影像学诊断先天性左冠状动脉主干闭锁

目的探讨影像学诊断先天性左冠状动脉主干闭锁(LMCAA)的可行性。方法回顾性分析6例LMCAA患者,男3例、女3例,1例成人、5例婴幼儿。6例均接受超声心动图、多排螺旋CT(MDCT)及心血管造影检查。结果 1例超声提示左冠状动脉内径偏细,起源显示欠清晰,不除外左冠状动脉异常起源于肺动脉;1例超声提示左冠状动脉近段发育细,似壁内走行、开口狭窄;另4例超声仅提示二尖瓣脱垂并大量反流,冠状动脉未探及异常。5例经MDCT明确诊断为左冠状动脉主干闭锁,1例不除外左冠状动脉主干开口重度狭窄或闭锁。6例经心血管造影检查均明确诊断为LMCAA。结论 LMCAA属罕见先天性心脏病,超声可提示该病;CT对多数患者可明确诊断;心血管造影检查是诊断LMCAA的金标准。

Imaging diagnosis of congenital left main coronary artery atresia

Objective To explore the feasibility of imaging diagnosis of congenital left main coronary artery atresia (LMCAA). Methods Data of 6 patients with LMCAA, including 3 males and 3 females, 5 infants and 1 adult were retrospectively analyzed. All 6 patients underwent echocardiography, multidetector CT (MDCT) and cardiovascular angiography. Results Echocardiography showed small diameter of left coronary artery without clear ostium, could not exclude the abnormal origin of left coronary artery from pulmonary artery in 1 case, small caliber of proximal segment of the left main coronary artery, intramural left coronary artery or ostial stenosis of the left main coronary artery in another case, mitral valve prolapse with severe regurgitation without coronary arterial abnormality in the rest 4 cases. MDCT correctly diagnosed LMCAA in 5 cases, considered ostium atresia or severe stenosis of the left coronary artery in 1 case. All the 6 patients were definitely diagnosed as LMCAA using cardiovascular angiography in all 6 patients. Conclusion LMCAA is extremely rare. Echocardiography could reveal this disease, while MDCT may be an useful method. Angiocardiography remains the gold standard for preoperative diagnosis of LMCAA.