先天性二尖瓣畸形的超声心动图特征

Echocardiographic features of congenital mitral valve anomalies

目的探讨不同类型的先天性二尖瓣畸形的超声心动图特征。方法根据病史、超声心动图、心导管和外科手术记录,分析15例不同类型先天性二尖瓣及瓣下结构畸形超声心动图特征,11例进行了外科手术治疗,1例进行了动脉导管未闭封堵治疗。结果降落伞二尖瓣畸形6例,超声心动图特征是发育不良的二尖瓣通过短的腱索附着到单组左心室乳头肌上,二尖瓣活动受限。双孔二尖瓣畸形4例,表现为左侧房室瓣倍增,显示两个分开的瓣口和各自的瓣下支持结构,合并不同程度的二尖瓣关闭不全。1例先天性二尖瓣狭窄表现为两组乳头肌,前外侧乳头肌为优势乳头肌,后中乳头肌较小。1例显示左心室两组乳头肌和二尖瓣口旋转。3例显示左心室明显的三组乳头肌和三个联合部位的二尖瓣畸形。结论超声心动图评价先天性二尖瓣畸形有显著优越性,可以清晰地显示瓣膜厚度和腱索的附着情况,乳头肌的位置、数量、大小和相互关系,对先天性二尖瓣狭窄的诊断具有重要价值。

Objective To summarize the echocardiographic features of a wide spectrum of congenital mitral valve anomalies. Methods The medical records, echocardiograms, cardiac catheterization studies, and surgical reports were reviewed. The mitral valve anomalies evaluated in the study included parachute mitral valve, double orifice mitral valve, congenital mitral stenosis with 2 papillary muscles, anomalous papillary muscle rotation, and 3 commissures and papillary muscles in 15 cases. Surgeries were performed in 11 patients, and 1 patient underwent transcatheter closure of the patent ductus arteriosus. Results The echocardiograms of 6 cases of parachute mitral valve were characterized by a hypoplastic mitral valve with short chordal attachments to a single posterior medial papillary muscle. The mitral valve demonstrated restricted motion. The double orifice mitral valve were featured by two separate mitral valve orifice, with each suborifice supported by its own tension apparatus in 4 patients. Asymmetric hypoplastic mitral valve stenosis with two papillary muscles was found in 1 patient with short and unbalanced chordal attachments to the anterior lateral major papillary muscle. Anomalous papillary muscle rotation was found in 1 patient. Mirtal anomaly with 3 major commissures and 3 papillary muscles was found in 3 patients. Conclusions Echocardiography offers clear demonstration of the mitral valve thickness and chordal attachments, and allows visualization of the position and the number of the papillary muscles and interpapillary spaces for evaluation of the mobility of the valve leaflets, therefore can be a valuable diagnostic modality for congenital mitral valve anomalies, especially congenital mitral stenosis.