Guigui Sarah A, Torres Christian, Escolar Esteban, Mihos Christos G
Echocardiography Laboratory, Columbia University Division of Cardiology, Mount Sinai Heart Institute, Miami Beach, FL, USA.
Columbia University Division of Cardiology, Mount Sinai Heart Institute, Miami Beach, FL, USA.
J Thorac Dis. 2022 Jun;14(6):2309-2325. doi: 10.21037/jtd-22-182.
The prevalence of hypertrophic cardiomyopathy (HCM) is estimated to be 1 in 200 to 500 individuals, with systolic anterior motion (SAM) of the mitral valve (MV) and left ventricular outflow tract (LVOT) obstruction present in 60% to 70%. In this narrative review, we aim to elucidate the pathophysiology of SAM-septal contact and LVOT obstruction in HCM by presenting a detailed review on the anatomy of the MV apparatus in HCM, examining the various existing theories pertaining to the SAM phenomenon as supported by cardiac imaging, and providing a critical assessment of management strategies for SAM in HCM.
A literature review was performed using PubMed, EMBASE, Ovid, and the Cochrane Library, of all scientific articles published through December 2021. A focus was placed on descriptive studies, reports correlating echocardiographic findings with pathologic diagnosis, and outcomes studies.
The pathophysiology of SAM involves the complex interplay between HCM morphology, MV apparatus anatomic abnormalities, and labile hemodynamic derangements. Echocardiography and cardiac magnetic resonance (CMR) vector flow mapping have identified drag forces, as opposed to the "Venturi effect", as the main hydraulic forces responsible for SAM. The degree of mitral regurgitation with SAM is variable, and its severity is correlated with degree of LVOT obstruction and outcomes. First line therapy for the amelioration of SAM and LVOT obstruction is medical therapy with beta-blockers, non-dihydropyridine calcium-channel blockers, and disopyramide, in conjunction with lifestyle modifications. In refractory cases septal reduction therapy is performed, which may be combined with a 'resect-plicate-release' procedure, anterior mitral leaflet extension, surgical edge-to-edge MV repair, anterior mitral leaflet retention plasty, or secondary chordal cutting.
Recent scientific advances in the field of HCM have allowed for a maturation of our understanding of the SAM phenomenon. Cardiac imaging plays a critical role in its diagnosis, treatment, and surveillance, and in our ability to apply the appropriate therapeutic regimens. The increasing prevalence of HCM places an emphasis on continued basic and clinical research to further improve outcomes for this challenging population.
肥厚型心肌病(HCM)的患病率估计为200至500人中1例,其中60%至70%存在二尖瓣(MV)收缩期前向运动(SAM)和左心室流出道(LVOT)梗阻。在本叙述性综述中,我们旨在通过详细回顾HCM中MV装置的解剖结构、研究心脏成像支持的与SAM现象相关的各种现有理论,并对HCM中SAM的管理策略进行批判性评估,来阐明HCM中SAM - 间隔接触和LVOT梗阻的病理生理学。
使用PubMed、EMBASE、Ovid和Cochrane图书馆对截至2021年12月发表的所有科学文章进行文献综述。重点关注描述性研究、将超声心动图结果与病理诊断相关联的报告以及结局研究。
SAM的病理生理学涉及HCM形态、MV装置解剖异常和不稳定血流动力学紊乱之间的复杂相互作用。超声心动图和心脏磁共振(CMR)向量流图已确定与“文丘里效应”相反的拖曳力是导致SAM的主要水力。伴有SAM的二尖瓣反流程度各不相同,其严重程度与LVOT梗阻程度和结局相关。改善SAM和LVOT梗阻的一线治疗是使用β受体阻滞剂、非二氢吡啶类钙通道阻滞剂和丙吡胺进行药物治疗,并结合生活方式改变。在难治性病例中,进行间隔减容治疗,可结合“切除 - 折叠 - 释放”手术、二尖瓣前叶延长、二尖瓣边对边手术修复、二尖瓣前叶保留成形术或二次腱索切断术。
HCM领域的最新科学进展使我们对SAM现象的理解更加成熟。心脏成像在其诊断、治疗和监测以及我们应用适当治疗方案的能力中起着关键作用。HCM患病率的增加强调了持续进行基础和临床研究以进一步改善这一具有挑战性人群的结局。