Specific Injuries Management in the Postoperative of Congenital Heart Diseases(Ⅱ):Univentricular Hearts

It is very important to understand that the univentricular heart surgery is just palliative, not being in anyway a definitive or curative surgery, but nowadays it’s the best initial treatment of this complex heart disease. The fundamental philosophy of treatment of every univentricular heart is to ensure the flow system and/or restrict the lung flow. Thus, initially a patient with univentricular heart who is undergoing surgery may need to ensure systemic flow (reconstruction of the aortic?arch type Norwood), to restrict the lung flow (pulmonary banding) or to provide enough?pulmonary flow (pulmonary-systemic fistulae). However, some heart diseases with univentricular physiology remain “balanced” autonomously, until the “second” stage of palliation is performed (cavo-pulmonary anastomosis type Glenn), but others require performance of pulmonary banding, if there’s no native lung protection and/or repair of the systemic circuit in a first stage, to reach next palliation steps in the best possible conditions.

MRI-guided ablation of wide complex tachycardia in a univentricular heart

Magnetic resonance imaging can be used for preprocedural assessment of complex anatomy for radiofrequency(RF) ablations,e.g.,in a univentricular heart.This case report features the treatment of a young patient with a functionally univentricular heart who suffered from persistent sudden onset tachycardia with wide complexes that required RF ablation as treatment.

Total Cavopulmonary Connection:Lateral Tunnel Anastomosis or Extracardiac Conduit?——an Analysis of 114 Consecutive Patients

Objective To compare the postoperative outcomes of patients with the diagnostic univentricular heart undergoing lateral tunnel （LT） operation with extracardiac conduit （EC） operation. Methods- From June 1996 to July 2007, 114 consecutive patients with a single ventricle underwent total cavopulmonary connection （TCPC） in Children＇s Heart Center, lAniversity Hospital Giessen and Marburg GmbH, Germany. A LT was performed in 19 （16.7%） patients, and an EC in 95 （83.3%） patients. The mean age of EC group was 50.8±31.6 （ranging from 22 to 212） months, and that of LT group was 61.5±41.2 （ranging from 30 to 168） months. Early and midterm outcomes of two groups were analyzed.Results One died in LT group （5.3%） and three in EC group （3.2%）. The overall mortality was 3.5%. There was no significant difference in mortality between EC-and-LT groups （P〉0.05）. The postoperative pulmonary arterial pressure, oxygen saturation, and effusion time of two groups had no significant difference （all P〉0.05）. No significant difference in the occurrences of complications （arrhythmias, enteropathy, and thrombosis） was found between two groups after operation （P〉0.05）. Conclusions There seems no difference between LT and EC in the clinical results in the early and middle postoperative stage. Glenn anastomosis followed by an EC seems to have some advantages.

单纯左室起搏与双心室起搏治疗慢性心力衰竭的疗效比较

目的比较单纯左室起搏与双心室起搏治疗慢性心力衰竭（CHF）的可行性及临床效果。方法选择窦性心律、NYHA心功能分级Ⅲ～Ⅳ级、左室射血分数（LVEF）≤0．35、QRS波时限≥120ms的CHF患者36例，成功植入心脏再同步化（CRT）起搏器后，随机分为两组，第一组先以右房左室起搏模式治疗（LV起搏模式）7天，然后以右房双室起搏模式治疗（Biv起搏模式）7天；第二组先以Biv治疗7天，然后以LV治疗7天。收集术前、术后第7天、第14天的临床资料，所有数据应用交叉设计资料方差分析及单变量一般线性模型分析。结果6min步行距离、明尼苏达生活质量评分、体表心电图QRs波时限、左室舒张末期内径、左室射血分数、左室间隔部与侧壁基底段收缩期达峰时间差值等6种评价指标的结果相似：评价指标测量值在处理效应即LV、Biv两种起搏模式上、在顺序效应即两种起搏模式顺序上的差异均无统计学意义（P均〉0．05）；但在阶段效应即术后第7天、第14天两阶段上的差异有统计学意义（P〈0．05）。结论单纯左室起搏与双心室起搏治疗CHF的疗效相仿；对窦性心律、完全性左束支传导阻滞的CHF患者，单纯左室起搏可作为CRT的一种选择。

Results of Fontan in Patients with Apicocaval Juxtaposition or/and Separated Hepatic Venous Drainage

Objective:Modifications of the Fontan operation,which are also known as total cavopulmonary connection(TCPC),are widely applied for patients with functionally univentricular hearts(FUH).Herein,we summed up the different surgical pathways and clinical outcomes in FUH patients with apicocaval juxtaposition(ACJ)or/and separated hepatic venous(SHV)drainage.Methods:Between January 2009 and December 2019,123 patients who undergone TCPC in our institute were included in this retrospective study.We have included 70 patients with ACJ(Group 1)and 53 patients with SHV(Group 2).Moreover,Group 2 included 17 cases combing with ACJ(32.1%).In Group 1,three different TCPC methods were conducted.While 45 cases were conducted with the extracardiac conduit-TCPC(EC-TCPC)method,24 cases used the intracardiac conduit-TCPC(IC-TCPC)method,and only one case used the lateral tunnel-TCPC(LT-TCPC).In Group 2,four TCPC methods were conducted on patients.Forty cases used the EC-TCPC-common open technique,6 cases with IC-TCPC technique,4 cases with LT-TCPC,and 3 cases with intra-extracardiac conduit-TCPC(IEC-TCPC).Results:There were 7 patients in Group 1 and 14 patients in Group 2 who required early re-operation during hospitalization(p<0.05).Postoperative mean pulmonary artery pressure(mPAP)greater than 15 mmHg emerged as a predictor for early re-operation(p<0.01)and early death(p<0.001)in univariate analysis.Conclusions:TCPC can be performed in these patients and shows beneficial results.Under the Fontan principle of connecting systemic venous to the pulmonary vasculature unimpededly,surgeons should carefully evaluate three components when choosing for the surgical technique:The distance between inferior vena cava(IVC)and the apex;the site of the vertebrae relative to the ACJ;the distance between ACJ and SHV if coexisting.However,the technique should be altered when the postoperative mPAP was greater than 15 mmHg.

Upper body peripherally inserted central catheter in pediatric single ventricle patients

BACKGROUND There is risk of stenosis and thrombosis of the superior vena cava after upper extremity central catheter replacement.This complication is more serious among patients with single ventricle physiology,as it might preclude them from undergoing further life-sustaining palliative surgery.AIM To describe complications associated with the use of upper extremity percutaneous intravenous central catheters(PICCs)in children with single ventricle physiology.METHODS A single institution retrospective review of univentricular patients who underwent superior cavopulmonary anastomoses as their stage 2 palliation procedure from January 2014 until December 2018 and had upper body PICCs placed at any point prior to this procedure.Clinical data including ultrasonography,cardiac catheterization,echocardiogram reports and patient notes were used to determine the presence of thrombus or stenosis of the upper extremity and cervical vessels.Data regarding the presence and duration of upper extremity PICCs and upper extremity central venous catheter(CVC),and use of anticoagulation were recorded.RESULTS Seventy-six patients underwent superior cavopulmonary anastomoses,of which 56(73%)had an upper extremity PICC at some point prior to this procedure.Median duration of PICC usage was 24 d(25%,75%:12,39).Seventeen patients(30%)with PICCs also had internal jugular or subclavian central venous catheters(CVCs)in place at some point prior to their superior cavopulmonary anastomoses,median duration 10 d(25%,75%:8,14).Thrombus was detected in association with 2 of the 56 PICCs(4%)and 3 of the 17 CVCs(18%).All five patients were placed on therapeutic dose of low molecular weight heparin at the time of thrombus detection and subsequent cardiac catheterization demonstrated resolution in three of the five patients.No patients developed clinically significant venous stenosis.CONCLUSION Use of upper extremity PICCs in patients with single ventricle physiology prior to super cavopulmonary anastomosis is associated with a low rate of catheterassociated thrombosis.

右位心合并单心室的外科治疗

目的探讨右位心合并单心室的外科治疗。方法回顾性分析我院收治的2例右位心合并单心室患儿的临床资料。结果例1,5岁,为右旋右位心,大动脉转位,单心房,单心室,二尖瓣闭锁,肺动脉瓣狭窄,行双侧双向Glenn术;例2,6岁,为镜面右位心,大动脉转位,单心房,单心室,肺动脉狭窄,疑合并Kartagener综合征,行全腔静脉肺动脉连接术。例1手术成功,于术后12天出院,术后4个月随访患儿无特殊不适。例2术后并发左侧上肺不张、乳糜胸,于术后49天出院,3个月后,出现大量乳糜胸水、紫绀、气喘、腹胀、双下肢水肿,再次入院,于入院后16天死亡。结论右位心合并单心室是一种少见的紫绀型复杂先天性心脏病,Fontan类手术是可供选择的术式,但合并Kartagener综合征则应慎重选择术式。

Veno-Venous Extra-Corporeal Membrane Oxygenation (ECMO) in a Child with Hemoptysis and Fontan Circulation

Background: Extracorporeal membrane oxygenation is a rescue life support technique used in life threatening conditions of refractory respiratory and/or cardiac distress. Indication for extracorporeal life support in children depends on age and varies from pulmonary to cardiac pathologies. In some cases, it may be used as a bridge to a therapeutic procedure. We described here the management of respiratory failure due to hemoptysis in a child with a Fontan circulation and veno-venous extracorporeal membrane oxygenation which served as a bridge to angio-embolization. Hemoptysis can be a life threatening condition which can lead to hypovolemic shock and impaired alveolar gas exchange. The latter can result in respiratory failure and consequent asphyxia. When hemoptysis occurs in a patient with a univentricular heart and a Fontan circulation, management of this clinical situation can be challenging due to the particular physiology of the latter. Total cavopulmonary connection is a palliative surgical repair which constitutes Fontan circulation as a definitive treatment in patients with a univentricular heart. Methods: Case report description of a 16 year-old boy with a univentricular heart and a Fontan circulation who presented hemoptysis managed with a veno-venous extracorporeal membrane oxygenation (ECMO) as a bridge to angio-embolization. Results: Hemoptysis due to diffuse intra-alveolar hemorrhage from collateral circulation was successfully treated in this young patient with pulmonary vascular embolization. This allowed to wean the patient from extra-corporeal membrane oxygenation. Conclusion: Veno-venous ECMO can be life-saving as a bridge to angio-embolization for severe hemoptysis in patients with Fontan circulation. The reported case allows to underline that our multidisciplinary approach in this complex pediatric patient surely participated to improve outcome.

Hemodynamic Changes and Clinical Outcomes after the Intra/Extracardiac Fenestrated Fontan Procedure

Background: The primary concern of the Fontan procedure has been atrial arrhythmias, but little attention has been given to hemodynamic changes. This study’s purpose was to examine the hemodynamics and arrhythmias of the Intra/extracardiac fenestrated Fontan, and determine any advantages/disadvantages of this newer Fontan procedure. Methods: Patients undergoing the intra/ extracardiac fenestrated Fontan procedure at this institute between December 2009 and June 2017 were included in this retrospective evaluation of preoperative and postoperative hemodynamics during cardiac catheterization. End Diastolic Pressure (EDP), Pulmonary Artery Pressure (PAP), Left Atrial Pressure (LAP), and Transpulmonary Gradient (TPG) were the hemodynamic markers of interest. Occurrence of arrhythmias was also examined preoperatively, less than 2 weeks postoperatively, and greater than 2 weeks postoperatively. Morbidities and mortalities were also evaluated. Results: Fourteen patients underwent the procedure between December 2009 and June 2017. Preoperative data was available in all of these patients. Postoperative data was partially incomplete for atrial arrhythmias and hemodynamic data. EDP rose from 9.29 ± 3.50 mmHg to 10.33 ± 3.12 mmHg. PAP rose from 12.57 ± 2.50 mmHg to 14.27 mmHg. TPG dropped from 5.00 ± 2.29 mmHg to 3.25 ± 1.67 mmHg. LAP rose from 7.57 ± 2.87 mmHg to 10.30 ± 1.95 mmHg. Atrioventricular valve (AVV) regurgitation remained the same pre and postoperatively. 8 of 13 patients developed arrhythmias at less than 2 weeks postoperatively, and 5 of 12 patients developed arrhythmias at greater than 2 weeks postoperatively. There were no morbidities or mortalities. Conclusions: We found a statistically significant change in EDP, PAP, LAP, and TPG levels. It is unclear whether this is an advantage or disadvantage. The significance of these changes is unclear in this small population of patients. Further evaluation of hemodynamics, arrhythmias, morbidities and mortalities associated with the intra/extracardiac Fontan procedure is needed.

全腔静脉-肺动脉连接术的回顾与展望

Fontan手术迄今仍是治疗单心室一类复杂先天性心脏病的主要手段。Fontan手术方法从1968年问世以来有许多改进,早期应用的心房-肺动脉连接术已为全腔静脉-肺动脉连接术所取代。通过对心房内隧道和心外管道全腔静脉-肺动脉连接术的中、晚期手术疗效进行比较分析,心外管道优点更多。然而,由于缺乏右心室的泵血功能,晚期循环衰竭最终都难以避免。当Fontan循环衰竭药物治疗无效时,惟一的选择是心脏移植,但后者供体来源缺乏。目前正研究开发的,旨在"双心室化"单心室Fontan循环的腔-肺机械辅助装置,有很好的前景。近年来胎儿心脏病诊疗技术的进展也拓宽了治疗复杂先天性心脏病的径路,经导管扩张严重主动脉瓣狭窄防止发展为左心室发育不全综合征,以增加双心室修复的机会;或对不能根治的复杂先天性心脏病患者终止妊娠,以降低此类复杂先天性心脏病的出生率等。我们结合文献对Fontan手术的方法进行回顾和展望。

Fontan extracardiac tunnel connection： fenestration or not？

Background The fenestration function is by allowing a right-to-left shunt resulting in an increased cardiac index, associated with mild arterial oxygen desaturation. Subsequent transcatheter fenestration closure can be performed after haemodynamic assessment. The purpose of this study was to compare the outcomes of extracardiac connection （EC） with or without fenestration. Methods Ninety-five consecutive patients diagnosed with univentricular heart disease underwent EC using Gore-Tax conduits at the Department of Children＇s Heart Center, Justus-Liebig-University Giessen Germany from June 1996 to July 2007. According to EC with or without fenestration, the patients were assigned to two groups （group A with fenestration and group B without fenestration）. Mortality, effusions, postoperative mean pulmonary artery pressure, postoperative oxygen saturation, postoperative thrombosis, postoperative neurological problems, and the postoperative loss of sinus rhythm were compared. In group A, 23 patients had fenestration closed interventionally after a mean time of 20-22 months. Results Mortality and postoperative mean pulmonary artery pressure in group B （3 and （15.1±3.4） mmHg, respectively） were significantly higher than group A （0 and （13.2±2.8） mmHg, respectively）. Postoperative oxygen saturation, postoperative thrombosis, postoperative neurological problems, and the postoperative loss of sinus rhythm did not differ between cohorts. Conclusions Fenestrating an extracardiac tunnel seems to improve acute postoperative mortality by rising cardiac output. The induced right-to-left shunt shows no morbidity postoperatively. If a stabilized chronic hemodynamic situation is achieved, an interventional closure of the fenestration can be performed to advance the arterial saturation and improve the exercise tolerance of the patients.

单左室起搏逐跳跟踪生理性房室延迟实现双心室再同步的可行性

目的探讨单左室起搏(LUVP)逐跳跟踪生理性房室延迟(AVD)实现心脏再同步化治疗(CRT)的可行性及其算法。方法入选符合CRTⅠ类适应证并植入三腔起搏器的慢性充血性心力衰竭患者34例,术前动态心电图采集房性早搏。心脏超声下优化AVD及V-V间期,测定LUVP脉冲到右室腔内图起始(LVP-RV)的间期及LUVP需优先于右室的间期(V-R间期),比较两间期的差异并计算左室优先系数(ε),比较LUVP与标准双室起搏(BVP)两种模式的心脏超声指标及QRS波时限差异,测定并比较1min内两个相邻窦性心跳间及最大与最小ASVS间期差异,建立LUVP逐跳跟踪生理性AVD实现CRT的算法。结果房性早搏的生理性AVD的回归方程为:P′R′=0.022+0.954PR,除左室射血分数及左室十二节段达峰时间标准差两种模式比较无差异外(P>0.05),二尖瓣返流面积、主动脉瓣前向血流速度时间积分、心室间机械延迟时间、QRS波时限LUVP均较BVP改善(P<0.01或0.05)。LVP-RV间期(103±16)ms明显大于V-R间期[(20.2±7.0)ms,P<0.01]。ε为0.72±0.03。1min内相邻两个窦性心跳AS-VS间期的最大差值(6.47±1.62)ms明显小于最大与最小AS-VS间期的差值[(16.24±3.29)ms,P<0.01]。结论 LUVP逐跳跟踪生理性房室延迟可实现CRT。

Gore—Tax心外管道治疗先天性心脏病单心室95例临床体会

目的总结Gore—Tax心外管道治疗先天性心脏病单心室的临床体会，探讨心外管道开窗术的治疗效果。方法1996年6月至2010年7月我们对95例先天性心脏病单心室患儿行心外管道纠治术治疗，71例行心外管道开窗术（开窗组），其中58例（58／71）术前高风险患儿术中行开窗术，13例（13／71）患儿因术后低心排行介入开窗术；24例（24／95）术中未行心外管道开窗术（非开窗组）。结果开窗组无死亡；非开窗组死亡3例。开窗组术后胸腔引流时间平均（10．0±3．2）d，非开窗组平均（14．2±2．3）d，差异有统计学意义（P=0．016）；开窗组术后平均肺动脉压（13．2±2．8）mmHg，非开窗组（15．1±3．4）mmHg，差异有统计学意义（P=0．017）；开窗组及非开窗组术后血氧饱和度[（90．3±4．0）％、（91．7±5．2）％]、术后血栓[11．3％（8／71）、12．5％（3／24）]及心律失常发生率[18．3％（13／71）、20．8％（5／24）]相比差异均无统计学意义（P均〉0．05）。结论Gore—Tax心外管道术式治疗先天性心脏病单心室安全、有效；心外管道通过开窗术增加左心室的心输出量，从而降低术后早期病死率。

单左室起搏通过频率适应性房室延迟实现双心室再同步

目的探讨单左室起搏通过频率适应性房室延迟(RAAV)算法跟踪生理性房室延迟(AVD)实现双心室再同步的可行性及治疗慢性充血性心力衰竭(CHF)的效果。方法入选符合心脏再同步化治疗(CRT)Ⅰ类适应证并植入带RAAV功能的三腔或双腔起搏器的CHF患者64例,其中RAAV单左室起搏(LUVP)组(单左室组)32例,以标准双室起搏(BVP)组(标准双室组)32例为对照组。两组给予标准心脏超声优化,比较主动脉前向血流速度时间积分(AVI),左室射血分数(LVEF)、十二节段达峰时间标准差(TS-SD12)、主肺动脉射血前时间差(IVMD)、二尖瓣返流面积(MRA)、EA峰间距(E/A pd)、QRS波时限、年平均治疗费用、NYHA分级、6min步行试验(6MWT)等指标。结果与标准双室组比较,单左室组电池寿命更长[(7.8±0.3)年vs(4.5±0.2)年,P<0.001],QRS波时限短[(136±10)ms vs(142±11)ms,P<0.05],优化耗时较短[(20±4)min vs(52±8)min,P<0.001],MRA更少[(3.1±1.1)cm^2 vs(3.7±1.2)cm2,P<0.05],IVMD缩短[(64.2±12.8)ms vs(72.3±13.6)ms,P<0.05],年均治疗费用低[(1.3±0.1)万元vs(2.2±0.2)万元,P<0.001];AVI增加[(21.8±2.3)cm vs(20.6±2.1)cm,P<0.05],余指标两组比较均无统计学意义(P>0.05)。结论 RAAV单左室起搏可实现双室再同步,疗效不劣于标准BVP,且更符合生理性并降低治疗费用。

单左室起搏实现心脏再同步化治疗对左房结构及功能的影响

目的探讨分析单左室起搏(LUVP)实现心脏再同步化治疗(CRT)对左房结构功能的影响。方法30例接受CRT治疗的慢性心力衰竭(简称心衰)患者,根据起搏模式不同分成两组,其中15例为LUVP,15例为双心室起搏(BVP),收集30例患者CRT术前及术后1、3、6个月内心功能、十二导联心电图及超声心动图临床资料,比较两组NYHA心功能分级,QRS波时限,左房容积指数(LAVI),左房射血分数(LAEF),左室射血分数(LVEF),心室间机械延迟时间(IVMD),室间隔与左室后壁收缩期轴向应变达峰时间差(SPWMD),二尖瓣返流面积(MRA),左室舒张末期内径(LVEED)指标的术前及术后变化。结果与术前相比,LUVP组及BVP组术后6个月NYHA心功能分级降低,QRS波时限、IVMD、SPWMD、MRA、LAVI、LVEED较术前减小,LAEF及LVEF增加(P均<0.05)。与BVP组相比,LUVP组术后6个月QRS波时限缩短[(133.8±9.1)ms vs(141.3±8.4)ms,P<0.05],MRA减小[(1.9±1.0)cm^2 vs(3.0±1.0)cm^2,P<0.05]。结论慢性心衰患者左室及左房结构、功能在CRT术后得到明显改善;同时,在缩短QRS波时限、减小二尖瓣返流面积方面LUVP较BVP更有优势。