CAJ | 학술논문

目的分析先天性左冠状动脉主干闭锁(CLMCA-A)4例患儿的临床诊断及治疗特点并进行相关文献复习.方法回顾性分析2010年6月至2012年6月北京安贞医院明确诊断为CLMCA-A的4例患儿的临床表现、超声心动图、心电图及选择性心血管造影特点并复习文献,总结本病的临床表现、诊断及治疗特点.结果 4例患儿发病年龄3个月～2岁,3例经血管造影检查证实,1例经冠状动脉CT血管造影(CTA)证实.患儿临床表现多以心功能不全起病,常见有多汗、气促、喂养困难、反复肺炎、活动耐量减低等.患儿心电图:Ⅰ、AVL、V4～V6导联异常Q波及ST-T低平或倒置.患儿超声心动图均显示左心室扩大,左心室收缩功能正常或轻度减低,二尖瓣中到大量反流,左心室肌小梁增多,左冠状动脉发育纤细,其内可见逆向血流;右冠状动脉略增粗;可见冠状动脉侧支循环.3例患儿行主动脉根部造影检查,示右冠状动脉发自右冠窦,右冠状动脉略增粗,左冠状动脉主干呈盲端,内径纤细,直径1.1 ～2.0 mm.左冠状动脉未与主动脉连接.造影剂显影顺序为右冠状动脉、冠状动脉侧支、左冠状动脉远端分支、左冠状动脉主干.2例患儿行冠状动脉CTA检查,1例明确诊断.4例患儿均在密切随访中进行抗心功能不全治疗,临床症状稳定.结论 CLMCA-A并非罕见,临床表现易与扩张型心肌病、婴儿心内膜弹力纤维增生症、先天性瓣膜病、左冠状动脉异常起源于肺动脉等混淆.4例CLMCA-A提示对于心脏扩大、心功能异常、伴有二尖瓣中度以上反流、心肌致密化不全改变、心电图出现异常Q波的婴幼儿,应注意冠状动脉的先天发育异常,尤其CLMCA-A应引起儿科医生的重视.
목적 분석선천성좌관상동맥주간폐쇄(CLMCA-A)4례환인적림상진단급치료특점병진행상관문헌복습.방법 회고성분석2010년6월지2012년6월북경안정의원명학진단위CLMCA-A적4례환인적림상표현、초성심동도、심전도급선택성심혈관조영특점병복습문헌,총결본병적림상표현、진단급치료특점.결과 4례환인발병년령3개월～2세,3례경혈관조영검사증실,1례경관상동맥CT혈관조영(CTA)증실.환인림상표현다이심공능불전기병,상견유다한、기촉、위양곤난、반복폐염、활동내량감저등.환인심전도:Ⅰ、AVL、V4～V6도련이상Q파급ST-T저평혹도치.환인초성심동도균현시좌심실확대,좌심실수축공능정상혹경도감저,이첨판중도대량반류,좌심실기소량증다,좌관상동맥발육섬세,기내가견역향혈류;우관상동맥략증조;가견관상동맥측지순배.3례환인행주동맥근부조영검사,시우관상동맥발자우관두,우관상동맥략증조,좌관상동맥주간정맹단,내경섬세,직경1.1 ～2.0 mm.좌관상동맥미여주동맥련접.조영제현영순서위우관상동맥、관상동맥측지、좌관상동맥원단분지、좌관상동맥주간.2례환인행관상동맥CTA검사,1례명학진단.4례환인균재밀절수방중진행항심공능불전치료,림상증상은정.결론 CLMCA-A병비한견,림상표현역여확장형심기병、영인심내막탄력섬유증생증、선천성판막병、좌관상동맥이상기원우폐동맥등혼효.4례CLMCA-A제시대우심장확대、심공능이상、반유이첨판중도이상반류、심기치밀화불전개변、심전도출현이상Q파적영유인,응주의관상동맥적선천발육이상,우기CLMCA-A응인기인과의생적중시.
Objective To investigate the clinical manifestations and treatment of congenital atresia of the left main coronary artery (CLMCA-A).Method Four patients were diagnosed to have CLMCA-A from June 2010 to June 2012 in Beijing Anzhen Hospital.Clinical manifestations,ultrasound,ECG and angiographic characteristics were analyzed and summarized.Result Of the 4 cases,age of onset was 3 months to 2 yrs.Three cases were diagnosed by angiography,and 1 case by CTA.All 4 cases had chronic heart failure symptoms and signs,such as sweating,shortness of breath,easily choked by milk,predispose to pneumonia,activity intolerance.ECG showed abnormal Q wave and other ischemic signs such as ST-T segment depression.Ultrasonography showed left ventricular enlargement,left ventricular systolic function was normal or slightly reduced,and there was moderate to large amount of mitral valve regurgitation.Left ventricular trabeculations increased.Coronary collateral circulation increased.Left coronary artery appeared to be slender and disconnected with left coronary artery sinus.Aortic root angiography was the golden diagnostic standard.Angiography was performed in 3 patients and showed that left main coronary artery had a blind end,diameter 1.1-2.0 mm.The right coronary artery was found rising from the right coronary sinus and visible on coronary collateral circulation.Contrast agent developing sequence:right coronary arterycollateral vessels-left coronary artery distal branches-left main coronary artery.CTA exam was performed in 2 cases and in 1 case the diagnoses was confirmed.All the 4 patients are currently in the close follow-up,digoxin and diuretics were taken everyday and clinical symptoms were improved.Conclusion CLMCA-A is not rare,its clinical manifestations should be differentiated from those of cardiomyopathy,endocardial fibroelastosis,congenital valvular disease and abnormal left coronary artery originating from pulmonary artery etc.For pediatric patients with cardiac enlargement,abnormal heart function,mitral valve regurgitation etc,attention must be paid to consider the developmental abnormality of coronary artery,particularly the CLMCA-A diagnosis.