儿童心室预激性扩张型心肌病临床随访研究

Follow-up study of ventricular preexcitation dilated cardiomyopathy in children

目的探讨儿童心室预激性扩张型心肌病的临床特征、诊疗方法和预后。方法回顾性分析湖南省儿童医院心血管内科2016年9月至2019年6月诊断的预激性心肌病患者的临床表现、心电图及超声表现、治疗经过及射频消融术后转归。结果5例患者,女性2例,男性3例,年龄6个月至6岁,术后随访1~12个月;5例体表心电图均提示为B型心室预激,超声心动图提示左室扩大、左心收缩功能减低和室壁运动不协调,斑点追踪技术发现左心室收缩不同步;3例消融靶点位于右侧前间隔,2例位于右侧前游离壁,射频消融后,4例心电图预激波消失,超声心动图提示左心室射血分数提高,4例术后24 h左心室运动协调性明显改善,左心室舒张末期内径回缩至正常或接近正常;1例术后呈现间歇性预激波,术后6个月复查心电图未显示心室预激波,左心室运动协调性明显改善,术后1年左心室舒张末内径及射血分数恢复正常。结论右侧前间隔或游离壁显性旁路可导致扩张型心肌病样改变;左心室收缩不同步可能是其重要发病机制;心室预激性扩张型心肌病为射频消融良好指征,术后预后良好。

Objective To explore the clinical manifestations,treatments and prognosis of ventricular preexcitation dilated cardiomyopathy(DCM)in children.Methods Clinical manifestations,electrophysiological findings and echocardiographic results were retrospectively reviewed for 5 children of ventricular preexcitation DCM at Hunan Children's Hospital from September 2016 to June 2019.Results There were 3 boys and 2 girls with an age of initial examination of(0.5-6)years.The follow-up period was(1-12)months after catheter ablation.Electrocardiogram revealed type B ventricular preexcitation while ultrasound hinted at left ventricle(LV)dilation,LV dysfunction and incoordination movement between posterior wall of left ventricle and basal segments of ventricular septum.Asynchronization of left ventricular wall motion was detected by speckle tracing.The locations of accessory pathway were right-sided anteroseptum and free wall.Electrocardiogram indicated the presence of sinus rhythm and delta wave disappeared completely in 4 children.Echocardiograph demonstrated synchronous contraction.Left ventricular end diastolic diameter(LVEDD)gradually decreased to almost normal during follow-ups.In one child,delta wave of QRS disappeared completely and contraction regained synchrony during 6-month follow-up while LVEDD and LVEF normalized during 1-year follow-up.Conclusion Overt right-sided accessory pathways may cause DCM in anteroseptum and free wall.Asynchronous ventricular contraction is a vital mechanism of ventricular preexcitation DCM.Radiofrequency ablation therapy is indicated for ventricular preexcitation DCM with an excellent prognosis.