超声心动图诊断先天性双孔二尖瓣畸形

Echocardiographic diagnosis of congenital double orifice of mitral valve deformmity

目的探讨先天性双孔二尖瓣（DOMV）畸形的超声心动图表现及诊断。方法对20例先天性双孔二尖瓣畸形进行常规心脏检查，重点观察二尖瓣短轴切面、心尖四腔和两腔切面及胸骨旁左室长轴切面，观察瓣口形态、瓣膜回声、血流动力学情况及有无合并其他心血管畸形，并与手术比较。结果成人患者13例，均伴有明显血流动力学异常，行二尖瓣置换术。学龄患儿1例，合并肌部室间隔缺损，因明显血流异常行二尖瓣置换术。2岁以下患儿6例，其中2例合并完全型心内膜垫缺损伴肺动脉高压、1例合并部分型心内膜垫缺损＋主动脉缩窄，行矫治手术；无显著血流异常2例，分别合并主动脉缩窄＋动脉导管未闭、膜部室间隔缺损；重度狭窄1例且合并乳头肌发育异常，因无适用手术而采取保守治疗。17例经手术探查证实超声诊断。结论超声心动图技术能够准确地诊断DOMV，并评价其血流动力学改变，是临床诊断的首选方法。

Objective To discuss the morphological features and diagnosis of congenial double orifice mitral valve （DOMV） deformity by echocardiography. Methods Twenty consecutive patients were examined. The changes of the morphology and flow dynamics of DOMV were studied,and other congenital cardiac abnormalities were also observed. These results were compared with those of surgery. Results Thirteen cases of adult patients were all with significant hemodynamic abnormalities,and underwent mitral replacement. One case of school child with muscular ventricular septal defect underwent mitral replacement because of obvious hemodynamic changes. Six cases were under 2 years old, 2 cases with complete endocardial cushion defect accompanied with pulmonary hypertension, 1 case with partial endocardial cushion defect accompanied with coarctation of the aorta underwent corrective operation. No significant hemodynamic abnormalities in 2 cases, 1 case was associated with coarctation of the aorta and patent ductus arteriosus,the other patient with membranous ventricular septal defect. Severe stenosis in 1 case with papillary muscle dysplasia, because there was no suitable operation and take conservative therapy. Seventeen cases were confirmed by the results of open-heart surgery. Conclusions Echocardiography can provide an accurate method to diagnose DOMV and evaluate the hemodynamic changes.