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.     

Surgical repair for double outlet right ventricle and intact ventricular septum

We report the case of a neonate with a complex cardiac anomaly that consisted of a double outlet right ventricle, intact ventricular septum, small left ventricle, and pulmonary stenosis who underwent surgical repair with a successful outcome. We have not previously found a case report of a patient with double outlet right ventricle and intact ventricular septum who is still alive and in good health at an intermediate postoperative follow-up.     

Biventricular repair of double outlet right ventricle with noncommitted ventricular septal defect

Double outlet right ventricle (DORV) with noncommitted ventricular septal defect (VSD) (DORVncVSD) represents the most extreme form of DORV, raising challenging surgical difficulties for biventricular repair. The considerable distance between the VSD and the aorta is primarily because of the very abnormal location of the aorta. The definition of DORVncVSD includes: (1) a VSD distant (greater than aortic diameter) from both arterial valves; (2) both great vessels arising fully from the right ventricle; and (3) a double conus. Double outlet right ventricle with noncommitted ventricular septal defect is a primitive right ventricle, as seen during embryologic development, characterized by the presence of the entire conotruncus from the right ventricle. One surgical technique for repair of DORVncVSD is rerouting of the VSD to the aorta by a long intraventricular tunnel. This technique is limited by the presence of conal tricuspid chordae and by the distance between the tricuspid and pulmonary valves, and is associated with an important risk of subaortic obstruction. Rerouting through the pulmonary artery followed by arterial switch seems a more satisfactory surgical solution. When the VSD is distant from the aorta, it is almost always quite close to the pulmonary artery. Rerouting to the pulmonary artery creates a smaller channel, and its application is not limited by the presence of tricuspid chordae or the tricuspid-to-pulmonary valve distance. However, the arterial switch frequently involves relocating complex coronary arteries. Copyright © 2002 by W.B. Saunders Company     

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。     

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.     

Biventricular repair of double outlet right ventricle with non-committed ventricular septal defect (VSD) by VSD rerouting to the pulmonary artery and arterial switch

Objectives: Biventricular repair of double outlet right ventricle non-committed ventricular septal defect (DORVncVSD) is usually achieved by a VSD rerouting to the aorta. This technique can be limited by the presence of tricuspid chordae and by the pulmonary artery to tricuspid valve distance. Furthermore, there is an important risk of late subaortic obstruction related to the long patch required that creates a potential akinetic septal area. Presented here is another technique; by VSD rerouting to the pulmonary infundibulum and arterial switch. Methods: Ten patients, with DORVncVSD, underwent a VSD rerouting to the pulmonary infundibulum followed by arterial switch. Seven had a previous pulmonary artery banding and one a moderate infundibular stenosis. The median age at surgery was 16 months (range 3 weeks to 4.5 years). All patients had a bilateral infundibulum, with a large persistent subaortic conus, D malposition of the aorta, side-by-side vessels and double loop coronary patterns. The VSD was perimembranous with inlet or trabecular extension. Subaortic obstruction was constant. The VSD was severely distant from both the aortic and the pulmonary annulus. The operation was conducted through a combined approach. The VSD was constantly enlarged superiorly. The almost permanent subaortic obstruction was released. The VSD was always found quite close to the pulmonary infundibular ostium. The arterial switch technique was adapted to the complex coronary anatomy. Results: There was one non-cardiac death. At a mean follow-up of 20 months, all nine survivors are in NYHA class I, in sinus rhythm, and have no subaortic gradient greater than 15 mm. Conclusion: This technique of VSD rerouting to the pulmonary artery and arterial switch limits greatly the size of the rerouting patch, respects the tricuspid chordae and is independent of the pulmonary artery–tricuspid valve distance. In this early series of biventricular repair of DORVncVSD, the VSDs were always found close to the pulmonary artery, allowing this new type of repair.     

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.     

右心室双出口的手术治疗

目的　总结右心室双出口手术治疗的早期和远期结果及经验。　方法　 1985年 5月～ 2 0 0 1年 12月 4 1例右心室双出口患者接受了外科治疗 ,其中男 2 6例、女 15例 ,年龄 10个月～ 2 1岁 ,平均 (10± 6 )岁 ,体重 6 5～ 6 5 0kg ,平均 (2 6± 16 )kg。 4 1例中 ,14例行经典的Rastelli手术 (内隧道外管道手术 ) ;11例行经内隧道成形左心室流出道、右心室流出道直接疏通或用心包片加宽 ;11例行改良Rastelli手术 ;2例行全腔静脉肺动脉吻合术 ;2例行双向格林手术 ;1例行大动脉调转术。结果　早期 (术后 1个月 )死亡 1例 ,出院 4 0例。随访 36例 ,随访 5个月～ 17年 ,中位数为 7年 ,无死亡 ,二次手术 3例。所有患者紫绀消失 ,症状明显改善 ,心功能Ⅰ级 34例 ,心功能Ⅱ级 2例 ,能够进行正常的生活、学习和工作。　结论　绝大多数右心室双出口患者经双心室矫治可以取得很好的早期及远期结果 ;对于合并其他严重畸形的患者可行右心室旁路手术。     

Reoperation of Blalock-Taussig shunt for double outlet right ventricle and pulmonary atresia in an adult

A 36-year-old man was admitted to our hospital due to dyspnea and pneumonia. He had undergone left classical Blalock-Taussig shunt at 5 years old and right modified Blalock-Taussig shunt at 16 years old, because he was judged as no indication for any radical operation. Since the last surgery, he had stopped visiting hospital. Computed tomography showed pneumonia and pulmonary embolism in the left lower lung and occlusion of the right modified Blalock-Taussig shunt. After pneumonia was improved by antibiotic treatment, he underwent reoperation of the right modified Blalock-Taussig shunt using 8 mm knitted Dacron graft. Postoperative course was uneventful and dyspnea and cyanosys were improved. We successfully treated a complicated case of an adult congenital heart disease by reoperation of the right modified Blalock-Taussig shunt.     

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).     

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.