Experience with the extracardiac conduit.

A review is made of all Mayo Clinic cases wherein 468 patients have received 516 extracardiac conduits in the repair of congenital heart defects. All patients had complex defects, which are classified in 10 basic diagnostic categories. The early mortality rate (which ranged from 4% to 49%, according to diagnostic group) averaged 25%, improving with experience. The postoperative complication rate was 70%. In 1% of operations, compression of the conduit was encountered at chest closure. The conduits placed in 333 patients contained a porcine valve. The conduit diameter averaged 22 mm. At completion of the operation, the mean gradient across the conduit was 22.8 mm Hg. At late study this mean gradient remained at 26 mm Hg in patients who had received the currently employed porcine-valved conduit. The average ratio of intraoperative postrepair ventricular (RV/LV) systolic pressures was 0.68. It was lower among the group who were to survive but was not ov prognostic value in individual cases. Among patients who survived the postoperative period, the late mortality rate (based on a mean of 3.6 years' follow-up) averaged 3.5% per year. Of the long-term survivors, 18% have required reoperation, primarily for replacement of calcified aortic homograft conduits used in the earlier part of the experience. Only 0.8% of porcine-valved conduits have required reoperation. Unrestricted life-styles were possible for 90% of survivors.     

体外循环心脏不停跳下心外管道Fontan手术

目的评估体外循环心脏不停跳下心外管道Fontan手术的方法及其效果。方法本组42例中,男性31例、女性11例,年龄3～19岁,其中心室双人口19例,三尖瓣闭锁10例,二尖瓣闭锁3例,其他复杂先天性心脏病10例。均在常温体外循环心脏不停跳下施行心外管道Fontan手术,其中8例加行开窗术;1例先做了双向腔肺动脉分流术,术后2年行心外管道Fontan手术。结果术后早期和晚期各死亡1例,分别死于急性肝功能衰竭和反复肺部感染,随访1．0～4．5年,40例心功能为Ⅰ级和Ⅱ级,动脉血氧饱合度92％～96％。结论体外循环心脏不停跳下心外管道Fontan手术的近、中期效果满意．可在单一心室修复中选用。     

Method to make the tricuspid extracardiac conduit by heterogeneous pericardium]

We described here, how to make tricuspid extracardiac conduit by heterogeneous pericardium for Rastelli procedure. We have developed some ingenious devices which allow to obtain good hemodynamics. One of the devices is large valvular leaflets as long as 130% of the circumference of the conduit. Another device is the commissural suture as figure of eight. We used 121 tricuspid extracardiac conduits between January 1985 and March 1991. There were two reoperations: One from stenosis at the suture with ventricle and the other from infective endocarditis. This hand-made conduit has the advantages of flexibility, fitness with the pulmonary artery, wide range of size and very little regurgitation. These advantages indicate that the tricuspid extracardiac conduit made by heterogeneous pericardium is a valved conduit substitute of choice for Rastelli procedure. The durability of the conduit is to be further evaluated.     

Total cavopulmonary connection with an extracardiac conduit: experience with 100 patients

BACKGROUND: In the Fontan procedures total cavopulmonary connection with an extracardiac conduit is a concern. The potential benefits of an extracardiac conduit may be the avoidance of postoperative supraventricular arrhythmias over the long-term, hemodynamic benefits due to laminar flow, possibility of completion without anoxic arrest, and applicability to anomalous systemic or pulmonary venous return, or both anomalous systemic and pulmonary venous return. We demonstrate early to midterm results of total cavopulmonary connection with an extracardiac conduit. METHODS: Between March 1994 and February 2000, a total of 100 patients underwent total cavopulmonary connection with an extracardiac conduit. In 27 patients, who underwent a single stage total cavopulmonary connection operation, 7 were done without palliation. Seventy-three patients had undergone a bidirectional Glenn shunt before completion of the total cavopulmonary connection. We used an expanded polytetrafluoroethylene tube graft as the extracardiac conduit. RESULTS: Cardiopulmonary bypass time was 133.2+/-55.2 minutes. Myocardial ischemic time was 38.5+/-23.2 minutes in 40 patients who needed cardioplegic cardiac arrest for intracardiac procedures. Intraoperative fenestration was done in only 1 patient. There were no operative deaths. During follow-up of 37.3 months, there were 5 late deaths. When compared with the patients treated by the lateral tunnel technique in our institute, there was no significant difference in actuarial survival rate, but the event free rate of the extracardiac conduit group was significantly superior to the lateral tunnel group. CONCLUSIONS: Total cavopulmonary connection with the extracardiac conduit produced good results in short to midterm follow-up.     

Pathologic finding of restenosis in stent-implemented right ventricle-pulmonary artery extracardiac conduit.

We describe an excised specimen of a stent-implanted valved equine pericardial extracardiac conduit in the right heart. It appears from careful pathologic examination that the stent acted as a nidus for thrombus formation followed by thick neo-intimal development over the stent, which caused restenosis. Restenosis occurred despite anticoagulation.     

Venous cannulation for extracorporeal circulation after total cavopulmonary connection with extracardiac conduit

We report two cases of venous cannulation after a total cavopulmonary connection (TCPC) with extracardiac conduit. Venous cannulation was performed via side graft sutured to the extracardiac conduit in an end-to-side fashion. The first case was a 3-year-old girl, who suffered from an atypical inferior vena cava obstruction after TCPC. The obstruction region was distal to the anastomosis site of extracardiac conduit and inferior vena cava. She underwent a surgical release of obstruction under cardiopulmonary bypass without circulatory arrest. The second case was a 2-year-old girl, who needed an extracorporeal membrane oxygenation support after TCPC due to severe low cardiac output syndrome. She was decannulated successfully after thirty-nine-hour support.     

改良外管道Fontan术治疗心脾综合征型复杂先天性心脏病

目的　回顾总结改良外管道Fontan术治疗无脾或多脾综合征伴发的复杂型先天性心脏病(先心病)经验。方法　青紫型复杂先心病病儿11例,无脾9例(右房异构,单心室合并共同房室瓣6例,房室连接不一致的右室双出口2例,纠正性大动脉错位1例) ;多脾2例(左房异构,右室双出口合并共同房室瓣)。平均年龄(6 3±3 7)岁,平均体重(2 1 0±5 5 )kg。直接行改良外管道Fontan术3例,双向Glenn术后行二期外管道Fontan术8例。结果　术后早期发生低心排出量综合征2例,肾衰1例,室上性心动过速1例,胸腔引流(12 0ml d) >10d 2例。超声提示上腔吻合口通畅(流速0 6～0 8m s) ,下腔血流速度0 3～0 5m s ;动脉血氧饱和度9例在0 92～0 95 ,2例为0 86 ;活动能力明显改善,无早期死亡。随访中超声显示上腔静脉血流速度0 8m s,下腔静脉血流速度0 4～0 7m s ,随呼吸而变化。外管道通畅,无血栓形成,管道窗口直径0 34cm ,血流右向左分流。肺静脉回流无梗阻,心室射血分数>0 6 0。心电图未见明显心律失常。生长发育正常,活动自如,经皮氧饱和度>0 90 ,无慢性渗出、蛋白丢失肠病等并发症,无中期死亡。结论　改良外管道Fontan术适用于治疗无脾或多脾综合征型复杂先心病,术后心律失常发生率低。     

心外管道全腔静脉-肺动脉连接术治疗复杂先天性心脏病

目的总结心外管道全腔静脉-肺动脉连接术（ECTCPC）治疗复杂先天性心脏病的临床经验,并就手术适应证、手术方法及手术效果进行讨论。方法1998年6月至2006年12月,68例先天性心脏复杂畸形的患者接受了ECTCPC。包括单心室伴有大动脉转位、肺动脉瓣狭窄45例：三尖瓣闭锁、右心室发育不良19例;三尖瓣下移畸形并右心室发育不良4例。其中合并永存左上腔静脉6例,双向Glenn术后行全腔静脉-肺动脉连接术18例（其中包括单心室、肺动脉闭锁、左肺动脉狭窄双向Glenn术后1例）,单心房、单心室、心上型完全性肺静脉异位引流、多发粗大体肺侧支1例。全组采用体外循环下手术共57例,其中8例患者因需要矫正心内畸形在主动脉阻断下手术外,其余49例均在全身麻醉并行体外循环心脏跳动下进行;非体外循环下手术11例。结果术后早期死亡2例,病死率为2．9％。其中1例死于术后反复肺内出血,1例死于上消化道反复大出血。66例痊愈出院,术后随访1个月至8年,无晚期死亡。所有患者症状消失,血氧饱和度90％～96％,恢复良好。结论ECTCPC方法简便易行,术后并发症较少,效果好,较其他术式有较大优点。     

Inferior vena cava-pulmonary artery extracardiac conduit. A new form of right heart bypass

From November 1988 to May 1989, four patients underwent total right heart bypass by means of bidirectional cavopulmonary anastomosis and interposition of an extracardiac conduit from the inferior vena cava to the pulmonary artery. All of them had an uneventful postoperative course, and there have been no early or late deaths. We propose this technique as an alternative surgical option in candidates for a Fontan procedure with (1) hypoplasia or atresia of the left atrioventricular valve, (2) common atrioventricular valve, (3) anomalies of systemic and pulmonary venous return, or (4) auricular juxtaposition.     

Total cavopulmonary connection: open anastomosis of an extracardiac conduit with vacuum-assisted venous drainage

Insertion of a tube conduit for total cavopulmonary connection is sometimes technically demanding due to the crumpled stump of the inferior vena cava caused by a tourniquet of the inferior vena cava near the division line. Herein we describe an alternative in which the anastomosis is completed during removal of the tourniquet with the application of vacuum-assisted venous drainage. This new technique may alleviate, if not completely eliminate, a concern associated with total cavopulmonary connection with extracardiac conduit in small patients.     

Autologous pericardial pulmonary conduit with single point attached commissures in a sheep model.

OBJECTIVE: For the surgical treatment of congenital heart disease and in Ross procedure a valved conduit is frequently required. Since homografts are not readily available in every country, a reliable alternative is needed. We developed a novel technique to construct a valved pulmonary conduit with single point attached commissures (SPAC) in a simple and fast way from a small strip of autologous pericardium, molded and briefly treated with glutaraldehyde. METHODS: Autologous pericardial pulmonary conduit was constructed intraoperatively and implanted in pulmonary position in a beating heart in six sheep. The prosthesis size was 31 mm for all sheep and the construction time (including 10 min glutaraldehyde treatment) was 19.0+/-3.3 min. Implantation time and cardiopulmonary by-pass was 27.3+/-5.4 min and 40.5+/-7.7 min, respectively. The sheep were euthanized after 6 months (222.7+/-5.8 days) postoperatively. RESULTS: In all sheep, the autologous pericardial valve was immediately competent. At sacrifice, the pericardial valve was pliable and competent in all cases with SPAC well anchored to the pericardial conduit wall. The maximum transvalvular gradient at implant and at sacrifice was 3.3+/-2.8 mmHg and 3.3+/-2.0 mmHg, respectively. CONCLUSIONS: This novel autologous pericardial pulmonary conduit with SPAC can be reliably produced in a very short time intraoperatively before cardiopulmonary by-pass. The simplicity of construction, biocompatibility and freedom of stenosis or thrombosis makes this autologous pulmonary conduit especially useful for patients at locations where homografts are not readily available.     

Immunohistological findings for an extracardiac conduit in Fontan pathway constructed with pedicled autologous pericardium

Although autologous pericardium has been used in pedicled fashion for various reconstructive procedures in congenital cardiovascular surgery with the expectation that it will be able to grow and remain viable after implantation, no clinical study has evaluated the histological characteristics of implanted pedicled pericardium long after previous implantation. We describe herein histological findings of pedicled pericardium, which had been used for three years as an extracardiac conduit in Fontan pathway. A four-year-old boy with a history of Fontan operation using pedicled autologous pericardial roll required conduit replacement three years after the previous operation, and resected pericardial tissue was examined immunohistologically. Staining for CD34 revealed abundant microvasculature, suggesting preservation of viability. Staining for factor VIII demonstrated the presence of endothelium on the luminal surface of the conduit. Elastica-van Gieson staining revealed a band of elastic tissue, which is generally found not in the native pericardium but in the vascular wall structures. These findings suggested that the pedicled pericardium thus appeared to have remained viable and might have differentiated to resemble tissue of the vascular wall after having been used in Fontan pathway.     

Total cavopulmonary connection with innovative combined lateral tunnel and extracardiac conduit in heterotaxy syndrome

Total cavopulmonary connection remains a challenging procedure when treating heterotaxy syndrome patients with a widely separate drainage of the inferior vena cava and the hepatic vein into the common atrium. We trimmed a Gore-Tex tube graft (W.L. Gore & Associates, Flagstaff, AZ) to form a skirt to cover both openings of the inferior vena cava and the hepatic vein. This tubular structure pierces the atrium and travels outside the heart, and then reaches the inferior side of the pulmonary artery to avoid pulmonary vein obstruction. We then sutured the pierced atriotomy margin to the conduit. This innovative procedure has been shown to accommodate the widely separated hepatic vein drainage with a promising outcome.     

An alternative method to fenestrate the extracardiac fontan conduit in a patient with atrial situs inversus and levocardia

This report describes the technique to fenestrate the extracardiac Fontan conduit without cardiopulmonary bypass in a patient with levocardia and atrial situs inversus.     

A case report of replacement of the extracardiac conduit for tetralogy of Fallot: reconstruction with autogenous tissue around the conduit]

Replacement of the valved extracardiac conduit with autogenous tissue was performed on a 13-year-old female. The procedure was based on that reported by Danielson in 1987. She underwent the first operation for tetralogy of Fallot with pulmonary atresia using a valved extracardiac conduit (#16 mm Bj?rk-Shiley valve) 8 years ago. Recently, she complained exertional dyspnea and chest pain. Reoperation was scheduled because of bleeding tendency, somatic growth of the patient, and severe distal anastomotic stenosis of the conduit by right ventriculogram. At operation, the valved conduit was removed under cardiopulmonary bypass, and autogenous connective tissue around the conduit was preserved as the posterior wall of the new conduit. An onlay patch (pericardial monocusp patch) was sutured to form the roof of the new tract. Postoperative catheterization showed no residual stenosis or pressure gradient between the right ventricle and the pulmonary artery. This technique is useful as the replacement of extracardiac conduit because of its simplicity, no necessity of postoperative anti-coagulant therapy, and possibility to make a generous-sized new outflow tract.     

心外管道全腔静脉肺动脉吻合术治疗复杂性先天性心脏病

目的 评价心外管道全腔静脉肺动脉吻合术(TCPA)治疗复杂先天性心脏病的临床应用价值。方法 1998年6月～2002年7月，26例先天性心脏病复杂畸形的患者接受了心外管道TCPA，包括单心室伴完全型大动脉转位16例，三尖瓣下移畸形2例，右心室双出口伴大动脉转位3例，三尖瓣闭锁伴右心室发育不良5例。19例在全身麻醉低温体外循环下手术，7例在非体外循环下手术。结果 无手术死亡，全部患者治愈出院。术后随访1～47个月，无晚期死亡。所有患者症状消失，无静脉压明显升高现象，超声心动图检查示心外管道血流通畅，无血栓形成，心电图检查示无严重的心律失常，血氧饱和度0．93～0．96，心功能均达Ⅰ～Ⅱ级。结论 心外管道TCPA是一种较为简单的手术方式，易于掌握；术后疗效满意，优于其他术式。     

非体外循环与体外循环心外管道全腔静脉-肺动脉连接术的对比研究

目的比较常温非体外循环(OPCPB)和低温体外循环(CPB)下心外管道全腔静脉-肺动脉连接术(TCPC)治疗功能性单心室复杂畸形的效果.方法根据术中是否采用CPB,将28例功能性单心室复杂心脏畸形患者分为两组,OPCPB组:11例,均在OPCPB下行心外管道TCPC;CPB组:17例,均在CPB下行心外管道TCPC.记录两组患者中心静脉压(CVP)、平均体动脉压、胸腔引流液量、机械辅助通气时间、术后住院时间、血液制品用量、住院费用和出院时脉搏血氧饱和度(SpO2),并进行比较.结果两组术后早期死亡率无明显差别,术后OPCPB组CVP明显低于CPB组(P<0.05),胸腔引流液量、机械辅助通气时间、住ICU时间和术后住院时间较CPB组明显减少或缩短(P<0.05),血浆用量明显减少(P<0.05),住院费用明显低于CPB组(P<0.05),两组SpO2差别无显著性意义(P>0.05).结论在OPCPB下行心外管道TCPC并非十分困难,术后血流动力学明显优于CPB下行心外管道TCPC,围术期血液制品用量减少,有利于早期恢复;术后住院时间短,住院费用明显降低.