CAJ | 학술논문

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.
목적 탐토선천성쌍공이첨판(DOMV)기형적초성심동도표현급진단.방법 대20례선천성쌍공이첨판기형진행상규심장검사,중점관찰이첨판단축절면、심첨사강화량강절면급흉골방좌실장축절면,관찰판구형태、판막회성、혈류동역학정황급유무합병기타심혈관기형,병여수술비교.결과 성인환자13례,균반유명현혈류동역학이상,행이첨판치환술.학령환인1례,합병기부실간격결손,인명현혈류이상행이첨판치환술.2세이하환인6례,기중2례합병완전형심내막점결손반폐동맥고압、1례합병부분형심내막점결손+주동맥축착,행교치수술；무현저혈류이상2례,분별합병주동맥축착+동맥도관미폐、막부실간격결손；중도협착1례차합병유두기발육이상,인무괄용수술이채취보수치료.17례경수술탐사증실초성진단.결론 초성심동도기술능구준학지진단DOMV,병평개기혈류동역학개변,시림상진단적수선방법.
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.