先天性主动脉弓中断及合并畸形的一期手术治疗

目的回顾总结先天性主动脉弓中断及合并畸形一期手术治疗经验。方法1988年7月至2004年8月,手术治疗主动脉弓中断及合并畸形20例。男12例,女8例;手术年龄18d～9岁。其中<1岁者10例,平均年龄(3.37±2.45)个月,平均体重(5.33±1.72)kg;>1岁者10例,平均年龄(2.60±1.24)岁,平均体重(13.10±6.53)kg。所有病儿术前均有反复呼吸道感染史。A型15例,B型5例。2例经双切口手术,即先通过左后外侧切口纠治主动脉弓中断,再经正中切口纠治合并畸形。18例采用正中切口同时纠治主动脉弓中断和合并畸形。结果全组死亡3例,死于术后严重肺动脉高压危象2例、严重心功能衰竭1例。17例随访3个月～12年,均恢复良好。结论主动脉弓中断采用正中切口方法,操作简便,暴露清楚,创伤小,有利于术后恢复。本病早期即出现肺动脉高压,临床上一经确诊宜尽早手术。     

主动脉缩窄或主动脉弓中断合并心内畸形一期修复

目的 探讨采用经胸骨正中切口一期修复主动脉缩窄(CoA)或主动脉弓中断(IAA)合并心内畸形的治疗效果.方法 2002年7月至2009年6月,经胸骨正中切口行降主动脉远端和主动脉弓下缘端侧吻合术一期修复CoA或IAA合并心内畸形病儿43例,其中CoA 34例,IAA 9例(A型6例、B型3例),合并心内畸形包括室间隔缺损42例、动脉导管未闭34例、房间隔缺损12例、主动脉瓣下隔膜狭窄5例、二尖瓣关闭不全2例,右心室双出口1例.结果 手术死亡1例,为术后肺动脉高压和严重低心排血量综合征者.术后并发症包括严重低心排血量综合征3例,低氧血症6例,肺部炎症11例,肺不张14例,声音嘶哑19例,室上性心动过速23例.8例失访.34例随访3个月～5年,生活质量明显改善,心脏超声心动图和CT检查显示吻合口无明显再缩窄发生.结论 经胸骨正中切口,采用主动脉远端和主动脉弓下缘端侧吻合技术一期修复CoA或IAA合并心内畸形的手术早、中期效果良好,能明显减少术后再狭窄.     

经胸骨正中切口一期矫治小儿主动脉缩窄及合并畸形

目的探讨经胸骨正中切口一期矫治小儿主动脉缩窄(CoA)及合并畸形的手术方法和治疗效果。方法2002年6月至2005年8月手术治疗CoA及合并畸形36例,其中男27例,女9例;年龄18d~8岁,平均(14·3±9·5)个月,其中小于1岁27例,小于3月龄18例;体重3~23kg,平均(6·6±3·0)kg。有症状的婴儿型27例,无症状的大儿童型8例,经左胸切口矫治CoA后再狭窄1例;合并室间隔缺损、动脉导管未闭、房间隔缺损、主动脉弓发育不良、完全性大动脉转位及主动脉瓣下狭窄等心内畸形。36例均经胸骨正中切口以扩展端端吻合术一期矫治CoA及合并畸形。结果手术死亡1例,系术前反复心力衰竭及充血性肺炎,术后15d死于心肺功能衰竭。35例随访4~36个月,晚期死亡1例。生存34例恢复良好,无再缩窄发生。结论经胸骨正中切口一期矫治CoA及合并畸形效果良好;扩展端端吻合法是一种值得推荐的技术。     

自体肺动脉组织一期矫治主动脉弓病变合并心内畸形

目的 探讨自体肺动脉组织一期矫治主动脉弓病变合并心内畸形的手术方法和治疗效果.方法 1993年4月至2009年11月,共25例主动脉弓病变合并心内畸形的患儿接受了一期矫治手术.男性17例,女性8例;年龄26 d～6.5岁,平均11个月;体质量3.0～14.5 kg,平均12.4 kg.其中6例患儿为主动脉弓缩窄,6例为主动脉弓中断,13例为主动脉弓发育不良.手术技术包括切开肺动脉前壁,切除动脉导管,应用部分自体肺动脉壁重建主动脉弓,重建肺动脉,并同时矫治合并心内畸形.结果 24例患儿术后恢复顺利,1例患儿死于围手术期肺动脉高压危象.随访心脏超声及心脏CT检查证实主动脉弓重建及心内畸形矫治满意.术后未发生神经系统及其他并发症.随访过程中,患儿生长发育正常,主动脉弓无再狭窄.结论 自体肺动脉具有生长潜力且柔韧性好,是一种理想的重建主动脉弓的材料.一期矫治手术治疗先天性主动脉弓病变合并心内畸形可以得到满意的结果.     

One-stage repair of absence of the aortopulmonary septum and interrupted aortic arch

Absence of the aortopulmonary septum, interrupted aortic arch, aortic origin of the right pulmonary artery, intact ventricular septum, and patent ductus arteriosus is a rarely reported association. A 3-year-old boy underwent successful one-stage repair of this constellation of anomalies. A Dacron baffle was used both to close the huge aortopulmonary window and to direct blood to the right pulmonary artery. Type A interrupted aortic arch was repaired by direct anastomosis. Postoperatively, pulmonary artery pressure was less than half systemic pressure.     

One-stage total repair of aortic arch anomaly using regional perfusion.

OBJECTIVE: Primary repair of aortic arch obstructions and associated cardiac anomalies is a surgical challenge in neonates and infants. Deep hypothermic circulatory arrest prolongs myocardial ischemia and might induce cerebral and myocardial dysfunction. METHODS: From March 2000 to December 2005, 69 neonates or infants with aortic arch anomaly underwent one-stage biventricular repair with continuous cerebral perfusion in the presence of a nonworking beating heart using the dual perfusion technique on the innominate artery and aortic root. Preoperative diagnoses of arch anomaly comprised aortic coarctation (n=54) or an interrupted aortic arch (n=15). Combined anomalies were ventricular septal defect (n=52), anomalous origin of the right pulmonary artery from ascending aorta (n=3), hypoplastic left heart syndrome (n=2), truncus arteriosus (n=2), atrioventricular septal defect (n=2), double outlet right ventricle (n=1), total anomalous pulmonary venous return (n=1), partial anomalous pulmonary venous return (n=1), and aortic stenosis (n=1). RESULTS: The mean regional perfusion time was 27.8+/-9.8 min. There was no operative mortality. Postoperative low cardiac output was present in four patients (5.8%). A neurologic complication was noted in one patient (1.5%) who developed transient chorea, but recovered completely. During 32.8+/-17.5 months of follow-up, one late death (1.5%) occurred. There was neither reoperation associated with arch anomaly nor recoarctation except in one patient. One patient developed left main bronchial compression necessitating aortopexy. CONCLUSIONS: One-stage total arch repair using our regional perfusion technique is an excellent method that may minimize neurologic and myocardial complications without mortality. Our surgical strategy for arch anomaly has a low rate of residual and recurrent coarctation when performed in neonates and infants.     

未行尿液转流一期修复中段型和后型尿道下裂

目的评价未行尿液转流一期修复中段型和后型尿道下裂的效果,总结手术经验。方法166例中段型和后型尿道下裂患者,其中阴茎型94例,阴茎阴囊型59例,阴囊型13例,平均尿道缺损长度(4.49±1.93)cm。166例患者运用不同术式一期修复尿道下裂,其中横行带蒂包皮瓣法(Duckett术)79例,阴囊纵隔皮瓣法83例,原位阴囊皮管加横行带蒂包皮瓣法(Duckett+Duplay术)4例,所有患者均未行尿液转流。结果166例患者术后随访2~17个月,平均9.1个月。其中,治愈142例(85.5%),术后发生尿瘘15例(9.0%),尿道狭窄5例(3.0%),尿道憩室1例(0.6%),尿道毛石形成2例(1.2%),阴茎残余轻度下曲畸形1例(0.6%)。结论未行尿液转流一期修复型尿道下裂的效果良好,并发症发生率低,可运用于中段型和后型尿道下裂。     

Arch reconstruction without circulatory arrest: Scientific basis for continued use and application to patients with arch anomalies

Aortic arch hypoplasia is a common constituent of congenital heart disease. While repair of these lesions has been performed routinely during deep hypothermia and circulatory arrest, new approaches are emerging. One such approach, regional low-flow perfusion, will be described here. This technique exploits the anticipated modified Blalock-Taussig shunt as a perfusion conduit. With control of the brachiocephalic vessels and the descending thoracic aorta, circulatory support can be provided to the neonate with exposure identical to that obtained by circulatory arrest. While first applied to children undergoing the Norwood operation for hypoplastic left heart syndrome, this technique has recently been applied to children requiring complex arch surgery in the setting of biventricular repair. To date, 36 neonates requiring arch reconstruction (27 Norwood operations, 9 biventricular repairs) have been supported with regional low-flow perfusion. Thirty-day and hospital discharge survival has been 74% (20/27) for neonates undergoing Norwood operation, and 88% (8/9) for those undergoing biventricular repair. We will review the operative technique, methodologies, and clinical studies that led us to conclude that regional low-flow perfusion provides cerebral, as well as somatic, circulatory support to the neonate undergoing arch reconstruction. Copyright © 2002 by W.B. Saunders Company     

One stage repair of aortic arch anomalies and intracardiac defects

Between August 1985 and May 1990, 27 neonates and infants underwent combined correction of intracardiac and aortic arch anomalies through a median sternotomy. Coarctation (CoA) was combined with VSD (6), AVSD (2), Taussig-Bing (TB) heart (5), transposition of the great arteries (TGA) (1), TGA + VSD (2), congenitally corrected TGA + VSD (1) and VSD + myxoid stenotic outlet valves (1). Interrupted aortic arch (IAA) was combined with VSD (10) and TB heart (1). Two patients had IAA type B as well as CoA. Age at operation varied from 2 to 243 days with a mean age of 51 days. Twenty patients (70%) were younger than 30 days. One TGA + VSD and all TB hearts had maligned outlet septum and right ventricular outflow tract obstruction (RVOTO). Posterior outlet septum deviation and left ventricular outflow tract obstruction (LVOTO) occurred in 8 patients with malalignment VSD and IAA (7) or CoA (1). Aortic arch reconstruction was performed using extended end-to-end anastomoses. In 3 patients, arch hypoplasia necessitated patch implantation. VSDs were closed through the right atrium unless the anatomy dictated otherwise. One TB heart was reconstructed with a Kawashima procedure. All other TB hearts and all TGAs were corrected with arterial switch operation. Obstructing outlet septum was resected whenever necessary. Follow-up was complete and included echo-Doppler control. Eleven patients had postoperative heart catheterisation. Early mortality was 18.5% (5 patients). Persisting LVOTO or RVOTO was responsible. There was no late mortality. Five patients were reoperated upon: 3 for stenotic anastomoses and 2 for a subaortic membranous stenosis. Successful balloon dilatation of recoarctation was performed four times.(ABSTRACT TRUNCATED AT 250 WORDS)     

Totally normothermic aortic arch replacement without circulatory arrest

The authors propose a new strategy of normothermic perfusion for replacement of the aortic arch to avoid the complications of profound hypothermic circulatory arrest. Six patients underwent complete replacement of the aortic arch under normothermia using two pumps for the body (one for the brain and the thoracoabdominal aortic branches) and one for the heart. The surgical procedure was performed with no time limit. There were no operative or late deaths. No patients had neurologic deficit and all were rapidly extubated with uneventful postoperative courses. The method preserves autoregulation of cerebral blood flow and maintains body perfusion without high vascular resistances.     

An alternative approach to diffuse thoracic aortomegaly: on-pump hybrid total arch repair without circulatory arrest

Diffuse thoracic aortomegaly has conventionally been managed with a two-stage elephant trunk procedure, requiring prolonged circulatory arrest, with an inherent risk of major morbidity and mortality. Recently, to improve outcomes, several hybrid arch procedures have been proposed using off-pump techniques. We have adopted an alternative, single-stage hybrid strategy using cardiopulmonary bypass without circulatory arrest to replace the ascending aorta and perform arch debranching and antegrade endovascular stent graft deployment. Unlike off-pump procedures, pathology of the aortic valve, root, and ascending aorta is addressed while avoiding the complications of stent graft placement in the native ascending aorta.     

Total aortic arch aneurysm repair using a stent graft without cardiac or cerebral ischemia

A 74-year-old man with an aortic arch aneurysm and a chronic type IIIb aortic dissection underwent total aortic arch repair without cerebral or cardiac ischemia. After confirming no atheromatous change in the ascending aortic wall, a custom-designed 4-limbed graft, prepared for both arterial return of cardiopulmonary bypass and reconstruction of the arch vessels, was anastomosed onto the right side of the ascending aorta. The 3 arch vessels were then bypassed sequentially during systemic cooling and monitoring cerebral perfusion with near-infrared oxymetry. After aortic cross-clamping, a stent graft was inserted into the distal arch from the distal ascending aorta, maintaining cerebral and cardiac perfusion. This procedure is indicated especially in a high-risk patient who has an aortic arch aneurysm without severe atheromatous change in the ascending aorta and the arch vessels.     

A severe respiratory distress in a new born infant with double aortic arch without cardiac anomalies

A 39-day-old girl underwent operation for double aortic arch. She required endotracheal intubation at 23-day-old because of severe respiratory distress. Echocardiography, esophagography and angiocardiography revealed double aortic arch without other cardiac anomalies. At the operation the lesser arch and ductus arteriosus were divided. Soon after the operation her respiratory distress disappeared and fiberbronchoscopic examination showed rapid recovery of the deformed trachea.     

One-stage repair of interrupted aortic arch and aortopulmonary window with an autologous arterial flap

Interrupted aortic arch associated with an aortopulmonary window is a rare congenital malformation that requires an early diagnosis and surgical treatment to avoid irreversible pulmonary vascular changes. We describe herein successful one-stage repair in a neonate without the use of pericardium or other prosthetic material. Use of a transaortic approach permitted both accurate planning of the appropriate size of pulmonary trunk flap and also facilitated easy repair of the aortopulmonary septal defect. The growth potential of both great arteries was optimized.     

Totally normothermic aortic arch replacement without circulatory arrest.

BACKGROUND: Various techniques have been proposed for cerebral protection during the surgical treatment of complex aortic disease. The authors propose a revisited strategy of normothermic replacement of the aortic arch to avoid limitations and complications of profound hypothermic circulatory arrest. MATERIALS AND METHODS: From April 2000 to May 2006, 19 patients with an aneurysm of the aortic arch and 10 patients with an acute (7) or a chronic (3) aortic dissection underwent a totally normothermic, complete replacement of the aortic arch using three pumps: One pump ensured antegrade cerebral perfusion, at a flow rate adapted to obtain a pressure of 70 mmHg in the right radial artery, and required a selective cannulation of the supra-aortic vessels. A second pump ensured body perfusion at a flow rate adapted to obtain a pressure of 55 mmHg in the left femoral artery and was situated between the right femoral artery and the right atrium. A special balloon aortic occlusion catheter was placed in the descending thoracic aorta. A third pump ensured intermittent normothermic myocardial perfusion via the coronary venous sinus. The arch reconstruction was performed with no time limit. RESULTS: There were two operative, in-hospital (6.8%) mortalities. All others patients were rapidly extubated, except one, with no neurological sequelae, and postoperative course was uneventful, without coagulopathy or hepato-renal impairment. CONCLUSIONS: In the light of these results, a normothermic procedure is possible for arch surgery and may ensure a more physiological autoregulation of cerebral blood flow while maintaining body perfusion without high vascular resistances.