婴儿期起病的大动脉炎合并心脏病变临床特点分析

Clinical characteristics of infantile Takayasu arteritis complicated with cardiac involvements

目的分析婴儿期起病的大动脉炎(TAK)合并心脏病变的临床特征。方法回顾性收集婴儿期TAK的临床资料及心脏病变情况,分析总结该病的临床特点。主要采用描述性统计方法。结果共20例患儿,其中16例(80%)发现心脏病变,仅2例出现症状。病变较常见为冠状动脉受累(CAL)、心脏瓣膜病、心肌酶升高,少见为心律失常、心包积液、高血压性心脏病、心力衰竭。1例出现急性心力衰竭,为收缩性心力衰竭,同时合并高血压心脏病。14例合并CAL均为常规冠状动脉超声筛查发现,共发现39处冠状动脉病变,均为扩张,且左冠状动脉主干均受累。5例出现心脏瓣膜病变,均为关闭不全,受累瓣膜为二尖瓣、三尖瓣,其中1例为重度关闭不全。2例心律失常,其中1例病程3月时发现,表现为短阵性房性心动过速,部分伴高度房室传导阻滞。20例患儿经生物制剂和(或)糖皮质激素治疗后均存活且病情稳定。急性心力衰竭合并高血压心脏病的1例同时予抗心力衰竭和降压治疗,随诊4年心功能及血压恢复正常。14例合并CAL病变的患儿均加用阿司匹林口服,随访时10例CAL消失,4例冠状动脉扩张较前回缩,无心肌梗死发生。5例心脏瓣膜患儿随访时4例关闭不全消失,1例关闭不全好转,随访时无接受瓣膜置换术患儿。心律失常的患儿中1例予抗心律失常药治疗,治疗后心律失常消失,随访中无复发。结论婴儿期起病的TAK心脏病变发生率高,临床症状隐匿,病变广泛,以冠脉扩张常见,罕见心力衰竭,应尽早评估和治疗。

Objective To analyze the clinical characteristics of infantile Takayasu Arteritis(TAK)complicated with cardiac involvements.Methods The clinical data and cardiac lesions of infantile TAK were collected retrospectively,and the clinical characteristics of the disease were analyzed and summarized.Mainly using decriptive statistical methods.Results In these 20 cases,16 cases(80%)had cardiac involvements,only 2 cases had related symptoms.The common lesions were coronary artery lesion(CAL),valvular disease,and elevated myocardial enzymes,while the rare lesions were arrhythmia,pericardial effusion,hypertensive heart disease,and heart failure.One case had acute heart failure,which was systolic heart failure and was accompanied by hypertensive heart disease.All 14 patients with CAL were found by conventional coronary ultrasound screening.A total of 39 CAL were found,all of which were coronary artery dilation,and the left main coronary artery was involved.Five patients had heart valve disease,all of them were valve insufficiency.The involved valves were mitral and tricuspid valves,and one of them was severe insufficiency.Arrhythmias were found in 2 cases,of which P1 was found to have paroxysmal atrial tachycardia with high atrioventricular block at 3 months.All 20 children survived and were in stable condition after being treat with biological agents and/or glucocorticoids.A case of hypertensive heart disease complicated with heart failure was followed up for 4 years,and the cardiac function and blood pressure returned to normal.Fourteen children with CAL lesions were given oral aspirin disease,the CALs disappeared in 10 cases and retracted in 4 cases.During the follow-up of 5 children with heart valves,insufficiency disappeared in 4 cases and improved in 1.No child underwent valve replacement during the follow-up.One of the children with arrhythmia was treated with antiarrhythmic drugs.After treatment,the arrhythmia disappeared.Now they have been followed up for 5 years without recurrence.Conclusion Infantile TAK has a high incidence of heart involvement,with extensive lesions but insidious clinical symptoms.CALs are common,and heart failure is rare.It should be evaluated and treated as early as possible.