CAJ | 학술논문

目的：分析Berry综合征彩色多普勒超声心动图图像特征，评价多普勒超声心动图对Berry综合征的诊断价值，分析超声心动图漏误诊的原因，旨在提高超声心动图技术对Berry综合征诊断的准确性。方法21例经心外科手术或经核磁共振（M RI）检查明确为Berry综合征的患儿为研究对象，对其超声心动图检查结果进行回顾性分析。结果Berry综合征畸形主要包括远端主肺动脉间隔缺损（21例）、右肺动脉异常起源于升主动脉（21例）、主动脉弓离断（A型19例）及主动脉缩窄（2例）、室间隔完整（21例）；最常见的合并畸形有肺动脉高压（21例）、动脉导管未闭（19例）、房间隔缺损（9例）、卵圆孔未闭（4例）、主动脉瓣狭窄（1例）、二尖瓣狭窄（1例）。21例患儿中，超声诊断符合17例（占81．0％）；超声漏、误诊4例（占19．0％），其中2例漏诊右肺动脉异常起源于升主动脉、1例漏诊主肺动脉间隔缺损及右肺动脉异常起源于升主动脉；1例主动脉弓中断A型误诊为B型。结论多普勒超声心动图技术可以较准确地诊断Berry综合征的各种组合畸形，但容易漏诊，必要时可行M RI检查。
목적：분석Berry종합정채색다보륵초성심동도도상특정，평개다보륵초성심동도대Berry종합정적진단개치，분석초성심동도루오진적원인，지재제고초성심동도기술대Berry종합정진단적준학성。방법21례경심외과수술혹경핵자공진（M RI）검사명학위Berry종합정적환인위연구대상，대기초성심동도검사결과진행회고성분석。결과Berry종합정기형주요포괄원단주폐동맥간격결손（21례）、우폐동맥이상기원우승주동맥（21례）、주동맥궁리단（A형19례）급주동맥축착（2례）、실간격완정（21례）；최상견적합병기형유폐동맥고압（21례）、동맥도관미폐（19례）、방간격결손（9례）、란원공미폐（4례）、주동맥판협착（1례）、이첨판협착（1례）。21례환인중，초성진단부합17례（점81．0％）；초성루、오진4례（점19．0％），기중2례루진우폐동맥이상기원우승주동맥、1례루진주폐동맥간격결손급우폐동맥이상기원우승주동맥；1례주동맥궁중단A형오진위B형。결론다보륵초성심동도기술가이교준학지진단Berry종합정적각충조합기형，단용역루진，필요시가행M RI검사。
Objective To review the imaging characteristics of Berry syndrome and to evaluate the accuracy of the diag‐nosis by Doppler echocardiography ,so as to improve the initial echocardiographic diagnosis .Methods Doppler echocar‐diography was performed and these images were compared with operative or magnetic resonance imaging (MRI) findings in 21 children with Berry syndrome .Results The malformations of Berry syndrome consisted of the distal aortopulmonary septal defect (21 cases) ,aortic origin of the right pulmonary artery (21 cases) ,interruption of aortic arch (19 cases) ,co‐arctation of the aorta (2 cases) ,and intact ventricular septum (21 cases) .The most common associated malformations in‐cluded pulmanary hypertension (21 cases) ,patent ductus arteriosus (19 cases) ,atrial septal defect (9 cases) ,patent fora‐men ovale (4 cases) ,aortic valve stenosis (1 case) ,and mitral valve stenosis (1 case) .17 of 21 cases (81 .0% ) were diag‐nosed correctly .In the remaining 4 cases (19 .0% ) ,2 cases with aortic origin of the right pulmonary artery and 1 case with aortopulmonary septal defect and aortic origin of the right pulmonary artery were missed diagnosis ,1 case with type A of interruption of aortic arch was misdiagnosed as type B .Conclusion All containing malformations of Berry syndrome could be diagnosed accurately by Doppler echocardiography .