一期和分期全腔静脉-肺动脉连接术的围术期护理

目的探讨一期和分期全腔静脉-肺动脉连接术（total cavopulmonary connection，TCPC）的围术期护理方法。方法回顾性分析广东省人民医院2003年12月至2009年11月施行了TCPC术的88例患者的临床资料．其中一期手术41例，二期手术47例。术前做好术前状况评估；术后住监护室期间，做好循环、呼吸监测，观察体静脉与肺动脉的连接通路通畅与否，控制合适的补液量、促进静脉回流，降低肺血管阻力和压力；护理长时间停留的胸管，注意监测早期胸液量：术前、术后针对性地对患儿进行心理辅导和安慰。结果全体患儿术后早期死亡6例，总病死率6．8％（6／88）。一期与分期TCPC两组早期死亡例数各分为5例和1例，病死率率分别为12．2％（5／41）与2．1％（1／47）。所有存活患儿出院前复查超声显示吻合口通畅，心功能评级为纽约心脏协会心功能Ⅱ级。术后早期无1例出现心理异常。结论TCPC患儿的围术期护理，既要针对手术特点重点监护循环、呼吸系统，也要做好心理护理，才能有效提高手术的成功率和术后的恢复质量。     

全腔静脉肺动脉连接术治疗复杂先天性心脏病

目的总结全腔静脉肺动脉连接术(TCPC)的经验和体会。方法6例复杂先天性心脏病患者均在全麻低温体外循环下手术。上腔静脉与右肺动脉吻合完毕后,2例采用右心房内隧道法、4例采用心房外管道完成TCPC手术(合并左位上腔静脉的2例同时行左侧G lenn手术)。结果1例三尖瓣闭锁患者术后2d死亡,5例治愈者血氧饱和度由术前的0.75±0.03提高至术后的0.93±0.02,生活质量改善。结论TCPC矫治复杂先心病,能明显改善患者的缺氧症状;术中肺动脉直接测压是能否行TCPC的重要依据。     

应用双向Glenn手术治疗儿童功能性单心室

目的　探讨双向Glenn手术在治疗儿童功能性单心室类型的复杂先心病中的应用价值。方法　 1998年 1月～ 2 0 0 0年 10月 ,共有 15例患儿在我院接受双向Glenn手术 ,年龄 1～ 13岁 ,平均 (4 94± 3 18)岁 ,体重 10～ 2 9kg,平均(15 13± 5 71)kg。经超声心动图检查、左右心导管术及心血管造影术确诊 ,结合手术中探查确认不宜一期生理矫治。SVC RPA端 -侧吻合 10例 ;SVC与MPA端 -侧吻合 2例 ;LSVC LPA端 -侧吻合 2例 ;LSVC LPA端 -侧吻合及RSVC RPA端 -侧吻合 1例。同期 1例行ASD扩大、1例行PDA结扎、1例合并TAPVC者行肺静脉回流口扩大。结果　有 1例术后早期死于急性肺水肿 ,全组病死率为 6 7%。术后SaO2较术前明显上升 ,P <0 0 1。 1例于术后 6个半月死于上腔静脉与肺动脉吻合口阻塞。结论　双向Glenn手术效果良好 ,作为姑息手术为二期Fontan类手术创造条件 ;也可作为部分高危患儿的终结性姑息手术     

上腔静脉-肺动脉双向分流术后患者60例的心导管检查及临床综合评价

目的通过分析行上腔静脉-肺动脉双向分流术(双向Glenn术)后患者的心导管检查结果,评价双向Glenn术的手术效果及指导复杂先天性心脏病(congenital heart disease,CHD)患者的下一步治疗。方法选择2014年1月至2016年12月60例双向Glenn术后在广东省人民医院接受心导管检查的患者,记录其住院期间的Glenn及全腔静脉-肺动脉连接术(total cavopulmonary connection,TCPC)术前、术后经皮血氧饱和度(percutaneous oxygen saturation,SpO_2)、6 min步行试验(6-minute walk test,6MWT)、心导管检查资料[测量上腔静脉平均血压(mean superior vena cava pressure,m SVCP)、上腔静脉收缩期血压(superior vena cava systolic bloodpressure,sSVCP)、右肺动脉收缩压(right pulmonary artery systolic pressure,sRPAP)、右肺动脉平均压(meanright pulmonary artery pressure,mRPAP)、左肺动脉收缩压(left pulmonary artery systolic pressure,sLPAP)、左肺动脉平均压(mean left pulmonary artery pressure,m LPAP)、肺毛细血管楔压(pulmonary capillary wedge pressure,PCWP),并计算出相应的肺血管阻力(pulmonary vascular resistance,PVR)、肺小血管阻力(small pulmonaryvascular resistance,sPVR)]及术后并发症,通过受试者工作特征曲线(receiver operator characteristicu crve,ROC)寻找进行TCPC的最佳截断点。结果 60例Glenn术后患者的SpO_2较术前明显上升,差异有统计学意义(75.42%±9.62%vs. 86.98%±7.63%,P0.001)。47例TCPC术后患者的SpO_2较术前明显升高,差异有统计学意义(82.70%±5.99%vs. 95.00%±4.07%,P0.05)。42例Glenn术后患者完善6MWT,其步行距离为(362.7±75.0)m,步行6 min后SpO_2较步行前明显下降,差异有统计学意义(81.80%±7.84%vs. 67.59%±1.82%,P0.05)。47例患者能进行下一步TCPC手术。进行TCPC组的sRPAP、mRPAP、mLPAP、PVR、sPVR均明显低于不进行TCPC组,差异有统计学意义(P0.05)。两组间sSVCP、mSVCP、sLPAP比较,差异无统计学意义(P0.05)。sSVCP≤20 mmHg(P=0.025,1 mmHg=0.133 kPa)是TCPC最佳截断点。sRPAP≤22 mmHg(P=0.0001),mRPAP≤13 mmHg(P=0.003),sLPAP≤27 mmHg(P=0.03),mLPAP≤11 mmHg(P=0.01),PVR≤4.3 Wood U/m2(P0.0001)与TCPC相关,mSVCP≤19 mmHg(P=0.06)和sPVR≤2.0 wood U/m2(P=0.0531)与TCPC不相关。结论双向Glenn术、TCPC术使患者术后SpO_2得到明显改善;6MWT可作为患者接受TCPC术前的临床评价指标;mPAP、PVR等血流动力学指标是影响TCPC手术指征的重要影响因素;sSVCP≤20 mmHg、sRPAP≤22 mmHg、m RPAP≤13 mmHg、sLPAP≤27 mmHg、mLPAP≤11 mmHg、PVR≤4.3 woodU/m~2时,可认为耐受TCPC术。     

右心旁路及右心室肺动脉旁路手术后补液标准探讨

1　对象与方法　　自 1996年 6月至 2 0 0 1年 11月 ,采用右心旁路及右心室肺动脉旁路手术治疗肺血减少型重症复杂性先天性心脏病 62例 ,年龄 0 5～ 7 0岁 ,平均 (3 0 4± 1 5 8)岁 ,体重 6～ 2 3kg ,平均 (12 66± 3 5 8)kg。采用的手术方法分别是 :右心室肺动脉旁路手术 19例 (经典Rastelli手术 11例 ,右心室肺动脉外通道手术 8例 ) ;右心旁路术 43例 ,包括 :改良Fontan手术 12例 ,全腔静脉肺动脉连接 (TCPC) 4例 ,一个半心室矫治术 2例 ,双向Glenn手术 2 5例 (其中体外循环下 8例 ,非体外循环17例 )。手术后早期死亡 5例 (8 0 6% …     

肺动脉环缩术在先天性心脏病分期手术中的应用效果观察

目的观察肺动脉环缩(PAB)术在先天性心脏病分期手术治疗中的应用效果。方法选取2008年1月—2018年12月北京安贞医院小儿心脏中心行PAB术的先天性心脏病患儿205例,其中男101例,女104例;中位数年龄0.6个月;体重2.5～26.0 kg,中位数6.6 kg。根据实施PAB手术的目的分为双心室矫治组(97例)、单心室矫治组(72例)和左心室训练组(36例)。分别检测相应的临床指标,并进行随访。结果双心室矫治组和单心室矫治组Ⅰ期PAB术后肺动脉平均压(mPAP)以及经皮血氧饱和度(SpO_2)均较术前明显下降(P0.05);左心室训练组术后mPAP、SpO_2均较术前明显上升(P0.05)。205例患儿住院内期间死亡7例,死亡率为3.4%。PAB术后共实施Ⅱ期手术117例(57.1%)。其中双心室矫治组47例,距PAB术后间隔时间5～40个月,中位数17.6个月。单心室矫治组行双向腔肺吻合术(Glenn术)43例,距PAB术后间隔时间15～53个月,中位数18.3个月;行全腔静脉-肺动脉连接术(Fontan术)13例,距PAB术后间隔时间52～112个月,距Ⅱ期Glenn手术间隔27～52个月。左心室训练组完成大动脉调转术(ASO)27例,距PAB术后间隔时间6 d至35个月,中位数时间0.3个月。结论 PAB作为姑息手术,在降低肺动脉压力,保护肺血管床、实施心室功能训练、完成先天性心脏病的分期矫治方面具有重要作用。     

双向腔肺分流术在复杂先心病治疗中的应用

目的探讨运用双向腔静脉-肺动脉分流术（BCPS）治疗复杂先心病的疗效及安全性。方法47例复杂先心病患者,21例在体外循环下,26例在非体外循环下完成BCPS手术。共行单侧BCPS术30例,双侧BCPS术17例。结果术后同术期死亡3例,病死率6．38％。随访35例,有7例术后2年左右进行二期根治手术,2例术后2年死亡,余26例心彩超复查无吻合口狭窄,发绀得到不同程度的缓解,心功能改善,生活质量明显提高。结论双向腔静脉-肺动脉分流术对于不能一期矫治的功能性单心室类复杂先心病患者是一种有效的姑息手术方法,非体外循环下行腔肺吻合术有助于心、肺功能的早期恢复,还可减少患者的住院开支,是安全、可行且有利的。     

Fontan循环

Fontan手术为全腔静脉一肺动脉连接术,是Glenn术后的二期手术方式。常用方法为使用心内隧道或心外管道将腔静脉血流完全导入肺动脉,使功能性单心室患儿获得生存机会。目前Fontan手术的手术指征较“经典”十大标准大为扩大,对于尚难直接Fontan手术时推荐分期手术,手术方式的改进提高了Fontan技术。手术的一些并发症影响了术后的转归,同时,对Fontan手术尚存一些争议。     

Fontan循环

Fontan手术为全腔静脉一肺动脉连接术,是Glenn术后的二期手术方式.常用方法为使用心内隧道或心外管道将腔静脉血流完全导入肺动脉,使功能性单心室患儿获得生存机会.目前Fontan手术的手术指征较"经典"十大标准大为扩大,对于尚难直接Fontan手术时推荐分期手术,手术方式的改进提高了Fontan技术.手术的一些并发症影响了术后的转归,同时,对Fontan手术尚存一些争议.     

Glenn手术治疗合并静脉畸形引流的复杂先天性心脏病

目的探索双向Glenn手术治疗合并静脉畸形引流的复杂先天性心脏病（先心病）的临床效果。方法 2010年3月—2013年12月我科共对29例合并静脉畸形引流的复杂先心病病例实施双向Glenn手术。20例合并体静脉回流异常,6例合并肺静脉畸形引流,3例同时合并体肺静脉畸形引流。结果全组病例均行单侧或双侧双向Glenn手术,并有3例结扎细小的左上腔静脉,1例左侧改良B-T分流术,3例肺静脉畸形引流矫治,4例房室瓣成形术,3例肺动脉环缩术。围术期全组1例（3.4%）因恶性心律失常死亡,2例开胸止血;2例心包积液,1例脑栓塞,1例切口裂开。随访17.4个月±10.9个月无死亡,血氧饱和度（84±6）%,2例行完全性腔静脉肺动脉连接术,1例因预激综合征行射频消融术,2例需降肺动脉压力药物治疗。结论合并体静脉和/或肺静脉畸形引流的复杂先心病病例行双向Glenn手术早中期效果满意,肺静脉畸形情况和是否梗阻将直接影响手术方式,联合多种术式可能是改善手术效果的重要方法。     

心外管道与直接吻合全腔静脉-肺动脉连接术早期和中期疗效对比研究

目的比较心外管道与直接吻合全腔静脉-肺动脉连接术早期和中期疗效。方法选择2005年6月至2009年12月在广东省心血管病研究所行心外全腔静脉-肺动脉连接术的患者53例为研究对象，其中15例行下腔静脉-肺动脉直接吻合全腔静脉-肺动脉连接术（直接吻合组），38例行心外管道全腔静脉-肺动脉连接术（心外管道组）。随访（23．0±15．6）个月，比较两组患者的年龄、体质量、术前红细胞比容、血氧饱和度、M率分布等术前情况；术中体外循环和主动脉阻断时间；早期指标[术后早期失败率（死亡或撤除）、住院时间、外科加强监护病房（surgery intensive care unit，SICU）时间、机械通气时间、血氧饱和度、中心静脉压、术后红细胞比容、胸管引流时间、乳糜胸发生率及心律失常等其他并发症]及中期指标[中期病死率（死亡和纽约心脏协会心功能Ⅳ级）、心律失常、人工管道狭窄或梗阻及其他并发症]。结果（1）两组年龄、术前体质量、术前红细胞比容、血氧饱和度、M率分布、术中体外循环和主动脉阻断时间比较，差异无统计学意义（P〉0．05）。（2）直接吻合组术后胸管引流时间短于心外管道组，差异有统计学意义[（15．5±9．6）d眠（24．1±19．5）d，t=2．245，P=0．030]；两组其余早期临床疗效指标（住院时间、早期失败率、SICU时间、中心静脉压、术后红细胞比容、乳糜胸发生率、心律失常等其他并发症）比较，差异无统计学意义（P〉0．05）。（3）两组中期病死率比较，差异无统计学意义[8．3％（1／13）vs．5．7％（2／35），P〉0．05]。直接吻合组患者术后计算机断层扫描重建显示下腔静脉一主肺动脉吻合口血流通畅无梗阻．无狭窄。结论下腔静脉一肺动脉直接吻合全腔静脉一肺动脉连接术适合满足Fontan类手术条件，同时心脏解剖合适的患者。手术可以获得心外管道全腔静脉肺动脉连接术良好的临床效果，同时避免了使用外源性材料。     

心外管道全腔肺动脉连接术在复杂先心病中的应用

目的：总结心外管道全腔肺动脉连接（total cavopulmonary connection,TCPC）术治疗复杂先天性心脏病的临床经验。方法：2002年1月～2011年5月,采用外管道TCPC术矫治复杂先天性心脏病47例（病种包括三尖瓣闭锁、单心室、肺动脉闭锁、右心室发育不良、右室双出口）患者,手术年龄（8±4）岁。一期手术25例,分期手术22例。结果：术后早期（术后1月）死亡4例（8%）。死亡原因：多脏器功能衰竭、心脏骤停、感染、蛋白丢失性肠病和低心排。术后早期并发症发生率为17%,主要为肺水肿3例、胸腔积液2例、心律失常1例、心包积液1例、蛋白丢失性肠病和低心排1例。术后均随访,随访时间9～87月。远期再住院率为9%（4/43）,再次手术7%（3/43）。2例再次出现胸腔积液,2例因自行停服肠溶阿司匹林后出现外管道堵塞再次手术,1例于术后出现低心排死亡。其余患者恢复良好。结论：对于适应证明确的复杂先天性心脏病患者行心外管道TCPC手术治疗,近期临床效果满意。     

Reopening of persistent left superior caval vein after bidirectional cavopulmonary connections

Persistent left superior vena cava (SVC) is a not uncommon finding in patients with congenital heart disease. This anatomical variant must be recognised before doing a Glenn anastomosis, bidirectional cavopulmonary connection or a Fontan-type procedure. Following these procedures, reopening of a left SVC leading to clinical cyanosis can occur. Five cases are described in whom persisting left SVCs were excluded before performing a bidirectional cavopulmonary connection or Fontan procedure but (re-)opened after surgery, leading to cyanosis either by reducing effective pulmonary blood flow (bidirectional cavopulmonary connection) or by an obligatory right to left shunt (Fontan). These observations suggest that, embryologically, the lumen of the left SVC obliterates rather than disappears. Balloon occlusion angiography of the innominate vein before cavopulmonary connections or Fontan procedures might improve detection of these collateral vessels.

Keywords: persistent left superior vena cava; cavopulmonary connection; Fontan procedure; congenital heart disease     

A novel use of the Amplatzer muscular ventricular septal defect occluder.

We report a case of a 12-month-old-infant with double outlet right ventricle and pulmonary stenosis who presented with signs of superior vena cava syndrome secondary to a dysfunctioning bidirectional Glenn shunt. The patient was successfully treated with transcatheter obstruction of an accessory pulmonary blood flow using the Amplatzer muscular ventricular septal defect occluder.     

Occlusion of azygos vein via direct percutaneous puncture of innominate vein following cavopulmonary anastomosis

A 2-year-10-month-old boy was diagnosed with a complex congenital heart disease: right atrial isomerism, left superior vena cava (LSVC), complete atrioventricular septal defect, secundum type atrial septal defect, transposition of the great arteries with pulmonary atresia, patent ductus arteriosus, absence of a right superior vena cava (RSVC), and dextrocardia. He had received a left Blalock-Taussig (BT) shunt at the age of 3 months and a left bidirectional Glenn shunt one year after BT shunt. Progressive cyanosis was noted after the second operation and cardiac catheterization showed a functional Glenn shunt with an engorged azygos vein, which was inadvertently skipped for ligation. Because of the absence of RSVC, transcatheter occlusion of the azygos vein was performed successfully via direct puncture of the innominate vein.     

A new combined surgical interventional method for univentricular palliation of congenital heart defects

Total cavo-pulmonary anastomosis is frequently performed to palliate patients with a broad variety of congenital heart defects with functionally univentricular hearts precluding biventricular circulation. In patients with risk factors for primary repair a stepwise approach is frequently chosen with initial creation of an aorto-pulmonary shunt followed by a Glenn anastomosis or hemifontan procedure. Finally a total cavo-pulmonary connection is completed surgically. The aim of this feasibility study was to develop a combined surgical-interventional approach for creation of a total cavo-pulmonary anastomosis which reduces the number of surgical interventions, precludes extracorporeal circulation and intracardiac sutures, putting the patients at risk for subsequent rhythm disturbances. METHODS: A Glenn anastomosis was created in 10 sheep without cardio-pulmonary bypass. The superior vena cava was banded superior to the cavo-atrial junction leaving a minimal lumen for subsequent interventional balloon dilatation and implantation of a stent graft. A 15 mm Gore-Tex tube was sutured around the inferior vena cava intrapericardially to provide resistance for subsequent interventional stent implantation. Total cavo-pulmonary anastomosis was completed interventionally by dilating the banded vena cava superior and connection of the inferior vena cava to the superior vena cava by implanting an Aneurx covered stent graft. RESULTS: All animals survived the combined surgical-interventional procedure. Stent deployment was possible without causing obstruction to the hepatic veins. Neither stent dislocation nor rhythm disturbances were encountered. CONCLUSIONS: Total cavo-pulmonary anastomosis can be achieved without intracardiac sutures and cardio-pulmonary bypass by use of a combined surgical-interventional approach using the Aneurx covered stent graft.     

Catheter closure of accessory pulmonary blood flow after bidirectional Glenn anastomosis using Amplatzer duct occluder.

Additional source of pulmonary blood flow in patients with bidirectional cavopulmonary anastomosis (Glenn shunt) may cause elevation of the pulmonary artery pressure precluding safe completion of the Fontan operation. A case is presented with single-ventricle bidirectional cavopulmonary anastomosis and additional flow from the ventricle to the pulmonary artery resulting in elevated Glenn pressure. The communication was successfully occluded using Amplatzer duct occluder with satisfactory reduction in the Glenn pressure.     

Pulmonary blood flow distribution after the total cavopulmonary connection for complex cardiac anomalies

Summary In total cavopulmonary connection (TCPC), the anastomotic portion of the caval veins to the pulmonary artery (PA) is decided empirically based on personal experience. To compare the pulmonary flow distribution from both caval veins in various types of cavopulmonary anastomosis, intrapulmonary ventilation-perfusion distribution after TCPC was studied using lung scanning. We studied 11 patients, 2 to 37 years old, at 30–84 months after TCPC. Lung scanning was performed by administering 185 MBq of xenon-133 saline solution from their upper extremities and, after xenon-133 was washed out, from their lower extremities. Radionuclide counts on both lungs were obtained and intrapulmonary ventilation-perfusion distribution was assessed. In 4 patients whose superior vena cava (SVC)-PA anastomosis was on the right side of the inferior vena cava (IVC)-PA anastomosis, the blood flow distribution of the right and left lungs was 57.4%: 42.6%. In 3 patients whose SVC-PA anastomosis was on the left side of the IVC-PA anastomosis, the blood flow distribution of the right and left lungs was equal in both lungs (right, 53.1%; left, 46.9%). Systemic arterial oxygen saturation increased after TCPC (before TCPC, 85.3% ± 2.7% and after TCPC, 89.8% ± 2.3% (P < 0.05) in group R; before TCPC, 86.1% ± 2.8% and after TCPC, 93.6% ± 0.6% (P < 0.02) in group L). After TCPC, the value in group L had a tendency to be greater than that in group R (P < 0.04), in spite of the same values of systemic arterial oxygen saturation before TCPC and cardiac index (group R, 2.9 ± 0.96; group L, 3.4 ± 0.37). Lung scanning with xenon-133 revealed the distribution of pulmonary blood flow in the patients after TCPC quantitatively, and in the patients whose SVC-PA anastomosis was on the left side of the IVC-PA anastomosis, the right and left balance of the pulmonary blood flow distribution appeared to be more balanced compared with patients whose connection was done the opposite way.