Ross-Konno手术治疗儿童主动脉瓣及瓣下狭窄的临床疗效

目的评价Ross-Konno手术治疗儿童主动脉瓣及瓣下狭窄的早期临床疗效。方法 2018年12月至2019年12月,共12例患儿因主动脉瓣及瓣下狭窄在我科接受手术治疗,其中男8例、女4例,接受手术时中位年龄4岁(11个月~12岁)。Ross-Konno手术11例,单纯Ross手术1例。主动脉瓣重度狭窄8例,主动脉瓣关闭不全2例,狭窄合并关闭不全2例。合并主动脉二瓣化畸形6例,瓣下流出道显著狭窄4例,合并二尖瓣病变4例,合并弓部病变3例。手术采用自体肺动脉带瓣管道移植于主动脉瓣位,同期采用Konno法扩大左室流出道。采用牛颈静脉或带瓣Gore-tex人工血管重建右室流出道,并同期矫治心内合并畸形。所有患者均经多普勒彩色超声心动图定期进行随访评估。结果全组无手术死亡。术后中位随访时间5.5(1~12)个月,1例患儿残余左室流出道中度狭窄,其余患儿无显著左室流出道残余梗阻或复发梗阻,无新主动脉瓣显著反流。1例患儿肺动脉带瓣管道主干中度狭窄,余重建右室流出道血流通畅,无明显瓣膜反流及赘生物形成。结论对于不适合瓣膜成形或置换术的儿童主动脉瓣膜病变,Ross-Konno手术早期结果满意。     

肺动脉瓣缺如综合征的外科治疗

目的总结肺动脉瓣缺如综合征的外科治疗经验。方法1985年1月至2003年9月，纠治肺动脉瓣缺如综合征18例中男13例，女5例。伴法洛四联症17例，伴室间隔缺损1例。全组均在中低温体外循环下行纠治手术，即切除、折叠整形扩张的肺动脉；解除右心室流出道梗阻同时关闭室间隔缺损。7例肺动脉瓣处置单瓣。结果术后死亡1例，死亡率5．6％。术后并发低心排血量综合征3例，Ⅲ度房室传导阻滞和心包积液各1例，经治疗均痊愈。术后随访0．5—5．0年，轻度残余右室流出道梗阻1例、轻度肺动脉瓣反流7例、中度肺动脉瓣反流3例、重度肺动脉瓣反流伴充血性右心衰1例。结论婴幼儿型肺动脉瓣缺如需立即手术，在解除右室流出道梗阻和肺动脉做切除、折叠整形时，须在肺动脉瓣区置入单瓣或带瓣管道。儿童型肺动脉瓣缺如选择性根治，处理原则同普通伴发畸形。     

Nikaidoh术治疗完全型大动脉错位伴室间隔缺损和肺动脉狭窄的早期结果

目的回顾性研究Nikaidoh术治疗完全型大动脉错位伴室间隔缺损和肺动脉狭窄(TGA/VSD/PS)的早期结果。方法在2004年1月至2005年12月期间,有8例TGA/VSD/PS患者在我院接受Nikaidoh术矫治,手术年龄4～29个月(11.4±7.6个月),体重5.2～11.0kg(8.0±1.9kg);所有患者房室连接一致,其中伴房室瓣骑跨1例,左肺动脉狭窄1例,本组患者术前均未行其他手术,手术均采用改良Nikaidoh术,即主动脉根部移位和重建左、右心室流出道,除1例冠状动脉同时移植和使用Homograft重建右心室流出道外,用自体心包补片扩大重建右心室流出道。结果手术死亡1例,无1例出现明显左室流出道梗阻(LVOTO)和右室流出道梗阻(RVOTO),轻度肺动脉反流3例,中度4例,除1例死亡患者外,其余左心功能均正常;随访时间平均8.8个月(3～18个月),7例存活;心功能状况佳,射血分数(EF)0.64±0.02;短轴缩短率(FS)0.33±0.02。未出现进展性主动脉瓣反流、LVOTO、RVOTO和肺动脉反流加重。结论Nikaidoh术适宜于治疗TGA/VSD/PS,尤其当解剖上存在不宜行Rastelli术的患者,早期结果良好。     

同种带瓣主动脉和肺动脉移植在复杂先心病治疗中的应用

目的　研究同种带瓣主、肺动脉移植应用于复杂性先天性心脏病矫治的疗效。方法　制备同种带瓣管道 89根 (主动脉 5 5根、肺动脉 3 4根 ) ,临床应用 3 1例 ,其中主、肺动脉单瓣作右室流出道跨瓣补片术 14例 ,Rastelli手术 11例 ,右心室 -肺动脉连接 (RV -PA) 3例 ,肺动脉瓣置换1例 ,Ross手术 1例 ,改良Fantan手术 1例。结果　主、肺动脉单瓣作右室流出道跨瓣补片组 14例 ,1例术后 3d死于右心功能不全 ,存活 13例 ,随访 ( 4 3± 3 .2 )个月 ,超声心动图检查显示移植后瓣膜启闭功能良好 ,无增厚及粘连。复杂性先天心脏病矫治组 17例 ,死亡 7例 ,存活 10例 ,随访( 3 4.3± 6.3 9)个月 ,管道血流通过顺畅 ,管壁无变薄、钙化。结论　①同种带瓣管道具有管壁弹性好 ,易于缝合 ,术后排斥反应少等优点 ,可广泛应用于复杂性先天性心脏病矫治术 ,左、右室流出道的重建 ;②同种带瓣管道补片既能加宽右室流出道 ,且有良好抗返流作用 ,有利于术后患儿心功能恢复 ,使部分复杂的病例能够一次手术根治     

Nikaidoh手术的临床应用

目的 总结Nikaidoh手术治疗右心室双出口(DORV)和大动脉转位(TGA)患者的临床经验,以提高手术疗效. 方法 3例先天性心脏病患者中DORV 1例,TGA 2例(平均年龄11.6岁),均行Nikaidoh手术,术中行Lecompte操作,Gore-tex人工血管补片修补室间隔缺损并重建左心室流出道,18＃带单瓣牛心包片补片重建肺动脉及右室流出道. 结果 术后无早期死亡,平均住院时间13.6d.发生低心排血量综合征1例,肝功能损害2例,二次开胸止血1例,均经相应的处理治愈.随访3例,随访时间3～5个月,复查超声心动图未发现左、右心室流出道梗阻;其中2例发现轻度至中度主动脉瓣关闭不全,但定期随访未发现继续加重. 结论 采用Nikaidoh手术治疗DORV和TGA患者,术后可获得良好的血流动力学效果,早期临床结果满意.     

小儿永存动脉干右室流出道重建

目的 总结小儿永存动脉干(PTA)右室流出道重建治疗经验.方法 2000年1月至2007年12月共行PTA根治手术治疗43例,男26例,女17例.年龄1.5个月～3.8岁;体重3.2～23.0kg.Ⅰ型、Ⅱ型和Ⅲ型PTA分别为26例、11例和6例.18例PTA的肺动脉直接下拖至右室流出道切口上缘相吻合,前壁再用心包补片扩大;8例用Homograft管道、14例用牛颈静脉管道连接远端肺动脉和右心室,重建右室流出道;3例Ⅱ型者,肺动脉后壁用左心耳壁与右室流出道上缘做吻合,前壁再用心包补片扩大.结果 术后所有病儿均生存,5例表现为右心功能不全,2例右肺动脉(RPA)压差37.5～47.3 mm Hg(1mm Hg=0.133kPa),术后17d恢复至35.3mm Hg以下.左心室流出道阶差均小于20.3mm Hg.残余VSD(2mm)1例,乳糜胸1例.5例病儿肺血管阻力高,吸一氧化氮(NO)治疗7d左右后好转.随访3个月～3年,2例病儿RPA残余压差24.0～29.3 mm Hg,均无明显右心室或左心室流出道梗阻.结论 肺总动脉后壁直接与右心室切口作吻合重建PTA的右室流出道,早期和远期效果良好.牛颈静脉的带瓣管道的应用,解决了小尺寸同种带瓣管道来源不足的问题,操作简便.     

两种不同手术方式矫治完全性大动脉错位伴有室缺和肺动脉狭窄的效果分析

目的 回顾性分析两种不同手术方式矫治完全性大动脉错位(TGA)伴有室间隔缺损(VSD)和肺动脉狭窄(PS)的效果.方法 对46例TGA/VSD/PS的患者进行手术矫治,其中采用主动脉根部移位/重建双室流出道术,即Nikaidoh术27例(N组),Rastelli手术19例(R组).N组采用自身心包补片重建右室流出道(RVOT),其中1例用同种异体带瓣管道Homograft;R组使用Homograft重建RVOT.两组均无手术前姑息手术史.结果 N组因术后严重心功能衰竭死亡1例(3.7%),R组无死亡.术后早期并发症的发生率两组相近.手术平均年龄N组(16.3±16)个月,R组(51±20)个月,N组明显小于R组(P=0.028).N组术后无明显残余左、右心室流出道梗阻(LVOTO、RVOTO),而R组有37%患者分别存在LVOTO或RVOTO(P＜0.05),但N组术后89%患者存在轻-中度肺动脉血反流现象,R组仅1例患者存在轻度反流(P＜0.05),两组手术早期心功能状况差异无统计学意义;随访期两组均无死亡,但R组有4例(23.6%)因LVOTO、RVOTO再手术治疗.结论 Nikaidoh术矫治TGA/VSD/PS患者,在解剖上更胜一筹,适宜于小的年龄患者.     

法乐四联症合并一侧肺动脉缺如的外科治疗

法乐四联症合并一侧肺动脉缺如临床少见。作者单位自１９６６年５月以来共收治的法乐四联症２２９４例，其中合并一侧肺动脉缺如２３例（约占１％），左肺动脉缺如１９例，右肺动脉缺如４例。９例应用右心室到肺动脉心外管道，其余的应用带单瓣右心室流出道补片。手术死亡２例，手术死亡率为８．７％。存活者术后恢复顺利，远期疗效较满意。作者认为，四联症合并一侧肺动脉缺如应选用带单瓣右心室流出道补片，如有异常冠状动脉或肺动脉闭锁则用右心室到肺动脉同种带瓣主动脉心外管道。     

牛颈静脉带瓣管道重建犬右心室流出道的血流动力学研究

目的观察自制牛颈静脉带瓣管道重建犬右心室流出道后的血流动力学性能. 方法应用经戊二醛处理的牛颈静脉带瓣管道对7只犬行肺动脉与右心室连接,重建右心室流出道前后测定肺血流动力学,行超声心动图检查牛颈静脉带瓣管道通畅情况. 结果重建术后1年7只犬均存活.重建右心室流出道前后肺动脉收缩压、舒张压和平均压均无明显改变,右心室舒张压无明显变化,收缩压和平均压显著增加(P<0.01).术后超声心动图检查瓣膜关闭良好,无明显反流,跨瓣压差小;术后1年超声心动图检测发现全部带瓣管道通畅,未见明显血栓形成,除1只犬瓣叶活动稍差合并轻度反流外,其余犬瓣叶活动良好.心导管测压显示肺动脉与右心室之间压差为3～19 mm Hg(1kPa=7.5mmHg),管道内舒张压显著高于右心室舒张压,右心室造影显示牛颈静脉带瓣管道和肺动脉无明显梗阻.结论戊二醛处理牛颈静脉带瓣管道重建右心室流出道具有良好的血流动力学性能.     

Gore-Tex片作单瓣重建右心室流出道的早、中期疗效

目的评价0.1mm厚的Gore-Tex片作肺动脉单瓣重建右心室流出道(RVOT)的早中期临床效果。方法2002年6月至2006年7月,对48例合并肺动脉狭窄或闭锁的先天性心脏病患者施行矫治手术,术中采用0.1mm厚的Gore-Tex片作肺动脉单瓣的Dacron血管片重建RVOT,其中包括法洛四联症合并肺动脉狭窄33例、型肺动脉闭锁8例、肺动脉瓣缺如3例,右心室双出口合并肺动脉狭窄2例,永存动脉干1例,完全型大动脉错位、室间隔缺损、肺动脉狭窄1例。结果本组无手术死亡。术后血氧饱和度达1.00,右心室/左心室收缩压比值0.22~0.65,右心室与左、右肺动脉压差<10mm Hg。所有患者(100%)均随访3~48个月,无死亡和并发症。彩色多普勒超声心动图检查提示:无肺动脉狭窄,肺动脉瓣轻度反流13例,中度反流5例,Gore-Tex膜具有活动功能40例。结论采用0.1mm厚的Gore-Tex片作单瓣重建RVOT,其早中期临床结果显示有较满意的血流动力学效果。     

Aneurysm of pericardial right ventricular outflow tract patches.

Among 1,022 patients who underwent repair of tetralogy of Fallot, 252 received a pericardial patch of the right ventricular outflow tract; of these, 10 subsequently developed an aneurysm of the right ventricular outflow tract. Cardiac catheterization and angiography revealed moderate pulmonary insufficiency in all patients, a residual pressure gradient in the right ventricular outflow tract in 7, and a residual ventricular septal defect in 2 patients. Reoperation was indicated in 8 patients because of progressive distention of the aneurysm, residual infundibular or pulmonary artery stenosis, and recurrent ventricular septal defect. Reconstruction of the right ventricular outflow tract was accomplished by resection of the aneurysm and insertion of a woven Dacron patch in 5 patients, primary suture of the pulmonary artery in 2, and implantation of a woven Dacron conduit containing a Björk-Shiley cardiac valve prosthesis in 1 patient. There were no early or late deaths. When reconstruction of the right ventricular outflow tract is necessary, we recommend a woven Dacron patch because pericardium may form an aneurysm.     

Morphosurgical correlation of outcomes in complete double outlet right ventricle

Even though surgical experience with wide spectrum of double outlet right ventricle (DORV) is available, the experience with extreme form of complete DORV where both great arteries completely arise from the right ventricle is limited. We present our experience with this unique subset where the systemic outflow is far removed from the interventricular foramen and hence, the systemic ventricle. In this situation, biventricular repair can be technically demanding and challenging. Between June 2002 and February 2006, 12 patients underwent biventricular repair of this subset. The VSD was subaortic in all; eight patients had infundibular and valvar obstruction. Aorta was anterior and to the right, with the pulmonary artery far posterior and to the left. The interventricular foramen was patched with a long Gore-Tex patch to route the LV flow to the aorta. Eight patients had infundibular resection and right ventricular outflow tract (RVOT) enlargement with an autologous monocusp pericardial patch. No patient required a valved conduit. There was no operative mortality. In one patient, there was a small residual VSD that was not of haemodynamic significance. There was no RVOT and left ventricular outflow tract obstruction and no RV inflow obstruction. The early and mid-term results are good.     

Innovative technique for repair of Tetralogy of Fallot with absent pulmonary,valve syndrome using autologous pericardial patch with monocusp valve

Background  Conventional surgery for absent pulmonary valve is repair with valve conduit between right ventricle (RV) and Pulmonary artery (PA).We describe a technique of repairing absent pulmonary valve with autologous pericardial patch with monocusp valve, without using valve conduit and its results. Methods  From February 2004 to September 2006, 14 consecutive patients with absent pulmonary valve syndrome (APVS) were repaired using this technique. Age range was 5–168 months (median- 24 months) and weight range was 6–31 kgs (median-10 kgs), 5 were infants. Varying degree of respiratory symptoms were present in all patients including tachypnoea, difficulty in feeding, wheezing, recurrent pneumonia and cyanosis. Repair consisted of ventricular septal defect closure, relief of right ventricular outflow obstruction with autologous pericardial patch with monocusp valve. PA aneurysmorrhaphy and PA reduction arterioplasty were done in selected cases. Result  There was one mortality. The follow up ranged from 12 months to 36 months, respiratory symptoms disappeared or were significantly improved in all patients. Two dimensional (2D) Echo showed trivial to mild pulmonary regurgitation (PR) in 11 patients & moderate in two patients. One patient had minimal right ventricular outflow tract (RVOT) obstruction. None of them required reintervention. Conclusion  New innovative technique of using autologous pericardial patch with monocusp valve in absent pulmonary valve syndrome can be safely performed in infants and children. It avoids conduit related problems like reintervention and is economical. Our early and midterm results are encouraging, however long term results are awaited.     

Transatrial-Transpulmonary Repair of Atrioventricular Septal Defect with Right Ventricular Outflow Tract Obstruction

A bstract Twenty patients had a repair of an atrioventricular septal defect with tetralogy of Fallot (n = 13) or double outlet right ventricle (n = 7). Mean age was 3.5 years. Surgical technique included transatrial-transpulmonary resection of right ventricular outflow tract obstruction and transatrial two patch repair of the atrioventricular septal defect. Ten patients required a transannular patch and one patient had a right ventricle-pulmonary artery conduit placed. There was no hospital mortality, and mean hospital stay was 15 days. One patient had late sudden death of unknown cause. Six patients have required reoperation because of residual ventricular septal defect (VSD), mitral incompetence, residual right ventricular outflow tract obstruction, and/or conduit stenosis. No patient was reoperated on because of left ventricular outflow tract obstruction. Fifteen patients are asymptomatic, one has exertional dyspnea, and two have intermittent occasional bronchospasm. The transatrial-transpulmonary two patch repair and extensive relief of right ventricular outflow tract obstruction have given good immediate results. Reoperation rate has been high mainly due to residual VSD and mitral incompetence. ( J Card Surg 1993; 8:622–627 )     

阜外医院71例Rastelli手术的近中期结果

目的总结分析阜外医院Rastelli手术的近中期结果。方法2010年5月至2017年3月阜外医院完成71例Rastelli手术治疗大动脉转位(TGA)或者右心室双出口(DORV)合并室间隔缺损(VSD)和肺动脉狭窄患儿,男48例,女23例;手术时年龄(4.7±2.7)岁,其中10例完全型大动脉转位;27例DORV,34例矫正型TGA(CTGA)。30例有手术史,其中Blalock-Taussig分流13例,双向Glenn手术17例。31例同时扩大室间隔缺损。外管道使用同种带瓣管道9例,牛颈静脉管道56例,自制Gore-Tex外管道6例;管道直径(17.9±3.3)mm。结果本组主动脉阻断(132.0±71.1)min,体外循环(209.0±83.4)min。机械通气时间(102.6±81.7)h。术后住院(13.6±12.8)天。早期死亡1例(1.4%)。早期并发症12例(16.9%),其中因Ⅲ度房室传导阻滞安装永久起搏器4例,心包积液开窗3例,延迟关胸3例,二次开胸2例。术后随访4个月~6.8年。1、5年生存率分别为97.2%,97.2%;1、5年免除右心室流出道狭窄(RVOTO)率分别为98.6%,84.1%;1、5年免除干预率分别为98.6%,90.0%。随访期间置换外管道1例,介入球囊扩张7例10次。所有患儿最后一次随访均未见左心室流出道狭窄,左心室到主动脉压差(10.5±8.8)mmHg。结论Rastelli手术适用于部分DORV、CTGA、TGA合并VSD和严重的肺动脉狭窄或肺动脉闭锁患儿,早期死亡比例低,中期效果好,但是远期常需要再手术,尤其是置换外管道。     

完全性心内膜垫缺损合并右室双出口的双心室修复

目的 总结完全性心内膜垫缺损合并右室双出口双心室修复的经验.方法 2006年7月至209年11月对6例完全性心内膜垫缺损合并右室双出口病人采用双片法修补完全性心内膜垫缺损,人工血管补片作右心室内隧道,连接室间隔缺损与主动脉口,引导左心室血经内隧道进入主动脉;应用牛心包补片扩大右室流出道;肺动脉瓣环小者用带瓣补片跨环修补右室流出道;心室内隧道有可能阻塞右室流出道造成重度梗阻者,右室流出道与主肺动脉之间安置带瓣管道(Rastelli手术).结果 术后死亡1例,占16.6%,其余病例恢复良好.结论 完全性心内膜垫缺损合并右室双出口一期双心室修复可获得良好效果.

Abstract：

Objective Double-outlet right ventricle,which often associated with total anomalous pulmonary venous connection and complete endocardial cushion defects,has been considered a risk factor for biventricular repair procedure.To reviewed cases treated by biventricular repair for endocardial cushion defects with double outlet right ventricle.Methods From July to November of 2009,6 patients (3 males and 3 females) aged from 7 to 24 (16.17±5.98) years and with endocardial cushion defects and double outlet right ventricle underwent operation of biventricular repair The duration of follow-up ranged from 10 days to 2 years(median,16 months).Endocardial cushion defects were repaired with a 2-patch technique.The artificial vascular patch was implanted to connect the ventricular septal defects and the aorta for draining the blood stream from the left ventricle to the aorta.The other patch was used to repair the ostium primum atrial septal defects.Right ventricular outflow tract obstructions was released and reconstructed by transplanting a bovine pericardium patch.If the size of pulmonary valve annulus was far more below the normal,a transannular pericardial patch was used.Rastelli procedure with a valved conduit between the right ventricle and the pulmonary artery would be performed if the obstruction in the right ventricular outfolw tract was severe.Results One death occurred 2 days after the operation,resulting in a mortality rate of 16.6%.The case,a boy of 7 years old,had a mirror-image dextrocardia,complete endocardial cushion defect ( C type),anomalous pulmonary venous drainage and single atrium.In this case,the operation lasted for 8 hours,acute renal failure happened next day to the operation,the effect of CRRT was unsatisfied,and eventually cardiac arrest occurred as a result of hyperkalemia.The remaining cases had survived.Follow-up examinations showed that the systolic pressure gradients across the pulmonary valves decreased,with a range of 14 to 40 mm Hg,(23.9 ± 11.03) mm Hg.Mild mitral and tricuspid regurgiration were identified in 4 cases and moderate mitral regurgitation was identified in one case.The cardiac function in all patients was classified as NYHA class Ⅰ/Ⅱ,Conclusion Endocardial cushion defects with double outlet right ventricle can be corrected by means of biventricular repair procedure.The procedure was associated with a low mortality,The interim life quality of patients may be improved.The longterm outcomes should be further studied.     

Repair of complete atrioventricular septal defect with tetralogy of Fallot

Repair of complete atrioventricular canal with tetralogy of Fallot was performed in 9 patients. Ventricular septal defect was closed through the right atrium using a single polytetrafluoroethylene patch with ample anterior extension to avoid subaortic obstruction. The atrial septal defect was closed with a separate patch. Undivided atrioventricular valve leaflets were sandwiched between the two patches. Right ventricular outflow tract stenosis was relieved by pulmonary valvotomy and an infundibular patch in 7, a supravalvar patch (none transannular) in 6, and right ventricle-to-pulmonary artery conduit in 2. There was one hospital death (1/9, 11%) in a patient with persistent clinically significant postoperative pulmonary stenosis and low cardiac output requiring reoperation and right ventricle-to-pulmonary artery conduit insertion. There was no late mortality. All patients are asymptomatic 0.3 to 5.6 years after operation. Follow-up right ventricular outflow tract gradient ranged from 11 to 43 mm Hg and was 70 mm Hg in 1 patient who later had successful relief of obstruction. Three patients had mitral valve insufficiency; 1 needed reoperation. Aggressive relief of right ventricular outflow tract stenosis with maintenance of pulmonary valve competence and use of two separate patches for closure of the septal defects contribute to optimum immediate and long-term results after repair of this lesion.     

Fifteen years' experience with the aortic homograft: the conduit of choice for right ventricular outflow tract reconstruction

Ninety-seven patients with pulmonary atresia underwent right ventricular outflow tract reconstruction using a homograft conduit. There were 46 hospital deaths (47%). Hospital mortality was significantly related to irreversible pulmonary hypertension (p less than 0.001) and thoracotomy for ligation of bronchial collaterals (p less than 0.01). The actuarial survival was 37 +/- 7% at 10 years. Sixteen patients undergoing recatheterization at a mean of 6 years had a mean transconduit gradient of 24 +/- 15 mm Hg. Obstructed conduits (i.e., with a gradient of greater than 50 mm Hg) were replaced in 3 patients, corresponding to 13 +/- 8% at 10 years. In each instance, the obstruction was due to neointimal hyperplasia in the Dacron tube rather than calcification of the homograft valve. The fresh, antibiotic-sterilized aortic homograft is the conduit of choice for right ventricular outflow tract reconstruction. The valve itself appears more resistant to calcification than its xenograft counterpart, and the absence of Dacron removes the problem of fibrinous peel obstructing the conduit. We now construct a tube of autologous pericardium to increase the length of the conduit and avoid complementary thoracotomy for ligation of bronchial collaterals.     

Autologous Pericardium Patch Aneurysm after Ventricular Septal Defect Closure and Arterial Switch Operation

Abstract   A neonate with L-transposition of the great arteries with ventricular septal defect underwent complete repair using fresh autologous pericardium to close the ventricular septal defect as well as to reconstruct the neo pulmonary artery sinuses. Four months later, the child came back with right ventricular inflow obstruction related to aneurysmal pericardial patch, severe tricuspid regurgitation, and severe supra-valvular pulmonic stenosis. At reoperation, there was a redundant, aneurysmal pericardial patch densely adherent to the septal and posterior leaflets of the tricuspid valve, which was damaged. The pericardial patch was replaced, the pulmonary artery enlarged, and tricuspid valve repaired. Postoperative course was uneventful, but residual moderate tricuspid regurgitation required intensive medical treatment.     

冠状动脉畸形对复杂先心病右室流出道重建的影响

目的探讨冠状动脉畸形对复杂先心病右室流出道（RVOT）重建的影响。方法自1989年4月至2004年5月治疗18例合并冠状动脉畸形的法洛四联症（14例）和右室双出口（4例）患者。右冠状动脉开口缺如7例,左冠状动脉开口缺如4例,左前降支起自右冠状动脉6例,左右冠状动脉交通支形成蔓状血管丛1例。均在全身麻醉低温体外循环下手术,1例行姑息手术,17例行根治术,RVOT重建的方法：游离畸形冠状动脉从其下方做切口,RVOT心包补片扩大术4例;3例在异常交通支下方平行切口修补室间隔缺损（VSD）,交通支上方纵切口加宽RVOT和肺动脉瓣环;4例行右心房切口修补VSD,RVOT小切口和肺动脉切口心包补片扩大术;3例肺动脉主干与RVOT直接吻合;3例同种带瓣主动脉重建RVOT。结果术后早期死于顽固性低心排血量综合征1例,无严重出血并发症和呼吸道并发症,姑息手术1例,血氧饱和度由术前的68．0％升至82.0％。16例随访10个月～8年,1例术后1年不明原因猝死;超声心动图示轻度残余漏3例,残余梗阻3例。结论复杂先天性心病并冠状动脉畸形RVOT重建时,应根据冠状动脉畸形特点选择手术方式。