先天性右冠状动脉缺如一例并文献复习

摘要

目的 探索先天性右冠状动脉缺如的变异现象及其临床意义.方法 分析我科诊治的1例和国内外文献报道的51例先天性右冠状动脉缺如患者的临床资料,并结合相关文献进行讨论.结果 冠状动脉造影术诊断44例,冠状动脉CT血管成像诊断6例,外科探查诊断2例.25例患者表现为心肌缺血症状,合并左冠状动脉明显病变时可出现严重心脏症状,约1/3患者可出现心律失常,其中病态窦房结综合征3例,心房颤动6例,Ⅲ度房室传导阻滞1例.治疗上,10例行冠状动脉血运重建术,2例行心脏起搏器植入术,12例予药物治疗.结论 单纯先天性右冠状动脉缺如多表现为良性临床过程,但在心律失常方面有潜在的危险性,故需保持随访与治疗,一旦合并左冠状动脉病变应早期积极治疗.MDCT可作为检测冠状动脉变异的筛查手段.%Objective To explore the diagnosis and clinical features of congenital absence of right coronary artery. Methods Clinical data of 52 cases (1 case in our department and 51 cases reported in literatures) with congenital absence of right coronary artery were analyzed. Results Of the 52 cases, 44 diagnosed by coronary angiography, 6 by coronary CTA and 2 by surgical exploration. Most of the patients presented with symptoms of myocardial ischemia. Patients with severe left coronary artery disease often had severe cardiac symptoms. About 1/3 of the patients had arrhythmias, including 3 cases with sick sinus syndrome, 6 cases with atrial fibrillation and 1 case with complete atrioventricular block. 10 patients underwent coronary revascularization, 2 cases accepted pacemaker implantation and 12 patients with medical therapy. Conclusions Although patients with congenital absence of right coronary artery generally have benign clinical course, they need regular follow up due to the potential risk of arrhythmias. MDCT can be used as a screening method for detecting coronary artery variation.