20例双腔右心室外科治疗

报告手术治疗双腔右心室20例，合并室间隔缺损16例。均在体外循环下成功切除异常肌束，疏通右室梗阻修补合并室缺。认为手术中鉴别诊断对双腔右心室的疗效有决定意义，以右室流出道切口暴露右室操作方便，异常肌束切除应适度，疏通仍不满意者可采取用自体心包补片加宽右室流出道的方法予以解决。     

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 for double-outlet right ventricle

Double-outlet right ventricle (DORV) is a heart malformation that describes an anomalous ventriculoarterial connection which can be associated with considerable variant of associated lesions. When this malformation is present with two ventricles, biventricular repair is feasible in the vast majority of cases. This report describes the surgical techniques for biventricular repair in all forms of encountered DORV, as well as the surgical strategy employed at our institution. Copyright © 2000 by W.B. Saunders Company     

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.     

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     

A case report on a double chambered right ventricle (DCRV)--pre- and postoperative responses of the right ventricle to atrial pacing and the isoproterenol tolerance test

To evaluate the development of an anomalous muscle bandle of the right ventricle (RV) and results of surgical intervention, responses of the RV to atrial pacing and the isoproterenol tests were examined in both the pre- and postoperative periods. Although cardiac output did not increase in proportion to the rate of atrial pacing or the dose of isoproterenol, the pressure gradient through the RV increased abnormally from 38 mmHg to 59 mmHg (atrial pacing) and 116 mmHg (isoproterenol). On the other hand, postoperative evaluation showed normal RV responses in these tests. Since the response of the right ventricle to these tolerance tests in patients with DCRV in pre- and postoperative periods has not been well elucidated, a precise evaluation is reported in this paper.     

Transatrial repair of double-chambered right ventricle

Double-chambered right ventricles (DCRVCs) traditionally have been repaired via a right ventriculotomy. We describe the cases of 8 children (aged 7 months to 6 years; weight, 6.3 to 21.5 kg), who underwent repair of DCRV via a right atriotomy. Six patients had ventricular septal defects. Peak systolic gradients in the right ventricular outflow tract were 26 to 135 mm Hg. The QP/QS were 1.1 to 2.5. Cardiopulmonary bypass times were 52 to 89 min (mean, 65.5 min) with aortic cross-clamp times of 20 to 56 min (mean, 39 min). Mean intraoperative gradients following repair were 5.8 mm Hg. Three patients had transient postoperative atrioventricular block requiring temporary pacing. Two patients required minimal inotropic support (dopamine, 2.5 to 5 micrograms/kg/min). The children have all been asymptomatic after hospitalization with follow-up times of 5 months to 4.5 years. No patient requires cardiac medications or has significant, persistent dysrhythmias; however, 1 patient required reoperation. Transatrial repair of DCRV allows excellent operative anatomical visualization and should be considered as an alternative to the transventricular approach in patients with this congenital heart defect.     

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。     

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。     

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.     

A study of sixty cases of tetanus

Sixty cases of tetanus with a mortality of 46.6 per cent are presented. Prophylactic antitoxin did not prevent tetanus in 5 per cent of the cases. Patients receiving some surgical treatment of the infected wounds were four times as likely to recover as those not receiving any surgical care. The immediate administration of 50,000 units of antitoxin intravenously and the simultaneous administration of toxoid at five day intervals for two doses would maintain the blood antitoxin level at an adequate level.     

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.