Successful correction of double outlet left atrium associated with complete atrioventricular canal and l-loop double outlet right ventricle with stenosis of the pulmonary artery.

The first successful correction of "double outlet left atrium" was presented. This lesion is inevitably associated with complete A-V canal, and may have many associated anomalies. Technical problems concerning the total correction of this anomaly were discussed.     

Primary repair of pulmonary artery sling with double outlet right ventricle and distal tracheal stenosis

The fate of patients with pulmonary artery sling depends on associated tracheal lesions. Distal tracheal stenosis involving the carina frequently results in lethal obstruction due to secretion or inflammatory edema. Further-more, associated complex cardiac anomalies with excessive pulmonary flow make the situation more complicated. We present a case of successful simultaneous one-stage repair of pulmonary artery sling, double outlet right ventricle and distal tracheal obstruction. Pulmonary artery sling was relieved by relocation of the left pulmonary artery (left pulmonary artery to main pulmonary artery) and a tracheoplasty (resection and end-to-end anastomosis). We advocate early aggressive simultaneous repair of pulmonary artery sling with tracheal stenosis and concomitant repair of intracardiac anomalies whenever possible.     

Double-Switch手术纠治房室连接不一致的复杂先天性心脏病

目的 回顾和总结Double-Switch手术方法的临床应用体会.方法 从2002年1月至2006年12月,采用Double-Switch方法纠治房室连接不一致复杂型先天性心脏病(先心病)共14例.其中男11例,女3例;手术年龄3个月-7岁,平均(39.0±35.27)个月;体重5-31 kg,平均(14.23±8.27)kg.包括纠正型大血管错位9例,房室连接不一致型右心室双出口 5例.10例伴肺动脉狭窄,采用Senning+Ras-telli手术;余4例采用Senning+Switch手术.结果 死亡2例,为采用Senning加Restelli手术者.全组无发生完全性房室传导阻滞者.生存病儿随访2个月-5年,远期死亡1例.Senning+Rastelli手术后出现心室内0.2 cm残余分流1例,上腔静脉回流流速增快2例,分别为1.8 m/s和2.16 m/s,心功能均在正常范围,继续随访中.Senning加Switch手术者随访结果满意,无任何残余分流和梗阻.结论 Double-Switch手术纠治房室连接不一致的复杂型先心病,结果较好,但仍需进一步随访以观察长期疗效.     

Anatomical repair of double outlet right ventricle associated with complete atrioventricular septal defect and pulmonary stenosis: extending the indications for aortic translocation

The combination of double outlet right ventricle (DORV) and complete atrioventricular septal defect (CAVSD) remains a surgical challenge for anatomical repair. Inasmuch as the ventricular septal defect is noncommitted in this combination, the major concern regarding anatomical repair is the reconstruction of the unobstructed left ventricular outflow tract without compromising right ventricular volume and the right ventricular outflow tract. We report on a patient who underwent an anatomical repair using aortic root translocation for DORV with CAVSD and pulmonary stenosis (PS).     

Aberrant right pulmonary artery and double outlet ventricle: one-stage repair

We report a 13-month-old male child with anomalous origin of the right pulmonary artery from the ascending aorta and a double outlet right ventricle. Aortic wall was used for elongation of the pulmonary artery and side-to-end connection to the pulmonary trunk. Special emphasis is made on this particular operative technique for strictly laterally originating right pulmonary artery that requires no prosthetic material, avoids extreme stretching, and may enable normal growth potential.     

房室连接不一致的心室双出口外科治疗

目的　报道房室连接不一致的心室双出口外科治疗经验。　方法　从 1985年 1月至 2 0 0 2年 9月 ,共进行房室连接不一致的心室双出口外科治疗 11例 (年龄 3～ 2 5岁 ) ,其中右心室双出口 (DORV) 10例 ,左心室双出口(DOL V) 1例 ,行双心室修复术 9例 ,采用心内补片、心外带瓣管道或冷冻同种带瓣管道修复 ;行 Fontan类手术 2例。结果　双心室修复术后早期死亡 2例 ,死亡原因为严重低心排血量综合征 ;Fontan类手术修复后血氧饱和度和活动耐力明显改善 ,无早期死亡 ;双心室修复术后长期随访 4例 ,随访时间 1～ 11.5年 ,NYHA心功能 级或 级 3例 , 级 1例。　结论　房室连接不一致的心室双出口根据心室、肺动脉发育和合并心内畸形决定手术方式 ,完整修复室间隔缺损 ,保证心室大动脉流出道通畅 ,修复房室瓣和防止损伤传导束是传统心内修复术的关键。     

半旋转动脉干调转术治疗完全性大动脉转位合并室间隔缺损和肺动脉狭窄

目的 报道半旋转动脉干调转术治疗完全大动脉转位(TGA)合并室间隔缺损(VSD)和肺动脉狭窄(PS)的近期疗效.方法 2例病儿男、女各1例,分别为16岁和19个月.均为TGA/VSD/PS.采用半旋转动脉干调转术治疗.结果 2例病儿均生存.女病儿术后发生低心排出量综合征和毛细血管渗漏综合征,治疗后痊愈.男病儿术后恢复良好.术后分别随访10、9个月,心功能Ⅰ级.结论 半旋转动脉干调转术可有效治疗TGA合并VSD和PS.长期效果仍需观察.     

右室双出口的外科治疗

目的：总结手术纠治右室双出口６７ 例的经验。资料与结果：手术年龄４ 个月～１２ 岁,平均（４ ．９８ ±２ ．９６） 岁;体重４ ．１ ～３６ ．０ｋｇ,平均（１５ ．１３ ±５ ．５４）ｋｇ 。其中伴右室流出道梗阻４８ 例,肺动脉高压１８例,肺动脉瓣闭锁１ 例。１３ 例行姑息手术者无死亡。５４ 例行根治术,其中４ 例又行ＩＩ期根治,６ 例行Ｆｏｎｔａｎ 纠治术;根治手术死亡５ 例,病死率８ ．６２ ％ 。本组总病死率７ ．４６ ％ 。结论：右室双出口伴肺动脉高压者,必须早期手术,防止肺血管病变发生。对肺动脉瓣下型室缺的手术纠治较困难,死亡率高。心内隧道补片方法,术后需定期随访,及时发现左室流出道梗阻,必要时需再次手术解除。     

右心室双出口的解剖矫治

目的 总结不同方法进行解剖矫治右心室双出口(DORV)经验.方法 2007年5月至2012年5月,收治135例DORV患儿,男79例,女56例;年龄为出生后25天～12岁;体质量3.5 ～ 30.0 kg.根据室间隔缺损的位置选择不同手术方法:89例主动脉瓣下缺损及33例双瓣下缺损患儿均行右心室双出口解剖矫治术;8例肺动脉瓣下缺损(Taussig-Bing畸形)患儿中5例行Rastelli手术,3例行动脉调转术±室间隔修补术;5例远离大动脉缺损中3例行Rastelli手术,2例行动脉调转术±室间隔修补术.结果 住院死亡5例,占3.70％;分别为1例肺动脉瓣下缺损行动脉调转术±室间隔修补术,1例双瓣下缺损,2例主动脉瓣下缺损(均合并肺动脉瓣狭窄)均行合右室双出口解剖矫治术,1例远离大动脉缺损行Rastelli手术者.结论 右室双出口病理解剖比较复杂,须根据不同的解剖条件选择不同的手术方法才能减少手术死亡.     

Closed brock operation for an infant case of double outlet right ventricle with pulmonary atresia

One-month-old boy with double outlet right ventricle and pulmonary atresia was referred to us for sugical treatment. As he had a membranous obstruction of the pulmonary artery we chose closed Brock operation as an initial procedure. A 6.5 mm in diameter of Trocar was used to open the atretic valve. Postoperative course was uneventful and his oxygen saturation was maintained satisfactory. At the age of one year and nine months he underwent corrective surgry. The pulmonary valve was widely opened enough to perform the right ventricular outflow tract reconstruction with valvotomy and outflow patch. The boy got well and was followed in the outpatient clinic. Double outlet right ventricle with membranous pulmonary atresia is a rare combination and closed Brock operation is shown to be one of the most effective procedure for this type of anomaly.     

303例右室双出口手术后早期转归

目的　总结右室双出口 (DORV)病例的术后处理及不同外科术式的早期转归。方法　 1988年 1月至 2 0 0 3年 12月期间收治 30 3例DORV病儿 ,按室间隔缺损 (VSD)的部位分为主动脉下VSD ,肺动脉下VSD ,双动脉下VSD和远离大动脉下VSD 4组。总结比较不同外科术式术后的并发症和死亡率及其原因。结果　再手术 18例 ,死亡 2 9例 ,死亡率为 9 6 % ,其中肺动脉下VSD死亡率最高 ,为 2 4 . 4 % (χ2 =14 . 12 ,P <0 . 0 1) ;根治术和姑息术的死亡率分别为 10 . 6 %和 6 . 6 % (χ2 =1. 0 3,P >0 .0 5 )。死亡原因主要是低心排血量综合征 (LCOS) ,占 82 . 6 % ;其也是术后主要并发症 ,占 31 .0 %。结论　LCOS是术后主要并发症和死亡的高危因素。有效预防和控制LCOS是取得治疗成功的关键。     

Double outlet right ventricle with L-position of the aorta, D-loop, subaortic VSD, and pulmonary stenosis.

We describe a case of double outlet right ventricle with subaortic ventricular septal defect and pulmonary stenosis treated successfully with cardiopulmonary bypass. We consider the clinical history and angiocardiographic and surgical findings of this rare anomaly. We stress the difficulties of reconstruction of the outflow tract of the right ventricle, because of the anomalous pathway of the right coronary artery, the posterior situation of the pulmonary artery, and the abnormal anatomy present in the outflow tract of the right ventricle.