proposed, the association between DSS and left ventricle (LV) geometrical abnormalities has provided more support to a hemodynamic etiology by which congenital or post-surgical LVOT geometric derangements could generate abnormal shear forces on the septal wall, triggering in turn a fibrotic response. Validating this hypothetical etiology and understanding the mechanobiological processes by which altered shear forces induce fibrosis in the LVOT are major knowledge gaps. This perspective paper describes the current state of knowledge of DSS, articulates the research needs to yield mechanistic insights into a significant pathologic process that is poorly understood, and proposes several strategies aimed at elucidating the potential mechanobiological synergies responsible for DSS pathogenesis. The proposed roadmap has the potential to improve DSS management by identifying early targets for prevention of the fibrotic lesion, and may also prove beneficial in other fibrotic cardiovascular diseases associated with altered flow.
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影响因子：
2.4
作者：
Bazan, Ovandir;Ortiz, Jayme P.;Yanagihara, Jurandir I.
通讯作者：
Yanagihara, Jurandir I.
DOI：
10.1111/j.1439-0442.2006.00865.x
发表时间：
2006-09-01
期刊：
JOURNAL OF VETERINARY MEDICINE SERIES A-PHYSIOLOGY PATHOLOGY CLINICAL MEDICINE
影响因子：
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作者：
Chetboul, V.;Trolle, J. M.;Pouchelon, J. -L.
通讯作者：
Pouchelon, J. -L.
基质硬度与肝细胞生长因子相结合调节内皮细胞行为
影响因子：
6.2
作者：
Chang, Hao;Liu, Xi-qiu;Hu, Mi;Zhang, He;Li, Bo-chao;Ren, Ke-feng;Boudou, Thomas;Albiges-Rizo, Corinne;Picart, Catherine;Ji, Jian
通讯作者：
Ji, Jian
影响因子：
2.6
作者：
Donald, Julia S.;Naimo, Phillip S.;Konstantinov, Igor E.
通讯作者：
Konstantinov, Igor E.
DOI：
10.1177/2150135113485760
发表时间：
2013-07-01
影响因子：
0.9
作者：
Barboza, Laura A.;de Maria Garcia, Flor;Castaneda, Aldo R.
通讯作者：
Castaneda, Aldo R.