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。     

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。     

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     

儿童永存动脉干的外科治疗

目的　总结儿童永存动脉干外科治疗经验。　方法　近９ 年来共收治永存动脉干５例,年龄３～１２ 岁,平均６－３ 岁。Ⅰ型２ 例（肺动脉均起于动脉干远侧）,Ⅱ型２ 例, Ⅳ型１ 例。肺循环时间和肺动脉排空时间均无明显延长。手术在体外循环心内直视下进行,室间隔缺损（ＶＳＤ） 补片缝合于动脉干瓣环并使其环缩。右心外管道采用缝制自体新鲜心包瓣涤纶人工血管４ 例,同种异体主动脉１ 例。　结果　术毕肺动脉压均明显下降,跨外管道压差７～３５ ｍｍ Ｈｇ。２ 例术后早期死亡与肺动脉病变无关。存活３ 例中１ 例并发低心排出量和呼吸功能不全,２ 例术后恢复顺利。分别随访１１４、９６ 和３４ 个月,心功能均为Ⅰ级,１ 例术后８ 年死于外管道梗阻。　结论　儿童永存动脉干仍可有手术指征; 带自体心包瓣人工血管远期效果良好; 纠正动脉干瓣关闭不全,防止ＶＳＤ 残余漏,避免过大的跨外管道压差和缩短手术时间,可提高手术成功率和远期效果     

双向腔肺动脉吻合术的临床应用

双向腔肺动脉吻合术近来为复杂先天性心脏病伴肺血流减少者提供了一条有效的治疗途径。尤其是作为全腔肺动脉吻合术的一个组成部分，治疗单心室或三尖瓣闭锁取得了较Ｆｏｎｔａｎ手术更为良好的效果。本组有７例患者采用了这一手术方法，其中５蜍为全腔肺动脉吻合术的一个组成部分，１例用以治疗Ｅｂｓｔｅｉｎ畸形并三尖瓣狭窄、右室发育不良，１例用于治疗法乐四联症。早期存活６例。１例因急性心泵衰竭早期死亡，１例术后２个月死     

非体外循环心外管道全腔静脉肺动脉吻合术的临床应用

2000年8月至2002年4月，我们为6例病儿在非体外循环下行心外管道全腔静脉肺动脉吻合术，取得了良好的治疗效果，现报道如下。     

法洛四联症合并一侧肺动脉缺如的手术治疗

目的报告法洛四联症合并一侧肺动脉缺如的外科治疗。方法８例法洛四联症合并一侧肺动脉缺如患者，左侧肺动脉缺如７例，右侧肺动脉缺如１例；其中２例肺动脉闭锁。６例行一期根治术，２例先行体－肺动脉分流术，再分别于术后８个月和１１个月行二期根治术。６例应用带单瓣补片跨瓣加宽右心室流出道，２例采用右心室到肺动脉带瓣管道。结果全组术后出现呼吸衰竭２例，低心排血量综合征３例；其中１例死于心力衰竭，死亡率１２．５％。７例长期存活者术后随访０．５～５．０年，平均２．５６年，无晚期死亡，心功能Ⅰ级６例，心功能Ⅱ级１例。结论法洛四联症合并一侧肺动脉缺如根治术时，必须严格掌握手术适应证，右心室流出道跨瓣补片应带单瓣     

呼吸对全腔肺动脉吻合术后肺循环血流的影响

目的探讨呼吸对全腔肺动脉吻合术后肺循环血流的影响。方法对15例全腔肺动脉吻合术后的患儿进行超声检查,观察在静息及深呼吸条件下肺循环血流的变化特征。结果应用外管道的患儿,静息状态下,肺动脉为持续性前向血流,血流速为（38.0±2.3）cm/s;深吸气时前向流速增大为（67.0±3.5）cm/s;深呼气时前向流速减小为（26.0±2.3）cm/s,与静息状态时相比,差异均有极显著性意义（P＜0.01）。应用内隧道的患儿,其肺动脉血流同时要受心动周期的影响,静息状态下,上腔静脉为持续性血流,血流速为（42.0±2.1）cm/s;深吸气时流速增大为（71.0±4.3）cm/s;深呼气时流速减小为（21.0±1.0）cm/s,与静息状态时相比,差异均有极显著性意义（P＜0.01）,且无反向血流。应用人工血管外管道的患儿,吸气时,下腔静脉为峰值（37.0±2.3）cm/s的前向血流,呼气时为峰值（33.0±1.7）cm/s的反向血流;应用带瓣的同种血管外管道的患儿,吸气时下腔静脉为峰值（47.0±1.9）cm/s的前向血流,呼气时无前向流,且无明显反向血流。结论呼吸是全腔肺动脉吻合术后肺循环的另一能量来源;带瓣外管道可以减少或完全防止下腔静脉呼气时的反向血流。     

一种新型全腔静脉肺动脉吻合术动物模型的建立

目的 建立一种与临床术式相近的全腔静脉肺动脉吻合术实验动物模型。方法 选用成年杂交犬10条。根据下腔静脉吻合口的位置不同分为两组：1组为下腔静脉与右肺动脉吻合;2组为下腔静脉与主肺动脉吻合。两组动物模型中的上腔静脉全部与右肺动脉吻合。全腔静脉肺动脉吻合术模型在非体外循环下通过心外管道建立。结果 两组动物术后30min、1h及2h血液循环指标稳定,两组犬血流动力学指标差异无显著性（P＞0．05）。上腔静脉压维持在12mmHg(1mmHg=0.133kPa) 以上时才能维持 正常的血液循环。全腔静脉肺动脉吻合术后,肺血管阻力随静脉压的升高而下降。所有动物在手术结束后都能进行2h以上的实验数据监测 和观察。结论 犬心外管道全腔静脉肺动脉吻合术动物模型与临床实际术式相接近,是一种可靠的急性动物实验模型;全腔静脉肺动脉吻合术后,静脉压维持到合适水平是维护循环稳定的重要特征。     

全结肠切除手术9例报告

９６９年～１９９４年，我院收治溃疡性结肠炎患者３例，Ｇａｒｄｎｅｒ综合征１例及家族性息肉病５例，其中男８例，女１例，年龄１８～５３岁，平均３８岁。术前均经钡灌肠及纤维结肠镜检查并经病理检查确诊。９例中６例行全结肠切除回肠直肠吻合术，３例行全结肠直肠切...     

Valved homograft conduit repair of the right heart in early infancy.

BACKGROUND: Valved homograft conduit repair in neonates and young infants creates a physiologically normal biventricular circulation, and unlike shunts, avoids surgery on the branch pulmonary. METHODS: Retrospective chart review was used for 84 patients operated on between 1990 and 1995 (mean age 26+/-28 days, mean weight 3.3+/-0.8 kg) undergoing homograft conduit repair in the first 3 months of life. Cases were divided into simple and complex, eg, absent pulmonary valve syndrome or associated interrupted arch. Mean homograft size was 9.0+/-2 mm. RESULTS: Early mortality was 4.7% (simple) and 30% (complex). Mean hospital stay was 18 days. Mean follow-up was 34 months. Thirty-seven (47%) patients underwent conduit replacement. Median time to reoperation was 3.1 years. Mean size of replacement homograft was 17+/-2 mm. There were no deaths at reoperation. Mean hospital stay at conduit change was 6.3 days. Probability of survival at 5 years is 75%. CONCLUSIONS: Biventricular repair employing a conduit can be performed safely in noncomplex anomalies in the first 3 months of life. Time interval until repeat surgery is relatively short but equal or greater than that with most palliative procedures.     

Right ventricular outflow tract reconstruction with the Contegra bovine jugular vein conduit: a word of caution

BACKGROUND: Since introduction in 1999, pulmonary valve replacement in pediatric patients with the Contegra conduit (Medtronic Inc, Minneapolis, MN) has gained widespread application with increasing enthusiasm. However, unexpected graft related adverse effects may occur. METHODS: Between April 2001 and December 2002, 29 patients (20 male; mean age, 3.39 +/- 3.66 years; range, 0.01 to 13.0 years; mean weight, 11.62 +/- 8.73 kg) underwent right ventricular outflow tract reconstruction with the Contegra conduit. Seventeen patients underwent primary repair, 8 had prior homografts, and 4 had other previous operations. RESULTS: There were no deaths. Three early graft related complications were observed. In two infants (age, 1.8 and 3.5 months; weight, 3.6 and 3.8 kg, respectively) thrombus formation at the conduit valve was detected 2 weeks postoperatively. Under anticoagulation with low-molecular-weight heparin, thrombi resolved completely in both patients. One patient (4.5 months, 4.43 kg) developed severe regurgitation due to a fibrous layer covering the inner conduit wall and required conduit exchange 3 weeks postoperatively. After a mean follow-up time of 10.2 +/- 6.4 months all patients are in good clinical condition. However, one patient with systemic right ventricular pressure developed pseudoaneurysm at both graft insertion sites and is scheduled for reoperation. Two other patients underwent balloon dilation. Freedom from reoperation and intervention at 1 year is 89.4%. With regard to regurgitation and conduit stenosis all other conduits perform well. CONCLUSIONS: Contegra conduits are an alternative to homografts for right ventricular outflow tract reconstruction. However, there is a risk of thrombus formation in small infants so that prophylactic anticoagulation may be necessary. Patients with systemic right ventricular pressure require close observation as pseudoaneurysm formation has been observed.     

Bidirectional Glenn procedure without cardiopulmonary bypass

BACKGROUND: Whether the bidirectional Glenn procedure is better performed without the support of cardiopulmonary bypass is still a matter for debate. In this paper we discuss the indications and methods for bidirectional Glenn shunt without cardiopulmonary bypass. METHODS: Twenty patients with complex cyanotic congenital heart defects underwent a bidirectional Glenn shunt without cardiopulmonary bypass between May 2000 and August 2002. There were 10 male and 10 female patients, the mean age was 2.7 +/- 2.6 years (range, 3 months to 11 years), and the mean weight was 11.0 +/- 6.0 kg (range, 4.5 to 32 kg). The mean transcutaneous oxygen saturation was 74.3% +/- 5.7% before the operation. The Glenn shunt was performed under venoatrial or venopulmonary shunt. RESULTS: All patients survived. Mean superior vena cava clamping time was 24.3 +/- 4.7 minutes, and mean vena cava pressure was 26.9 +/- 5.5 mm Hg during clamping. There were no postoperative neurologic complications. Follow-up echocardiography showed functioning Glenn shunts without any obstruction at the anastomosis. CONCLUSIONS: The adverse effects of cardiopulmonary bypass could be eliminated by this method. This is an advantage during the postoperative recovery, but patients should be strictly chosen.     

Repair of transposition of the great arteries, ventricular septal defect and left ventricular outflow tract obstruction.

OBJECTIVES: This study was undertaken to compare the outcomes of the Lecompte procedure and Rastelli repair in the transposition of the great arteries (TGA) with ventricular septal defect (VSD) and left ventricular outflow tract obstruction (LVOTO) and to determine the risk factors associated with unfavorable events. METHODS: Over a 12-year period (April 1990-October 2002), 35 patients underwent complete repair for TGA, VSD, and LVOTO. Twenty-five patients (71%) underwent the Lecompte modification, and mean age and weight were 23.4+/-18.2 months and 10.2+/-3.0 kg. Ten patients (29%) underwent the Rastelli operation, and mean age and weight were 39.1+/-36.1 months and 13.8+/-6.8 kg. RESULTS: One early death (3%) occurred after the Lecompte procedure and no late death. The mean follow-up was 5.9+/-3.8 years. Eight patients in the Rastelli group (80%) underwent a late reoperation for obstruction of the extracardiac conduit, and in four of these patients, a reoperation for LVOTO was concomitantly required. Reoperation was also required in six patients of the Lecompte group (25%); five for right ventricular outflow tract obstruction (RVOTO) including one for LVOTO and two for VSD leakage, and one for mitral regurgitation and left pulmonary artery stenosis. The interval prior to reoperation ranged from 1.6 to 11.1 years, with a mean of 5.7+/-3.1 years. The actuarial figures for freedom from reoperation at 5 and 10 years were 40.0+/-15.5 and 26.7+/-15.0% after the Rastelli operation and 95.7+/-4.3 and 63.5+/-12.6% after the Lecompte procedure (P = 0.02). Multivariate analysis by Cox regression analysis revealed that the risk factors of RVOTO were a younger age at operation, the Rastelli operation, and ductus ligation during the operation. CONCLUSIONS: The Lecompte procedure and Rastelli repair provide satisfactory early and late results. However, substantial late morbidity is more associated with conduit obstruction, and LVOTO in Rastelli repair rather than Lecompte procedure.     

Apico-pulmonary artery conduit repair of congenitally corrected transposition of the great arteries with ventricular septal defect and pulmonary outflow tract obstruction: a 10-year follow-up

BACKGROUND: In conventional repair of the congenitally corrected transpositions of the great arteries associated with ventricular septal defect and pulmonary outflow tract obstruction, the placement of the left ventricle-pulmonary artery conduit is at risk owing to probable compression by the sternum, heart block, or injury to the mitral anterior papillary muscle. Apical placement of the left ventriculotomy for the inflow conduit rather than in the midportion or base placement may avoid these complications, although this results in a long and winding extracardiac conduit that may be short-lived because of the proliferation of pseudointima. METHODS: Between 1985 and 1990, a nonvalved Dacron woven-fabric graft conduit was placed between the left ventricular apex and pulmonary artery in 5 patients (mean age, 6.2 +/- 1.7 years) who were then followed for at least 10 years. RESULTS: No iatrogenic heart blocks or mitral regurgitation developed. All patients were complaint-free during the follow-up period, although 1 patient who was clinically well died suddenly in the 10th follow-up year. Cardiac catheterization in the 10th follow-up year indicated a pressure gradient of 21 +/- 6 mm Hg across the conduit, and angiography revealed that the conduit diameter was 91% +/- 6% of the original conduit diameter. CONCLUSIONS: The reportedly poor early and late outcomes that occur after a conventional repair of congenitally corrected transpositions of the great arteries associated with ventricular septal defect and pulmonary outflow tract obstruction, which places an extracardiac conduit between the left ventricle and the pulmonary artery, may be partially neutralized by relocating the inflow position to the apex.     

Conversion from colonic conduit into recto-sigmoid pouch (Mainz pouch II)

OBJECTIVE: To report our long-term results of conversion from conduit conversion into a continent anal urinary diversion, as after conduit urinary diversion in childhood, some patients wish to have a later conversion to a continent diversion to avoid external appliances and to improve their quality of life. PATIENTS AND METHODS: Between 1992 and 2003, 139 patients had a urinary diversion with a recto-sigmoid pouch (Mainz pouch II), of whom four had a conversion from a colonic conduit diversion to a recto-sigmoid pouch. The mean (range) age at conduit diversion was 5.5 (3-14) years and the mean interval between conduit diversion and conversion to a continent anal diversion was 8 (4-18) years. The mean age at conversion into a Mainz pouch II was 13 (8-32) years and the follow-up afterward was 11.5 (1-13) years. The conversion was done by incorporating the pre-existing colonic conduit into the recto-sigmoid pouch with no ureteric reimplantation. RESULTS: There were no early complications; one nephrectomy was required 5 years after conversion because of uretero-intestinal obstruction and pyelonephritis. All other reno-ureteric units remained stable and renal function was maintained. All patients are continent day and night; three require substitution with alkali at a base excess of < -2.5 mmol/L to prevent hyperchloraemia and acidosis. CONCLUSION: The recto-sigmoid pouch is a therapeutic option when patients desire conversion from an incontinent type of urinary diversion to a continent type.     

The extracardiac Fontan procedure without cardiopulmonary bypass: technique and intermediate-term results

BACKGROUND: The extracardiac Fontan procedure (ECF) usually requires cardiopulmonary bypass (CPB). In this report, the results and techniques of this procedure without CPB at a single institution are presented. METHODS: Between August 1992 and December 2001, ECF without CPB was achieved in 24 of 44 patients undergoing an ECF. Mean age at surgery was 5.9 +/- 2.9 years, and mean weight was 20.7 +/- 12.6 kg. Diagnoses were tricuspid atresia in 9 patients, single-ventricle with pulmonary outflow tract obstruction in 7, pulmonary atresia/intact septum in 5, and other complex single-ventricle physiology in 3. Initial palliation was by arterial to pulmonary artery shunt in 21 and pulmonary artery banding in 1. A bidirectional cavopulmonary connection was created in 23 patients. A temporary inferior vena caval-to-atrial shunt was used to complete the procedure without CPB. Median graft size was 16 mm (range 14 to 20 mm). RESULTS: There was no early mortality, and 68% of patients were discharged without complications. Complications included persistent cyanosis in 4 patients, persistent pleural effusions in 2 (one chylous), and phrenic nerve injury in 1. Median postoperative hospital stay was 16 days (range 10 to 50) days. At a mean follow-up of 44 +/- 28 months, there was no conduit obstruction. One patient died 11 months postoperatively, and 1 patient received a heart transplant 26 months post-ECF. CONCLUSIONS: At intermediate term follow-up, the ECF without CPB appears to be safe and technically reproducible in selected cases. Ongoing follow-up of these patients is necessary to document the theoretical advantages of avoiding CPB.     

Long-Term Results After Extracardiac Valved Conduits Implanted for Complex Congenital Heart Disease

Between August 1982 and December 1986, 56 patients survived implantation of an extracardiac valved conduit for complex congenital heart disease. The mean age at operation was 4.2 years (16 days to 24 yrs) and the mean weight was 15.9 kg (2.4 to 93.0 kg). The diagnosis was pulmonary atresia (PA) with ventricular septal defect (VSD) in 13 patients, tetralogy of Fallot in 11, transposition of the great arteries (TGA) with VSD in 8, truncus arteriosus, in 7, complex left ventricular outflow tract obstruction (LVOTO) in 6, complex left atrioventricular valve obstruction in 4, double outlet right ventricle with VSD and subaortic obstruction in 3, univentricular heart with pulmonary stenosis in 2, TGA with LVOTO in 1, and PA with intact ventricular septum in 1. In 35 patients, a preclotted conventional Dacron conduit (CDC) with bioprosthetic valve was used, in 19 patients a collagen-sealed Tascon valved conduit (TC) was implanted, and in 1 patient an aortic homograft was used. In a mean follow-up of 32.5 months (9 to 64 mo), there were two deaths (2/56, 3.6%) that were not related to the conduit. All survivors have been evaluated by two-dimensional and Doppler echocardiography, and 29/56 (51.8%) underwent cardiac catheterization. Nine patients (9/56, 16.1%) underwent successful valved conduit replacement, in seven cases with a nonvalved conduit. There was a significant difference (P = .011) with regard to the incidence of conduit replacement between the group with CDC (2/36, 5.5%) and the group with TC (7/19, 36.8%). Five patients underwent percutaneous transluminal balloon dilatation of the prosthetic conduit, with adequate relief of the gradient in four patients.(ABSTRACT TRUNCATED AT 250 WORDS)     

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。     

Resternotomy in patients with valved conduits adherent to the sternum

Twenty-two patients with valved conduits adherent to the sternum underwent resternotomy. Mean age was 10 +/- 6 years, and mean conduit age was 4 +/- 4 years. Diagnoses were D-transposition (7), truncus arteriosus (7), univentricular heart (6), Taussig-Bing anomaly (1), and corrected transposition (1). The majority of patients (68%) had reoperation for outgrown or degenerated conduits. In 17 patients, the sternum was opened with a chisel. Two of these patients sustained conduit neointimal collapse from manipulation, and 3 had conduit tear requiring immediate cardiopulmonary bypass through the femoral vessels. In the last 5 patients, the sternum was opened above and below the conduit, and the inner table was chiseled and left attached to the conduit avoiding injury and undue conduit manipulation. Cardiopulmonary bypass and operation were carried out uneventfully. We believe that the recent technique described provides a safe alternative approach to valved conduits adherent to the sternum.