超声心动图诊断小儿双孔二尖瓣19例

目的探讨小儿双孔二尖瓣(double orifice of mitral valve,DOMV)的超声心动图特征和临床特点。方法回顾性分析经超声心动图诊断的小儿DOMV 19例,分析其超声心动图特征、分类、功能评价、合并病变及预后判断。结果 19例DOMV患者中,均可经胸超声心动图明确诊断,二尖瓣短轴系列切面对诊断、分型及乳头肌功能评价非常重要,四腔心或两腔心切面也表现为典型的双瓣口征象,并且可以观察两组瓣口与腱索、乳头肌之间的连接关系。DOMV患者的预后取决于二尖瓣病变的严重程度及其并发的心脏畸形。16例表现为二尖瓣反流(1例已行外科整形术),8例表现为二尖瓣狭窄。12例合并先天性心脏病(包括室间隔缺损、房间隔缺损、主动脉缩窄等)。部分无症状的患儿,二尖瓣病变程度较轻,暂无需手术等处理,仍在随访中。结论超声心动图在DOMV患者诊断、分型、病变严重程度评估及随访中起到重要的作用。无症状患儿可随访观察。

Ultrasound diagnosis of double orifice of mitral valve in 19 children/infants

Objectives To explore the echocardiographic features and clinical characteristics of double orifice of mitral valve(DOMV)in children/infants.Methods The echocardiographic features,DOMV subtypes,mitral valve(MV)functional evaluation and patient outcome were retrospectively analyzed in 19 children/infants with DOMV.Results All the patients with DOMV were diagnosed by transthoracic echocardiography.Parasternal short-axis view of MV was the best view for showing the numbers of MV leaflets and orifices.Four-or two-chamber views could be used to define the chordal attachments to their respective papillary muscles.The management of DOMV depended on the severity of involvement and concomitant anomalies.Of 19 patients,16 cases were diagnosed with mitral regurgitation and surgical approach had been implemented in one patient,8 cases were diagnosed with mitral stenosis.Twelve cases were associated with congenital heart defect,including ventricular septal defect,atrial septal defect and coarctation of the aorta.Most particular asymptomatic patients needed neither medical therapies nor surgical correction and may be followed up using echocardiographic examinations.Conclusions Echocardiography plays an important role in diagnosis,classification,evaluation and follow-up in DOMV.Asymptomatic cases should be followed up.