完全型房室间隔缺损合并法洛四联症心内矫正术

目的总结心内矫正术治疗完全型房室间隔缺损合并法洛四联症(CAVSD-TOF)的经验. 方法从1985年1月至2002年5月,共行CAVSD-TOF心内矫正术12例,年龄6～16岁(11.1±2.8岁),采用右心房、右心室纵切口,前7例采用三片法补片,后5例用二片法补片修补房室间隔缺损,左侧房室瓣裂隙采用间断缝合,右心室流出道用跨瓣补片加宽. 结果术后早期死亡4例,前7例死亡3例,后5例死亡1例,死亡原因为严重低心排血量3例,灌注肺1例.长期随访6例,随访时间3个月至13.5年,无任何症状,NYHA心功能Ⅰ级或Ⅱ级. 结论 CAVSD-TOF采用右心房、右心室纵切口,二片法补片修补房室间隔缺损,常规间断缝合左侧房室瓣裂隙,跨瓣补片加宽右心室流出道能取得较良好的效果.     

双心室矫治术治疗右心室双出口合并完全性房室间隔缺损

目的总结右心室双出口(DORV)合并完全性房室间隔缺损(AVSD)的双心室解剖矫治经验。方法回顾性分析1996年1月至2010年12月阜外心血管病医院14例DORV-AVSD患者施行双心室解剖矫治术的临床资料,其中男9例,女5例;年龄6个月～31岁。患者均行双心室解剖矫治术,经右心房和右心室切口,疏通右心室流出道,分隔并成形房室瓣,采用"逗号状"补片修补室间隔缺损,同时构建通畅的左心室流出道,自体心包闭合Ⅰ孔房间隔缺损,用心包或跨瓣环补片加宽右心室流出道。结果 1996年1月至2008年12月收治的10例患者中,住院死亡5例,其中术中不能脱离体外循环3例,不能脱离呼吸机2例;住院时间23～105 d,住ICU时间5～90 d,机械通气时间1～52 d。2009年1月至2010年12月收治的4例患者中,无住院死亡,术后未发生并发症;住院时间21～41 d,住ICU时间4～21 d,机械通气时间1～7 d。随访9例,随访时间6～26个月,随访期间无死亡,无流出道残余梗阻。结论 DORV-AVSD患者可一期行双心室矫治术,近年来手术效果明显提高。     

小儿肺动脉闭锁伴室间隔缺损右室流出道重建方法

目的探讨和评价小儿肺动脉闭锁（PA）伴室间隔缺损（VSD）右室流出道重建方法的应用。方法1999年4月至2004年12月，收治81例PA伴VSD行一期或分期的双心室修补术病儿。一期根治术组32例，大部分病例采用自体心包片跨瓣环水平扩大右室流出道至肺总动脉，其中4例带心包单瓣，合并主肺动脉侧支（MAPCAs）的11例中有2例完成肺动脉血管的单元化手术。姑息手术组49例，分别采用不同的右室流出道重建方法。其中15例完成了二期根治术，大部分采用同种异体心包片跨瓣补片。结果一期根治术死亡4例（12．5％）；姑息手术死亡3例（6．1％）；二期根治术无死亡。出院时根治术组经皮氧饱和度0．95～0．98。随访3～24个月。姑息手术为0．79～0．87，McGoon指数从0．4～0．7增至1．1～1．6。结论术前判断肺动脉干、肺动脉分支发育情况、中央共汇、有无MAPCAs，对手术方法的选择极为重要。术后PRV／PLV的测定有助于右室流出道重建方法预后的评估。     

改良单片法矫治儿童完全型房室间隔缺损单中心应用

目的 总结应用改良单片法(modified single-patch,MSP)矫治儿童完全型房室间隔缺损(complete atrioventricular septal defect,CAVSD)的相关经验.方法 回顾性分析2009年6月至2017年12月间在我中心采用MSP技术行CAVSD双心室矫治术141例患儿的...     

法洛四联症合并肺动脉闭锁的外科治疗

目的探讨法洛四联症合并肺动脉闭锁(TOF-PA)的外科治疗，重点讨论不同类型的手术时机和方法及适应证。方法1984年6月至2003年12月对24例TOF—PA病儿进行矫治手术，其中男10例，女14例；年龄6个月～9岁；Ⅰ型14例，Ⅱ型8例，Ⅲ型和Ⅳ型各1例。全组均采用中度低温体外循环下进行一期矫治手术，Ⅰ型采用跨瓣环的带单瓣的人工血管补片加宽，Ⅱ型采用右心室到肺动脉带瓣管道，Ⅲ型和Ⅳ型TOF-PA采用胸骨正中切口一期单源化和心内修复术。结果术后早期死亡4例，其中Ⅰ型、Ⅱ型各2例，死因为严重低心排血量综合征2例、灌注肺和多脏器功能衰竭各1例。Ⅰ型和Ⅱ型长期随访16例，随访1个月～15．5年，NYHA心功能Ⅰ或Ⅱ级14例，Ⅲ和Ⅳ级各1例；Ⅲ型和Ⅳ型分别随访1个月和6个月，效果满意。结论TOF-PA一经诊断应尽快手术，条件较好的早期行矫治手术可得到满意的效果。     

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。     

完全性房室间隔缺损合并法乐四联症的手术矫正

目的　报告完全性房室间隔缺损合并法乐四联症的外科治疗。　方法　 6例患者 ,年龄 3～ 9岁 ,均施行手术根治 ,经右房 右室切口修补VSD ,房室间隔缺损采用两块补片技术修复 ,左房室瓣裂隙的修复均采用间断缝合 ,右室流出道均用带单瓣的补片跨瓣加宽。　结果　全组 6例术后出现低心排综合征、多器官衰竭、灌注肺并发症各 1例 ,早期死亡 1例为多器官衰竭者 ,5例长期存活。术后随访 6个月～ 5 5年 ,1例有轻度左房室瓣关闭不全。心功能Ⅰ级 4例 ,Ⅱ级 1例。　结论　完全性房室间隔缺损合并法乐四联症可应用 2块补片法行手术根治 ,左房室瓣瓣裂的常规修补可降低其关闭不全的发生率 ,大部分患者术后心功能可获得良好的改善。     

法洛四联症合并完全性房室间隔缺损的手术治疗

目的 总结并探讨法洛四联症合并完全性房室间隔缺损(TOF-CAVC)的外科治疗方式及时机.方法 2007年6月至2012年4月,11例TOF-CAVC患儿行双心室或部分双心室修补,男6例,女5例;年龄5 ～32个月.手术经右心房或右心室流出道纵切口,采用“双片法”修补房室通道,其中8例通过单一的右心房切口修补室间隔缺损.所有患儿均行右心室流出道-肺动脉扩大补片修补手术,其中7例由于肺动脉瓣发育欠佳需跨瓣修补,1例用带单瓣的心包补片扩大右室流出道,3例未跨瓣修补.结果 手术死亡1例,术后第2天死于严重低心排出量综合征.1例术后6个月死于肺炎、心力衰竭.生存9例术后均有效随访,随访3～60个月.根据Kaplan-Meier生存曲线,5年生存率为79.5％.术后3个月,2例患儿分别出现右心室流出道及肺动脉分支梗阻,2例残余房间隔缺损0.2 ～0.3 cm,1例心室水平残余细丝分流,患儿均无明显临床症状.随访期间所有生存患儿心功能Ⅰ～Ⅱ级.结论 法洛四联症合并完全性房室间隔缺损近期疗效肯定,采用双片法通过单一的右心房切口修补房室通道时,术中的精细操作是手术成功与否的关键.     

小儿重症法洛四联症二期根治术

目的　临床评价小儿重症法洛四联症二期根治术手术疗效。方法　1987年3月至1998年2月51例重症法洛四联症病儿,年龄2.1～13.9岁,平均(5.1±1.9)岁;体重11.1～28.0kg,平均(14.2±3.8)kg。姑息术与二期根治术时间间隔3～22个月。结果　1例远端肺血管发育差者,术后心衰死亡。手术成功率98%,术后随访满意。结论　二期根治术能提高重症法洛四联症病儿术后生存率。     

136例成人法乐四联症的外科治疗

目的探讨外科治疗成人法乐四联症的经验。方法从１９７５年４月至１９９７年１２月,对１３６例１４周岁以上成人法乐四联症施行根治术,最大年龄为５２岁。室间隔缺损修补材料中,１１２例采用涤纶补片,２４例采用自体心包补片。在１２６例使用补片扩大右心室流出道的病例中,９５例采用牛心包补片,３１例采用自体心包补片。结果住院死亡５例,住院病死率为３６８％。术后发生低心排综合征１７６％、心律失常１４０％、胸内出血需再次开胸止血１１８％、胸腔积液２１３％、心包积液５９％、肺部感染１２５％、感染性心内膜炎２２％。结论成人法乐四联症根治术有其自身特点,通过改进手术技术,加强术后监护处理,可以提高手术成功率     

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.     

Late results of total correction of tetralogy of Fallot in adults

Between 1966 and 1986, 30 patients underwent total correction of the tetralogy of Fallot. Preoperative presenting features were: dyspnoea on exertion, clubbing, cyanosis and polycythaemia. Twenty-six patients had one or more palliative procedures prior to definitive repair. Preoperatively, all patients had a significant gradient across the right ventricular outflow tract (mean gradient 70 ± 46 mmHg). Peak right ventricle to left ventricle systolic pressure ratio (pRV/LV) was 0.9 ± 0.2. A functioning Blalock Taussig shunt was ligated in 11 patients prior to the institution of cardiopulmonary bypass. All patients had a patch closure of the ventricular septal defect. An additional muscle bundle resection from the right ventricular outflow tract was performed in 15, pulmonary valvotomy in 6 and enlargement of the right ventricular outflow tract in 2 patients. There was a significant fall in pRV/LV ratio postoperatively (P < 0.05). There were 3 early and 2 late deaths. Mild right ventricular outflow tract obstruction has persisted in all survivors. Four patients have remained on antiarrhythmic drugs. Long term results after definitive repair were satisfactory in this group of adult patients who have survived due to palliative procedures performed during childhood.     

Correlation of ventricular septal defect height and outcomes after complete atrioventricular septal defect repair

 Open in a separate windowOBJECTIVESThere are limited data available on the height of the ventricular component of the septal deficiency (VSD) in patients undergoing complete atrioventricular septal defect (CAVSD) repair. VSD height may influence optimal choice of repair strategy with potential consequences for long-term outcomes. We aimed to measure VSD height using 2-dimensional echocardiography and review its association with postoperative outcomes.METHODSWe retrospectively reviewed the preoperative echocardiograms of 45 consecutive patients who underwent CAVSD repair between May 2010 and December 2015 at a single centre. VSD height and left ventricular length on the four-chamber view were measured. Demographic details and early and late outcomes including reoperation and long-term survival were studied.RESULTSTwenty patients underwent modified single-patch repair and 25 patients underwent double-patch repair of CAVSD. VSD height in the modified single-patch group ranged from 4.2 to 11.7 mm and in the double-patch group ranged from 5.1 to 14.9 mm. Nine patients had a deep ‘scoop’ with a VSD height of >10 mm, (7 double patch, 2 modified single patch). VSD height did not correlate with a specific Rastelli classification. There was no significant difference in the VSD height (P = 0.51) or the VSD height-to-left ventricular length ratio (P = 0.43) between the 2 repair groups. There was no 30-day mortality. Eight patients required reoperation; however, VSD height was not a significant predictor of reoperation (hazard ratio 0.95, 95% confidence interval 0.69–1.33; P = 0.08).CONCLUSIONSThere was no correlation between VSD height and risk of reoperation after CAVSD repair. A deep ventricular scoop is uncommon in CAVSD patients.