A follow-up analysis of cardiac function and complications in pediatric patients with permanent epicardial pacing

Background Nowadays, the number of pediatric patients receiving permanent pacemaker implantations is increasing. However, there is very little relevant, long-term follow-up analysis carried out in China. Methods Clinical information from pediatric patients who underwent epicardial permanent pacemaker implantation from January 1, 2005 to December 31, 2017 in Guangdong Provincial People's Hospital was collected. Eligible patients received 12-lead electrocardiograms and echocardiography preoperatively and postoperatively, as well as programming control during their follow-up. Meanwhile, complications were treated and analyzed. Results A total of98 pediatric patients with permanent epicardial pacing were included in the study, including 60 males and 38 females. The age of patients who underwent surgery for the first time ranged from 8 days to 14 years old( the median age of these patients was 3 years old). The weight of these patients ranged from 2.7 kg to 52 kg(the median weight of these patients was 12 kg). Three patients died while they were getting treatment. The follow-up was carried out on eighty-seven out of ninety-five patients(91.6%) and lasted 5 months to 13 years( average 3.79±3.06 years). One patient with single-chamber right ventricular pacing was found to have cardiac insufficiency in the second year after operation. There were 6 patients with cardiac insufficiency whose ejection fractions were less than 55%. All these patients had a single-chamber left ventricular pacing implant or double-chamber right atrial left ventricular pacing implant. Paired T-test was used to compare the preoperative LVEF with the last echocardiographic LVEF(45%±7% vs.67%±7%, P 0.05),which indicated statistical differences. Conclusion A permanent epicardial pacemaker is safe and effective and reasonable pacing mode can protect cardiac function.[S Chin J Cardiol 2019;20(1):10-14]     

儿童常见先天性心脏病介入治疗的并发症分析

目的总结儿童先天性心脏病(CHD)介入治疗并发症的发生率,并分析其发生原因。方法回顾性分析2011年1月至2013年12月间2 356例诊断为室间隔缺损(VSD)、房间隔缺损(ASD)、动脉导管未闭(PDA)、肺动脉瓣狭窄(PS),并行介入治疗的0~18岁CHD患儿,在介入治疗中及治疗后发生并发症的情况。结果 2 356例CHD患儿中159例发生并发症,发生率为6.75%;其中VSD组为11.40%(82/719),ASD组为7.50%(51/680),PDA组为3.09%(22/712),PS组为1.63%(4/245)。心律失常发生率为4.41%(102/2 356)。严重并发症发生率为2.71%(64/2 356),VSD组为3.62%(26/719),ASD组为2.21%(15/680),PDA组为2.53%(18/712),PS组为1.63%(4/245);术中严重并发症为0.51%(12/2 356),术后早发严重并发症为1.99%(47/2 356),迟发严重并发症为0.21%(5/2 356)。严重并发症经介入手术治疗0.13%(3/2 356),经外科手术治疗0.64%(15/2 356),经内科保守治疗1.95%(46/2 356);2例死亡,死亡率0.08%。结论 CHD介入治疗的并发症及死亡率低,但仍不容忽视。术前严格掌握适应证,术中按常规操作,术后进行规范的长期随访至关重要。     

内脏异位综合征合并肺动脉狭窄/闭锁患儿缝隙连接蛋白43基因突变的检测

目的:探讨内脏异位综合征合并肺动脉狭窄/闭锁患儿有无缝隙连接蛋白43(Connexin43,Cx43)基因突变。方法:选取47例内脏异位综合征合并肺动脉狭窄/闭锁患儿,45例无内脏异位的法洛氏四联症患儿以及30例无先天性心脏病的患儿,抽取外周血,采用PCR-SSCP和DNA测序技术检测有无Cx43胞质尾区基因突变。结果:所有样本DNA的PCR特异性扩增良好。对Cx43胞质尾区的PCR扩增产物进行SSCP分析和测序,未查找到单核苷酸多态性位点任何转换、颠换、缺失和插入的变异。结论:内脏异位综合征合并肺动脉狭窄/闭锁的患儿以及无内脏异位的法洛氏四联症患儿均无Cx43基因突变。     

门纯德先生治疗良性肿瘤的经验

<正>门纯德(1917-1984),字秉洁,祖籍河北蔚县,后定居山西大同,在山西中医界久负盛名,素有"北门"之誉。大型传记丛书《中华中医昆仑》精选了为中华现代百年贡献卓著、深受敬仰的150位中医药学家,门纯德先生赫然入列。门纯德先生把自己的一生都奉献给了中医事业,影响了整个山西中医学界,是"山西门氏杂病流派"的创始人。先生尊仲景,崇尚《伤寒杂病论》,其临证"智圆法活"、"方精药简",注重阴阳,长于温运。治学思     

胎儿严重肺动脉瓣狭窄或闭锁的产前超声心动图诊断评估、右心室发育评估及宫内介入治疗的结局随访

目的探讨胎儿室间隔完整型严重肺动脉狭窄(critical pulmonary stenosis with intact ventricular septum,CPS/IVS)或室间隔完整型肺动脉闭锁(pulmonary atresia with intact ventricular septum,PA/IVS)的产前超声心动图诊断、右心室发育评估及宫内介入治疗的结局随访。方法回顾性分析广东省人民医院2016年9月至2018年12月6例产前超声心动图诊断为PA/IVS或CPS/IVS(1例诊断PA/IVS,5例为CPS/IVS)行胎儿肺动脉瓣球囊成形术(fetal pulmonary valvuloplasty,FPV)胎儿的产前超声心动图诊断、右心室发育评估及结局随访资料。结果 6例胎儿诊断孕周为(26.48±2.15)周。6例胎儿术前三尖瓣环/二尖瓣环比值(tricuspid valve annulus/mitral valve annulus,TV/MV)分别为0.53、0.82、0.71、0.85、0.77、0.71,右心室纵径/左心室纵径比值(right ventricle length/left ventricle length,RV/LV)分别为0.42、0.63、0.52、0.61、0.75、0.61,三尖瓣流入时间/心动周期长度比值(tricuspid valve inflow duration/cardiac cycle length,TVID/CCL)比值分别为0.26、0.35、0.39、0.44、0.44、0.35,肺动脉瓣环/主动脉瓣环(pulmonary valve annulus/aortic annulus,PV/AV)比值分别为0.85、1.03、0.85、0.86、1.20、0.78。动态观察2周后,6例胎儿右心室各指标未见明显增长,于(29.45±1.19)周行FPV术,均取得技术性成功(100%),无宫内死亡,孕妇及胎儿无严重并发症。FPV术后,6例胎儿术后1～2周内TV/MV、RV/LV、TVID/CCL明显增长,术后2～6周增长趋于稳定,而PV/AV则在术后2～4周增长较明显。6例患儿分娩胎龄为(37.74±1.17)周,出生体质量(3.04±0.24)kg,于生后(15.33±7.31)d完成行一期手术,其中2例行外科手术,4例12的严重感染所致的多器官功能衰竭。存活的5例患儿血氧饱和度95%,无右心衰竭症状。结论产前可通过超声评估CPS/IVS、PA/IVS胎儿右心室发育状况,筛选宫内介入治疗适应证,适时进行FPV,可促进右心室小梁部及三尖瓣发育,争取患儿生后实现双心室循环。     

Cardiac catheterization and comprehensive clinical evaluation after bidirectional Glenn shunt surgery in 60 patients with complex congenital heart disease

Background The bidirectional Glenn shunt surgery is a palliative procedure for patients with complex congenital heart disease(CHD) who are not suitable for biventricular repair in early life. There is limited evidence of successful strategies for long-term hemodynamic stabilization. Furthermore, there have been no data on optimal hemodynamics that could be used as a reference for patients' follow-on management. Methods Sixty CHD patients, 44 male and 16 female, with bidirectional Glenn shunt surgery and cardiac catheterization were enrolled at our hospital between January 2014 and December 2016. Pre-and post Glenn shunt percutaneous oxygen saturation(SpO_2), 6-minute walk test(6 MWT), superior vena cava pressure(SVCP), pulmonary arterial pressure(PAP), pulmonary capillary wedge pressure(PCWP), pulmonary vascular resistance(PVR), small pulmonary vascular resistance(s PVR) were measured. Pre-and post-total cavopulmonary connection(TCPC) SpO_2, and in-hospital complications were monitored. The optimal hemodynamic cutoff values for TCPC patient selection were estimated by receive operating characteristic(ROC) curve analysis. Results SpO_2 was significantly increased by bidirectional Glenn shunt surgery(75.42 ± 9.62% to 86.98 ± 7.63%, P 0.001) from 82.70 ± 5.99% to 95.00 ±4.07% in the 47 patients with TCPC. Forty-two patients completed the 6 MWT with a mean distance of 362.7 ±75.0 m and a SpO_2 decrease from 81.80 ± 7.84% to 67.59 ± 1.82%(P 0.001). The △SpO_2 and 6-minute walk distance(6 MWD) in the 32 who underwent TCPC and ten of them did not reach statistical significance(17.22 ±13.82% vs. 13.87 ± 8.74%, P = 0.08 and 358.88 ± 78.97 m vs. 374.80 ± 62.55 m, P = 0.564]. After cardiac catheterization, 47 patients were selected for TCPC. The right pulmonary artery systolic pressure(s RPAP), mean right pulmonary artery pressure(m RPAP), mean left pulmonary artery pressure(m LPAP), PVR, and s PVR were significantly lower in the TCPC group than in the non-TCPC group. The differences in superior vena cava systolic blood pressure(s SVCP), mean superior vena cava pressure(m SVCP), and left pulmonary artery systolic pressure(s LPAP) were not significant. The optimal cutoff values for TCPC were s SVCP ≤ 20 mm Hg(P = 0.025),s RPAP ≤ 22 mm Hg(P = 0.0001, mRPAP ≤ 13 mm Hg(P =0.003), s LPAP ≤ 27 mm Hg(P =0.03), m LPAP ≤ 11 mm Hg(P = 0.01), PVR ≤ 4.3 Wood U/m~2(P 0.0001) and were significantly associated with TCPC selection,except for m SVCP ≤ 19 mm Hg(P = 0.06) and s PVR ≤ 2.0 wood U/m~2(P = 0.0531). One patient died because of low cardiac output after TCPC. In-hospital mortality was 2.1%. Conclusion The SpO_2 can be significantly improved after bidirectional Glenn shunt and TCPC surgery. The 6 MWT is an index of activity tolerance prior toTCPC. Hemodynamic values of s SVCP ≤ 20 mm Hg, s RPAP ≤ 22 mm Hg, m RPAP ≤ 13 mm Hg, s LPAP ≤ 27 mm Hg, m LPAP ≤ 11 mm Hg, and PVR ≤ 4.3 Wood U/m~2 can help identify post Glenn-shunt patients indicated for TCPC.     

儿童室间隔缺损封堵术后心律失常中远期随访结果分析

目的观察及分析儿童室间隔缺损(VSD)封堵术后心律失常的中远期改变情况。方法回顾性分析2002年3月至2010年12月行VSD介入封堵术的1 071例患儿在术后第l、3、6、12个月及其后每年的门诊随访资料。结果1 071例患儿中,早期(术后1个月内)出现心律失常者272例(25.4%),主要包括房室传导阻滞(AVB)、束支传导阻滞、交界性心动过速、房性及室性心动过速、频发期前收缩等,其中Ⅱ度以上AVB及完全性左束支传导阻滞(CLBBB)等严重心律失常22例(2.1%),经治疗后均好转,无患儿需安装永久起搏器。在以后的随访中,平均随访时间(2.8±1.7)年(1~107个月),持续存在心律失常者161例(18.2%),主要为AVB和束支传导阻滞,其中严重心律失常10例(1.1%);4例迟发型AVB中3例在术后早期就已出现过AVB,1例患儿随访过程中复发CLBBB并出现左心室增大,最终因心功能衰竭死亡;4例患儿随访过程中植入永久起搏器。结论 VSD封堵术后早期出现AVB或CLBBB等严重心律失常的患儿在随访过程中复发可能性大;传导阻滞类心律失常随访过程中可出现复发或加重;心动过速、期前收缩等起源异常的心律失常随访过程中大多可恢复正常。     

介入封堵术与外科手术治疗膜周部室间隔缺损效果的观察

目的　探讨介入封堵术对膜周部室间隔缺损的即时治疗效果,并与同期外科手术效果进行对比。方法　采用封堵术治疗膜周部室间隔缺损患儿4 8例,与同一时期采用外科手术治疗的73例患儿,进行年龄、身高、体重、肺动脉压力、室间隔缺损大小、手术及住院时间、手术成功率及并发症的比较。两组间均数的比较采用t检验,率的比较采用卡方检验。结果　两组小儿的年龄、身高、体重、肺动脉压力及手术成功率差异无统计学意义。封堵术组的室间隔缺损较手术组的小,但手术及住院时间较短。封堵组术中及术后出现左前分支或完全性左束支阻滞较多见,其他并发症包括再次外科手术、残余分流、术中出血多、神经受损、溶血、术中或术后Ⅲ度房室传导阻滞、气胸、胸腔积液、心包积液、主动脉关闭不全及术后感染在两组间差异无统计学意义。结论　室间隔缺损封堵术作为治疗室间隔缺损的新方法,即时的疗效确切。采用封堵术治疗室间隔缺损应遵循治疗原则,术中遵守操作规范,术后严密观察,从而减少并发症的发生。     

儿童白塞病合并急性心肌梗死一例

白塞病是一种反复发作,累及多系统的全身疾病,由土耳其医生Behcet于1937年首次报道。除口腔、生殖器和眼三联征外,尚有各种不同的全身或内脏表现,包括皮肤损害、关节症状、心血管病变等,现将我院收治儿童白塞病合并急性心肌梗死1例报告如下。     

经导管纽扣式堵塞装置关闭小儿室间隔缺损临床探讨

目的为探讨经导管纽扣式堵塞装置关闭小儿室间隔缺损的疗效。方法我科从1995年1月～1997年9月采用Sideris纽扣式补片关闭小儿室间隔缺损9例。结果堵闭成功8例，失败1例，经1月～2年随访，4例恢复良好，2例有轻或中度主动脉瓣关闭不全，1例残余分流2．smm，1例中度三尖瓣关闭不全。结论本方法关闭小儿室间隔缺损，从指征，堵塞装置及操作技术上尚待进一步研究。