临床小儿外科杂志  2020, Vol. 19 Issue (12): 1123-1129  DOI: 10.3969/j.issn.1671-6353.2020.12.012

引用本文  

王勋, 肖政辉, 陈智, 等. 儿童心室预激性扩张型心肌病临床随访研究[J]. 临床小儿外科杂志, 2020, 19(12): 1123-1129.
Wang X, Xiao ZH, Chen Z, et al. Follow-up study of ventricular preexcitation dilated cardiomyopathy in children[J]. Journal of Clinical Pediatric Surgery, 2020, 19(12): 1123-1129.

基金项目

国家自然科学基金(编号:81500041);湖南省重点实验室平台项目(编号:2018TP1028);湖南省临床医疗技术创新引导项目(编号:2018SK50413)

通信作者

肖云彬, Email:xiaoyunbinrui@126.com

文章历史

收稿日期:2020-01-22
儿童心室预激性扩张型心肌病临床随访研究
王勋1 , 肖政辉2 , 陈智1 , 王野峰1 , 杨舟1 , 肖云彬1     
1. 南华大学儿科学院, 湖南省儿童医院心血管内科(湖南省长沙市, 410007);
2. 南华大学儿科学院, 湖南省儿童医院急救中心(湖南省长沙市, 410007)
摘要目的 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。
关键词心肌病, 扩张型    预激综合征    导管消融术, 射频    随访研究    儿童    
Follow-up study of ventricular preexcitation dilated cardiomyopathy in children
Wang Xun1 , Xiao Zhenghui2 , Chen Zhi1 , Wang Yefeng1 , Yang Zhou1 , Xiao Yunbin1     
1. Department of Cardiology, Academy of Pediatrics, University of South China, Hunan Children's Hospital, Changsha 410007, China;
2. Intensive Care Unit, Academy of Pediatrics, University of South China, Hunan Children's Hospital, Changsha 410007, China
Abstract: 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.
Key words: Cardiomyopathy, Dilated    Pre-Excitation Syndromes    Catheter Ablation    Follow-up Studies    Child    

心室预激性扩张型心肌病(dilated cardiomyopathy,DCM)是指由心室预激造成左心室心肌电-机械兴奋异常而引发的心室重构、心功能降低,临床心脏改变与扩张型心肌病类似[1]。研究显示抗心律失常药物治疗和射频消融治疗阻断心室预激可逆转心室预激性DCM的心脏扩大及心功能降低[2, 3]。然而目前有关心室预激性DCM报道较少,其临床表现、治疗方法及预后尚不明确。本研究拟总结湖南省儿童医院诊断的心室预激性DCM的临床表现、治疗方法和随访结果,为该类疾病临床诊治提供参考。

材料与方法 一、临床资料

回顾性分析2016年9月至2019年9月在湖南省儿童医院心血管内科住院,诊断为心室预激性DCM患者的临床资料(表 1)。研究对象纳入标准:①存在扩张型心肌病的依据;②无症状的显性WPW(Wolff-Parkinson-White syndrome)综合征(即临床没有反复或持续发作的快速性室上性心律失常发作史),右侧旁道;③M型超声、组织多普勒或二维超声应变分析发现心室收缩不同步现象,伴左心室扩大及左室射血分数(ejection fractions, EF)下降;排除其他原因导致的扩张型心肌病,尤其是心动过速性心肌病[4]

表 1 心室预激性DCM患者临床资料 Table 1 Clinical data and characteristics of children with ventricular preexcitation dilated cardiomyopathy
二、超声检查及分析

经胸超声采用Philips EPIQ7C彩色超声显像仪。消融术前及消融术后24 h、1个月、3个月、6个月、12个月行超声检查,胸骨旁长轴M型超声测量左心室收缩末期内径及舒张末期内径;左心室射血分数通过双平面Simpson法测得,二维斑点追踪成像技术检测左心室室壁运动协调性。

三、电生理检查及射频消融术

于静脉复合麻醉下行完整的电生理检查及射频消融术。穿刺右颈内静脉及股静脉,经颈内静脉成功放置冠状静脉窦电极,经股静脉鞘放置右心室电极,行心内电生理检查,行心房、心室程序刺激诱发心动过速,体表心电图可见预激波。4例采用8F极间距3.5 mm的强生导航星消融导管,病例4采用7F强生导航星消融导管,在右房及三尖瓣电解剖建模,仔细标测心内电图。为确保消融电极的稳定性及良好贴靠,病例2采用Swartz鞘支撑消融导管到达相应靶点进行消融。消融功率30 W,温度55 ℃,消融终点为旁路的前传及逆传均消失;消融成功后观察20 min,重复检查以减少复发可能。术后24 h、1个月、3个月、6个月、12个月随访复查心电图、超声心动图。

结果

5例患者中女性2例,男性3例,年龄6个月至6岁,病史7 d至5年。5例体表心电图均提示B型心室预激,其中病例1、病例3 V1导联QRS波群呈rS型,病例2 V1导联QRS波群呈QS型(图 1),病例2和病例3有阵发性室上性心动过速发作史。根据心动过速发作频率及动态心电图结果排除心动过速性心肌病。病例3自2014年诊断DCM并口服地高辛、呋塞米、螺内酯、阿司匹林治疗;病例4 6个月诊断为DCM,予地高辛、利尿剂、卡托普利治疗;病例1、病例2和病例5术前未予药物治疗。


图 1 心室预激性DCM患者术前12导体表心电图 Fig.1 The 12-lead electrocardiograms of children with ventricular preexcitation dilated cardiomyopathy   窦性心律时显性预激,QRS部位可见δ波。病例1、病例3 V1导联QRS波群呈rS型,提示旁路可能位于右侧间隔;病例2 V1导联QRS波群呈QS型,提示旁路可能位于右侧游离壁

术前超声提示5例患者左心室舒张末期内径增大(图 2)及左室射血分数(left ventricular ejection fraction,LVEF)降低(表 2);室间隔基底段变薄且呈矛盾运动(图 2);M超声提示室间隔与左心室后壁呈同向运动。病例3和病例4同时合并中至重度二尖瓣反流、左室心尖部肌小梁增多。二维斑点追踪检测显示左心室室壁运动不协调(图 2E)。


图 2 病例3消融术前后室间隔运动及左心室大小及左室长轴应变曲线 Fig.2 Movement of interventricular septum, size of left ventricle and longitudinal strain curves of case 3 before and after ablation A:消融术前左心室明显扩大,室间隔基底段变薄且呈瘤样矛盾运动;B:消融术后左心室腔明显缩小,瘤样矛盾运动消失;C:消融术前室间隔与左室后壁呈同相运动;D:消融术后室间隔与左心室后壁恢复逆向运动; E:显示消融术前下壁、后壁及中间段与左心室其他节段呈矛盾运动; F:显示消融术后应变曲线正常,各节段运动同步

表 2 心室预激性DCM患者消融术前后超声参数 Table 2 Echocardiographic data of children before and after ablation

4例行电生理检查和射频消融术治疗,1例在外院行电生理检查和射频消融术治疗,术后继续随访治疗。电生理检查过程中5例均能诱发逆传型房室折返性心动过速,旁路前传不应期分别为310 ms、320 ms、290 ms、290 ms、300 ms。病例1、病例2、病例3、病例5均未见多发旁路,术中消融成功(图 3),消融靶点分别见表 1,病例4消融后心电图表现为间歇性心室预激,提示可能存在多发旁路(图 4)。3例射频消融术后1个月随诊超声心动图显示左心室射血分数恢复正常,室间隔节段性运动恢复正常,室间隔与左心室后壁呈逆向运动(图 2),二维斑点追踪检测显示室壁运动协调性恢复(图 2F),病例3术后1个月左心室射血分数为53%,继续口服地高辛、呋塞米、螺内酯、美托洛尔、卡托普利至术后3个月,复查超声心动图显示LVEF、LVEDD恢复正常;病例4术后6个月复查心电图未显示心室预激波(图 4),左心室运动协调明显改善,继续予地高辛、利尿剂、美托洛尔和卡托普利等药物治疗至术后1年,复查超声心动图显示LVEDD和LVEF恢复正常(表 2)。


图 3 病例1消融靶点和希氏束位点 Fig.3 Site of ablation and His-bundle electrogram of case 1   蓝色点为三尖瓣游离壁标测到理想靶点,红色为消融位点,白色为希氏束位点


图 4 5例心室预激性DCM患者术后12导体表心电图 Fig.4 The 12-lead electrocardiograms of 5 children with ventricular preexcitation dilated cardiomyopathy   术后可见δ波消失,V1导联恢复正常的QRS波群
讨论

心室预激性DCM是因心室预激引发左心室电-机械异常所致,表现为室壁运动异常、心脏扩大、心功能下降。文献报道此类患者心电图均为B型心室预激,电生理标测显示旁道主要位于右侧游离壁和右侧间隔[5, 6]。本研究5例体表心电图均为B型心室预激,电生理检查显示2例旁道位于右侧游离壁,3例位于右侧前间隔,提示右侧显性旁道与心室预激性DCM有密切关系。有研究显示预激综合征患者存在持续性或反复房室折返性心动过速时可出现心脏扩大、心功能下降,即心动过速性心肌病表现[5]。本研究5例均不符合心动过速性心肌病表现,表明心室预激性扩张型心肌病发病与旁道所致房室折返性心动过速发作无明显关联。

研究报道心室预激性DCM患者心脏彩超均提示室壁运动异常,主要表现为室间隔基底段变薄、瘤样矛盾运动,部分患者可表现为室间隔基底段、中段与左室其它节段收缩不同步[7]。本研究5例均存在室壁运动异常,主要表现为室间隔基底瘤样矛盾运动,二维斑点追踪检测显示室间隔基底段与左室其它节段收缩不同步,伴左心室扩大、射血分数不同程度下降,经射频消融术治疗后3例24 h后室壁运动恢复正常,射血分数恢复正常或者接近正常;1例术前心功能明显下降,术后24 h复查室壁运动未能恢复正常,随访至3个月时室壁运动协调性明显好转,伴随射血分数恢复正常;1例术后仍可见心室预激波,术后1年室壁运动协调性恢复伴随左心室舒张末期内径、射血分数恢复正常。这提示右侧旁道预激相关的室壁运动异常可能是导致心脏扩大、心功能下降的主要原因,其可能机制为右侧旁道尤其是右侧间隔旁道提前激动了较大范围室间隔,引发室间隔局部变薄、运动不协调,左心室不能达到最大充盈,心肌收缩力和做功下降,冠状动脉供血改变和心肌灌注不足,最终导致左心室扩大和射血分数下降,呈现DCM样改变[8-10]

心室预激性DCM出现心力衰竭前多无临床症状[11, 12]。本研究中3例因其他原因就诊行超声心动图检查时发现室壁运动异常、左室射血分数减低,体表心电图检查显示B型心室预激;1例出现心力衰竭临床表现,初始诊断为DCM合并B型心室预激,予抗心力衰竭药物治疗病情无改善,1例因肺炎在外院检查时胸片显示心影增大,超声心动图提示DCM样改变,初始诊断DCM、心力衰竭、B型心室预激,抗心力衰竭药物治疗效果欠佳。上述结果提示心室预激性DCM起病隐匿,临床医师认识不足,早期容易误诊和漏诊。研究报道心室预激性DCM经射频消融术治疗后大部分患者心脏大小、心功能可恢复正常[13-15]。本研究中5例行射频消融术后随访结果显示室壁运动均恢复正常,左心室舒张末期内径、射血分数恢复正常,提示心室预激性DCM为完全可逆性改变,较DCM的预后好,对于临床诊断DCM合并B型心室预激的患者需仔细鉴别是否为心室预激性DCM,明确诊断后应及时行射频消融术治疗以改善预后;右侧旁道占预激综合征的27%[16, 17],心室预激性DCM仅被报道百余例[18-20],结合现有报道及本研究结果,推测心室预激性DCM的发病率可能被低估,B型心室预激患者预激性DCM的发生率、发生的高危因素尚待进一步研究,因此仅表现为B型心室预激的患者虽无心动过速发作,亦应推荐长期随访,以利于早期发现心室预激性DCM。

综上所述,本研究提示右侧前间隔及游离壁显性旁路可能导致左心室收缩不同步,从而引起左心室扩大及左心室收缩功能下降,严重者可致心室预激性DCM;预激性DCM为射频消融术的适应证,术后心功能可明显改善,预后良好;术前射血分数下降明显,患者术后需继续规范抗心力衰竭药物治疗并密切随访。

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