非体外循环下改良全腔静脉-肺动脉连接术

目的：探讨非体外循环下行改良全腔静脉－肺动脉连接手术治疗功能性单心室技术。方法：3例功能性单心室病儿在非体外循环下施行了改良全腔静脉－肺动脉连接手术。术中先作上、下腔静脉插管并与右房插管连接，静脉血分流入右房，然后置右肺动脉侧壁钳，上腔静脉远心端与右肺动脉上缘行端侧吻合，吻合口尽量偏左；下腔静脉通过心外管道与右肺动脉下缘连接，吻合口尽量偏右。最后缝扎肺动脉口。结果：术后无早、晚期死亡，仅1例发生低心输出量综合征及胸腔渗出。动脉血氧饱和度0．94－0．97。术后随访1－3个月，心功能I级2例，Ⅱ级1例。结论：非体外循环下行改良全腔静脉－肺动脉连接术，是治疗功能性单心室的有效技术，可取得良好的手术效果。     

心外管道全腔静脉肺动脉连接术

目的 报告心管道全腔静脉肺动脉连接术（TECPC）应用经验。方法 横断上腔静脉与右肺动脉端侧吻合,切断下腔静脉前壁,保留原位吻合口,将下腔静脉通过人工血管与主肺动脉吻合,共治疗9例复杂性先天性心脏病,其中1例单心室改良Fontan术后4年频发室上性心动过速而改行TECPC。结果 全组手术后均生存。3例术后出现胸腔积液、乳糜胸并发症、均治愈。血流动力学指标满意,术后随访心功能Ⅰ-Ⅱ级,无心律紊乱发生。结论 TECPC手术操作简单,并发症少,适应证广泛,优于改良Fontan手术和传统的全腔静脉肺动脉连接术。     

心外管道全腔静脉-肺动脉连接术在复杂先天性心脏病外科治疗中的应用

目的总结心外管道全腔静脉-肺动脉连接术治疗复杂先天性心脏病的应用经验及其治疗效果。方法回顾性分析2006年9月至2012年12月间广州军区广州总医院心脏外科中心52例行心外管道全腔静脉-肺动脉连接术患者的临床资料。12例行一期心外管道全腔静脉-肺动脉连接术,40例为双向Glenn手术后行二期心外管道全腔静脉-肺动脉连接术。分析所有患者的临床资料,并比较两种手术方式患者的死亡率、并发症发生率、住院时间、住重症监护室（ICU）时间、机械辅助通气时间、动脉血氧饱和度的改善情况等。结果围术期死亡2例,死亡率3．8％。其中1例术后因严重低心排血量综合征死亡,1例术后因多器官功能衰竭死亡;50例治愈出院。二期心外管道全腔静脉．肺动脉连接术患者（40例肌械辅助通气时间、住ICU时间、住院时间明显短于一期心外管道全腔静脉-肺动脉连接术患者（12例）,但两种手术方式患者术后并发症发生率及术后动脉血氧饱和度（二期手术与一期手术比较：93％±3％vs．94％±3％）、死亡率（二期手术与一期手术比较：2．5％vs．8．3％）差异均无统计学意义（P〉0．05）。随访45例（90％）,随访时间6～52个月,随访期间无死亡。术后3个月,存活患者心功能均为Ⅰ～Ⅱ级,心脏彩色超声心动图显示：腔静脉肺动脉吻合口血流通畅。结论心外管道全腔静脉．肺动脉连接术血流动力学更符合生理血流动力学特点,手术操作简捷,是不能进行双心室治疗时的有效手术术式;分期心外管道全腔静脉-肺动脉连接术较一期心外管道全腔静脉-肺动脉连接术手术适应证广泛,术后恢复较好,更易推广。     

应用系列改良Fontan手术治疗复杂先天性心脏病

目的探讨系列改良Fontan手术的特点和治疗复杂先天性心脏病的效果，进一步提高对其临床应用的认识。方法回顾性分析1992年9月～2006年6月期间77例复杂先天性心脏病患者行改良Fontan手术治疗的临床资料，其中行右心房肺动脉吻合术21例，心房内板障或管道全腔静脉-肺动脉连接术28例，心外人工管道全腔静脉-肺动脉连接术24例，自体右房壁管道全腔静脉肺动脉连接术2例，自体带蒂心包心外管道全腔静脉-肺动脉连接术1例，主肺动脉与下腔静脉吻合全腔静脉-肺动脉连接术1例。结果术后早期死亡5例，其中死于心力衰竭3例，突发心律失常1例，脑出血1例。再次手术1例，术后早期生存率93．5％（72／77），手术成功率92．0％。左心室舒张期末内径（LVEDD）较术前减小（52．5±7．8mm vs．62．5±11．0mm，P=0．013），左心室射血分数（LVEF）较术前增加（68．5％±4．0％vs ．62．0％±4．5％，P=0．032）。随访63例，随访率87．5％（63／72），随访时间1～15年。随访期间死亡4例，远期再次手术1例，远期生存率88．3％，手术成功率86．0％。结论在复杂先天性心脏病的治疗中，改良Fontan手术有良好的疗效；同时根据具体解剖结构可选择不同的手术方式。     

心房内管道行全腔静脉—肺动脉连接术

自１９９０年１月至１９９５年１月共施行全腔静脉－肺动脉连接术３２例，其中单心室２例，三尖瓣闭锁１例。应用心房内管道行全腔静脉－肺动脉连接术。该手术方法常规将上腔静脉远心端与右肺动脉行端侧吻合，然后选用直径〉１．５ｃｍ人工血管，将下腔静脉与主肺动脉吻合，方法简便，易于操作。其优点是：右房组织损伤小；血流动力学合理；更适用于伴共同心房和共同房室瓣等复杂畸形；选用直径１．５ｃｍ以上人工血管无需再更换；术     

心房内管道行全腔静脉─肺动脉连接术

自１９９０年１月至１９９５年１月共施行全腔静脉—肺动脉连接术３２例，其中单心室２例，三尖瓣闭锁１例。应用心房内管道行全腔静脉—肺动脉连接术。该手术方法常规将上腔静脉远心端与右肺动脉行端侧吻合，然后选用直径＞ｌ．５ｃｍ人工血管，将下腔静脉与主肺动脉吻合，方法简便，易于操作。其优点是：右房组织损伤小；血流动力学合理；更适用于伴共同心房和（或）共同房室瓣等复杂畸形；选用直径１．５ｃｍ以上人工血管无需再更换；术后心律紊乱明显减少。     

不同全腔静脉肺动脉连接术式对腔静脉回流、肺血分布以及能量消耗的影响

目的：探讨不同的全腔静脉肺动脉连接（TCPC）术式对腔静脉回流、肺血分布以及能量消耗的影响。方法：建立接近临床TCPC术的实验动脉模型，采用3种不同的TCPC术式：（1）上、下腔静脉与右肺动脉吻合口相对；（2）下腔静脉与右肺动脉吻合口偏向上腔静脉与右肺动脉吻合口的右侧；（3）下腔静脉与主肺动脉吻合口位于上腔静脉与右肺动脉吻合口左侧。术后测量上、下腔静脉血流量，左、右肺动脉血流量，进行肺血管造影了解肺血分布，通过伯努力公式计算能量消耗。结果：3种TCPC术后上、下腔静脉血流量的分布发生了变化，上腔静脉／下腔静脉血流量比值在1．0左右；通过测定左、右肺动脉流量以及造影发现，术式（3）下腔静脉血基本进入左肺；术式（2）下腔静脉血较多进入右肺；右肺动脉／左肺动脉血流量与术前比差异有显著性（P＜0．01）；术式（3）能量消耗较术式（1）显著降低（P＜0．01）。结论：TCPC术后，体静脉回流情况发生了变化；不同TCPC术后，腔静脉位置决定了静脉回流的方向；右肺动脉／左肺动脉血流量比值较术前差异有显著性（P＜0．01）；TCPC术后，上、下腔静脉吻合口相对时能量消耗最大，完全错开时能量消耗小。     

全腔肺动脉连接术在复杂先心病手术中的应用

１９９２～１９９３年为９例复杂先心病病人行全腔肺动脉连接术。均在心脏跳动下作双向上腔静脉—肺动脉吻合；１例作上腔静脉近心端与右肺动脉侧侧吻合，８例作右房上腔静脉开口与主肺动脉端端吻合。心房内管道置入５例，复合性内通道２例，心房内分隔２例。平均主动脉阻断时间３０分钟，平均心肌温度１０℃，全部心脏自动复跳，病人均痊愈出院。结论：对于复杂的先心病，全腔肺动脉吻合术的姑息性效果较其它改良Ｆｏｎｔａｎ术式要好，但远期效果有待进一步观察和评估     

不同Fontan手术的动物实验对比研究

目的 通过动物实验模似右房－右室连接术、全腔静脉肺动脉连接术及右房－肺动脉吻合术,对比研究３种术式的血流动力学效果及能量损耗情况。方法 选１５只成年杂种犬,随机分成右房－右室连接、全腔静脉肺动脉连接及右房－肺动脉吻合３种,每组５只。右房－右室连接组分别利用５０％及２５％的右室容积参与Ｆｏｎｔａｎ循环。比较三间组的血流动力学参数及能量损耗。结果 与右房－肺动脉连接组相比,无论是５０％还是２５％的右室     

全腔静脉肺动脉连接术的疗效与术式评价

目的 探讨全腔静脉肺动脉连接术（TCPC）的外科治疗效果并对相关术式进行评价。方法 1998年2月至2006年6月，对51例复杂先天性心脏病病儿采用TCPC予以纠治，15例采用心内隧道术式、29例采用心外管道术式、7例采用主肺动脉与下腔静脉直接吻合术式。结果 手术死亡4例（7.8％）。此外，术后低心排出量综合征6例（12．8％）、心律失常14例（29．8％）。80．1％术后24h内撤离呼吸机。术后48．9％留置胸引流管超过7d。随访1个月至8年，9例（19．1％）术后6个月后仍需长期或间断服用强心、利尿剂，7例（14．7％）有房性心律失常。结论 全腔静脉肺动脉连接术可取得满意的外科治疗效果。应用主肺动脉下腔静脉直接吻合术式，在部分病例中可望成为理想术式。     

The Use of the Right Atrial Flap in Total Cavopulmonary Connection

A technical modification of total cavopulmonary anastomosis (TCPC) is described. Inferior vena cava (IVC) channel is constructed from the right atrial wall in a fashion similar to Senning's operation. The use of Gore-TexR (W.L. Gore & Associates, Inc.) patch or tube is avoided. We have used the technique in 11 patients between 1988 and 1991 (six complex transpositions or double outlets with one hypoplastic ventricular chamber and/or straddling of the atrioventricular (AV) valve, four double inlet ventricles with pulmonary stenosis, and one tricuspid atresia). Superior vena cava (SVC)/IVC to pulmonary artery gradient was less than 2 mmHg in all patients. SVC/IVC pressure was 10-15 mmHg (mean 12.3 mmHg), transpulmonary gradient 5-8 mmHg (mean 6.4 mmHg). We have not observed any adverse effects such as arrhythmias or increased pleural drainage when we compared those patients with 85 children in whom the TCPC was performed with Gore-TexR patch/tube. The presented technique is simple, avoids the use of anticoagulants, and may have a possible long-term advantage in allowing growth of the IVC channel.     

Modified Fontan Procedure: Atrial Baffle and Systemic Venous to Pulmonary Artery Anastomotic Techniques

Two technical modifications to the modified Fontan procedure are presented. Systemic venous to pulmonary artery continuity is achieved by superior vena cava (SVC) division and end to side anastomosis to the right pulmonary artery (RPA), particularly following a right Blalock shunt, or by RPA division and anastomosis to the SVC, particularly in the presence of RPA stenosis. Intra-atrial partitioning is achieved by a systemic venous baffle rather than a pulmonary venous baffle. This is particularly useful in the presence of left atrioventricular valve atresia, but may be a preferable technique with double-inlet single ventricle or single ventricle with common AV valve. These techniques were applied successfully to 10 of 12 children with various forms of single ventricle, including 5 with left AV valve atresia or stenosis.     

Presurgical Evaluation of Fontan Connection Options for Patients With Apicocaval Juxtaposition Using Computational Fluid Dynamics

Apicocaval juxtaposition (ACJ) is a rare congenital heart defect associated with single ventricle physiology where optimal positioning of the Fontan conduit for completion of total cavopulmonary connection (TCPC) is still controversial. In ACJ, the cardiac apex is ipsilateral with the inferior vena cava (IVC), risking kinking and collapse of the Fontan conduit at the apex of the heart. The purpose of this study is to evaluate two viable routes for Fontan conduit connection in patients with ACJ, using computational fluid dynamics. Internal energy loss evaluations were used to determine contribution of conduit curvature to the energy efficiency of each cavopulmonary anastomosis configuration. This percentage of energy loss contribution was found to be greater in the case of a curved extracardiac conduit connection (44%, 4.1 mW) traveling behind the ventricular apex, connecting the IVC to the left pulmonary artery, than the straighter lateral tunnel conduit (6%, 1.4 mW) installed through the ventricular apex. In contrast, net energy loss across the anastomosis was significantly lower with extracardiac TCPC (9.3 mW) in comparison with lateral tunnel TCPC (23.2 mW), highlighting that a curved Fontan conduit is favorable provided that it is traded off for a superior cavopulmonary connection efficiency. Therefore, a relatively longer and curved Fontan conduit has been demonstrated to be a suitable connection option independent of anatomical situations.     

腔静脉畸形的外科意义及手术治疗

本文报告先天性腔静脉畸形53例，双上腔静脉畸形，左上腔静脉引流入冠状静脉窦45例，对血流动力学无影响，术中仅4例作了插管引流，余仅在术中暂时阻闭了左上腔静脉，本文重点报道8例少见腔静脉畸形，包括右上腔静脉缺如（1例），左上腔静脉引流入右房顶（1例），引流入左房顶（2例），引流入冠状静脉窦并与左房交通（1例），下腔静脉引流入左房（1例，上腔和下腔静脉分别引流入左房（1例），以及全部体静脉分别引流入左房（1例），此8例的畸形矫治均较顺利，本文对少见腔静脉畸形的临床特征，诊断方法，手术矫治的要点和注意事项进行了较详细探讨。     

Laser flow measurements in an idealized total cavopulmonary connection with mechanical circulatory assistance

This study examined the interactive fluid dynamics between a cavopulmonary assist device and univentricular Fontan circulation. We conducted two-dimensional particle image velocimetry measurements on an idealized total cavopulmonary connection (TCPC) with an axial pump prototype intravascularly inserted into the inferior vena cava (IVC) and then in the IVC and the superior vena cava (SVC) for a dual-pump support case. The glass model of the TCPC consisted of rigid vessels having a diameter of 13.4 mm and a one-diameter vessel offset at the TCPC junction. Fluid velocity profiles were examined at a cardiac output of 3 L/min and SVC and IVC flow ratios of 30/70%, 40/60%, and 50/50% and pump rotational speeds from 3000 to 9000 rpm. In addition, cardiac outputs of 5 and 7 L/min were also examined. As compared to the flow profile with the pump present, the measured velocity field demonstrated the presence of rotational (i.e., out of plane) motion, which forced the higher-velocity regions toward the periphery of the vessel. As a result, few flow vortices were captured in the image plane downstream of the pump in the TCPC junction. However, the velocity profiles for all cases demonstrated the expected shunting preference of IVC flow toward the right pulmonary artery. Furthermore, the inclusion of the pump provided a pressure rise of 3 to 9 mm Hg, which would be sufficient to relieve systemic hypertension in Fontan patients with circulatory dysfunction.     

Management of a small bowel transplant with complicated central venous access in a patient with asymptomatic superior and inferior vena cava obstruction

During the past few years, small bowel transplantation (SBT) has become a realistic alternative for patients with irreversible intestinal failure who have or will develop severe complications from total parenteral nutrition (TPN). Transplantation can be associated with large fluid shifts and massive blood loss necessitating rapid infusions of large quantities of crystalloid and/or blood products. Invasive monitoring and large-bore venous access are necessary in order to manage these patients intraoperatively. Because patients with irreversible intestinal failure are often managed with total parenteral nutrition via a central venous catheter, thrombotic intraluminal obstruction of major vessels may develop over time. Additionally, this may lead to superior vena cava (SVC) syndrome as well as challenging problems with vascular access. We present a 34-year-old woman with a past medical history for long-standing Crohn's disease with multiple small bowel resections and short gut syndrome who presented for an SBT. The patient had a long history of TPN use, complicated by SVC syndrome and inferior vena cava (IVC) obstruction. She was presently asymptomatic from her SVC obstruction. Central venous access was obtained by an interventional radiologist. A 7-French double-lumen Hickman minicatheter was placed in the left femoral vein with the tip of the catheter positioned just distal to the IVC narrowing. A left radial 20-gauge arterial line was placed for hemodynamic monitoring and frequent blood sampling. The patient's left and right dorsal-saphenous veins were cannulated with 16-guage catheters and adequate flow was observed. Lower extremity pressure was measured via the Hickman catheter in the left femoral vein. A multiplane transesophageal echo was used to assess ventricular volume. The options and intraoperative management of such patients are discussed.     

Total cavopulmonary connection in children with body weight less than 10 kg.

OBJECTIVE: To evaluate the results after total cavopulmonary connection (TCPC) in small children, our clinical experience was retrospectively reviewed. METHODS: Of 164 patients undergoing TCPC, the body weight at operation was less than 10 kg (8.8+/-1.1 kg) in 54, including 21 with visceral heterotaxy. The superior caval vein (SVC) was anastomosed to the pulmonary arteries in a bidirectional fashion. To construct a channel draining the inferior caval vein (IVC), an extended polytetrafluoroethylene (ePTFE) tube was placed intraatrially (in 15 patients) or outside the heart (in 13), its diameter being 14 mm in two patients, 16 mm in 12, and 18 mm or greater in 14. A heterologous pericardial baffle was used for intraatrial rerouting in 12 patients. A pedicled autologous pericardial roll was tailored as an extracardiac conduit in 11 patients, and the pulmonary trunk was directly anastomosed to IVC orifice in three. RESULTS: Seven patients, including five with right isomerism, died in the intermediate term because of infection of the ePTFE tube in two, respiratory problems in three, atrioventricular valvar regurgitation in one, and pulmonary venous obstruction in one. Postoperative catheterization showed; SVC pressure, 11+/-2 mmHg without a pressure gradient between SVC and IVC; systemic ventricular end diastolic pressure, 5+/-2 mmHg; end diastolic volume, 122+/-54% of the anticipated normal value; ejection fraction, 0. 56+/-0.11; and cardiac index, 2.9+/-0.7 l/min per m(2). With the follow-up of 1-116 (35+/-31) months, the IVC channel has not become obstructive in all, except for one, in whom a pedicled pericardial roll was severely obstructed because of its tortuous extracardiac course crossing in front of the vertebrae. Postoperative growth was generally stable, although body weights and heights were smaller in the majority of patients when compared with the anticipated standards for Japanese children. CONCLUSION: TCPC can be justifiably established in small children. The use of autologous tissues seemed preferable for constructing the IVC channel unless anatomic orientation was unsuitable.     

Factors influencing permanent catheter performance

Permanent dual lumen catheters (PDLC) provide an alternative vascular access in patients considered unsuitable for arteriovenous fistula, graft or peritoneal dialysis. However, the use of PDLC is often complicated by inadequate blood flow. The aim of this study was to identify catheter dysfunctions. We studied prospec-tively 57 chronic hemodialyzed patients, 73+/-11 years of age, with PDLC for 18+/-14 (1-48) months. Catheters were tunneled in silicone (MedComp Tesio n= 40) or in polyurethane (Permcath Quinton n = 11, GamCath Gambro n = 6) in left or right internal jugular (n = 49), in left or right subclavian (n = 3) and in right femoral vein (n = 5). We studied the blood viscosity indices (hematocrit, total protein, cholesterol and triglycerides), catheter intra-dialytic parameters (pre-pump and venous pressure), localization of the catheter tip (superior vena cava = SVC, right atrium = RA, inferior vena cava = IVC), blood pressure before and after hemodialysis during the 3 last dialyses, use of anticoagulant (ACT) or antiaggregant therapy (AAT) and previous infectious episodes. The mean blood flow was 269+/-37 ml/min (median 280 ml/min). The patients were divided according to the median value into groups I (Qb < 280, n = 28) and group II (Qb > 280, n =29). Results: Blood viscosity, patients' mean arterial pressure and venous catheter line pressure did not differ between the two groups. Pre-pump pressure, at the start and at the end of treatment, was higher in group I. ACT, AAT and previous infectious episodes could not explain the low-performance. Blood flows of catheters localized in RA, SVC, and in IVC were respectively 287+/-20, 268+/-39, 244+/-27 ml/min. In the first case the Qb was significantly higher than IVC (p = 0.03) and SVC (p = 0.04). In conclusion, the most important factor influencing blood flow rates seems to be the position of the catheter tip in the venous system. The best blood flows were found in catheters with the tip localized in the right cardiac cavities, while PLDC placed in inferior vena cava showed lower blood flow.     

A case report of total anomalous pulmonary venous drainage corrected with Vargas' method

A 50-day-old infant with Darling's type Ib of total anomalous pulmonary venous drainage (TAPVD) was operated on with the Vargas' method. Pulmonary veins drained separately into the right superior vena cava (SVC). A J-shaped right atriotomy was performed according to the Vargas' method. The posterior flap was sutured to the anterior border of a previously enlarged atrial septal defect, directing the pulmonary blood flow toward the left atrium. The right SVC was divided just above the site of drainage of pulmonary veins, the proximal end of the right SVC was closed, and the anastomosis between the distal end of the right SVC and the previously opened right atrial appendage was performed. However, pulmonary hypertension remained because of the restrictive orifices of pulmonary venous drainage into SVC, and then side-to-side anastomoses between pulmonary veins and left atrium had to be added. Eight months after the operation pulmonary hypertension progressed markedly, because the orifices of the anastomoses became severely stenotic. Re-operation was performed to create a large anastomosis between pulmonary veins and left atrium. The indication and the long-term prognosis of the Vargas' method were discussed.     

Partial anomalous pulmonary venous drainage to the high superior vena cava; repair by direct anastomosis between the superior vena cava and the right atrial appendage; report of a case

A 10-year-old boy with partial anomalous pulmonary venous connection to the high superior vena cava (SVC) underwent surgical repair by Williams method. The SVC was divided above the orifice of the anomalous pulmonary vein. The proximal end of the SVC was closed and the distal end of the SVC was anastomosed to the right atrial appendage (RAA). The anomalous pulmonary vein was rerouted to the left atrium via the SVC and the surgically enlarged central type atrial septal defect (ASD). The postoperative course was uneventful except transient sinus bradycardia and catheter study showed no stenosis of the SVC and the RAA.