Baird Christopher W, Marx Gerald R, Borisuk Michele, Emani Sitram, del Nido Pedro J
Boston Children's Hospital, Boston, MA, USA.
Cardiovasc Eng Technol. 2015 Jun;6(2):167-73. doi: 10.1007/s13239-015-0223-0. Epub 2015 Apr 17.
The spectrum of congenital mitral valve stenosis (MS) consists of a complex of defects that result in obstruction to left ventricular inflow. This spectrum includes patients with underdeveloped left heart structures (Fig. 1) to those with isolated congenital MS. The specific mitral valve defects can further be divided into categories based on the relationship to the mitral valve annulus including valvar, supravalvar and subvalvar components. Clinically, these patients present based on the degree of obstruction, associated mitral regurgitation, secondary pulmonary hypertension, associated lung disease and/or associated cardiac lesions. There are a number of factors that contribute to the successful outcomes in these patients including pre-operative imaging, aggressive surgical techniques and peri-operative management.
先天性二尖瓣狭窄(MS)的谱系包括一系列导致左心室流入道梗阻的复杂缺陷。该谱系涵盖左心结构发育不全的患者(图1)至孤立性先天性MS患者。特定的二尖瓣缺陷可根据与二尖瓣环的关系进一步分为瓣叶、瓣上和瓣下成分等类别。临床上,这些患者根据梗阻程度、相关二尖瓣反流、继发性肺动脉高压、相关肺部疾病和/或相关心脏病变表现各异。有许多因素有助于这些患者获得成功的治疗结果,包括术前影像学检查、积极的手术技术和围手术期管理。