永存第五弓残存伴狭窄合并主动脉弓中断的外科治疗

目的总结永存第五弓残存伴狭窄合并主动脉弓中断的外科治疗经验,以提高手术疗效。方法自2000年1月至2008年5月,共手术治疗永存第五弓残存伴狭窄合并主动脉弓中断5例,手术年龄1．8～108．0个月,体重3．7～31．0kg。3例患者术前存在慢性心功能不全及反复呼吸道感染,5例患者均一期纠治第五弓狭窄／及合并的心血管畸形。结果手术死亡2例,1例术后并发心功能不全,肺动脉高压危象,严重肺部感染,依赖呼吸机,撤机困难,家属放弃治疗;另1例合并室间隔缺损,肺动脉高压,死于反应性肺动脉高压危象、低心排血量综合征和左心功能衰竭。术后随访3例,随访时间55．67±48．64个月,随访期间无死亡,无并发症发生。其中1例患者已随访8年,恢复正常学习,心脏磁共振成像（MRI）提示：第五弓与降主动脉连接处（Gore—Tex补片扩大修补处）轻度狭窄,直径9．3mm。结论永存第五弓残存伴狭窄合并主动脉弓中断患者手术采用胸骨正中切口径路,操作简便,暴露清楚,剖面小,有利于术后恢复。由于该病晚期亦出现体动脉高压,应尽早明确诊断及时手术治疗。     

同种主动脉治疗复杂先天性心脏病

目的　总结用同种带瓣主动脉和同种主动脉治疗 11例复杂先天性心脏病的经验。　方法　对 11例法洛四联症合并肺动脉闭锁和动脉导管未闭 ,矫正型大动脉转位合并室间隔缺损和肺动脉狭窄 ,完全型大动脉转位合并室间隔缺损和肺动脉狭窄 ,右心室双出口合并完全性房室通道 , 型永存动脉干 ,主动脉弓中断合并动脉导管未闭 ,主动脉缩窄合并动脉导管未闭和二尖瓣关闭不全患者施行了手术治疗 ;其中 Rastelli手术 7例 ,全腔静脉与肺动脉连接 (TCPC) 2例 ,升主动脉至降主动脉旁路移植 2例。　结果　手术死亡 1例 ;慢性心力衰竭 1例 ,内科治疗 1年后心功能 级。随访 1个月～ 7.2年 ,无远期死亡。　结论　同种主动脉是矫正复杂先天性心脏病的理想材料     

儿童永存动脉干的外科治疗

目的　总结儿童永存动脉干外科治疗经验。　方法　近９ 年来共收治永存动脉干５例,年龄３～１２ 岁,平均６－３ 岁。Ⅰ型２ 例（肺动脉均起于动脉干远侧）,Ⅱ型２ 例, Ⅳ型１ 例。肺循环时间和肺动脉排空时间均无明显延长。手术在体外循环心内直视下进行,室间隔缺损（ＶＳＤ） 补片缝合于动脉干瓣环并使其环缩。右心外管道采用缝制自体新鲜心包瓣涤纶人工血管４ 例,同种异体主动脉１ 例。　结果　术毕肺动脉压均明显下降,跨外管道压差７～３５ ｍｍ Ｈｇ。２ 例术后早期死亡与肺动脉病变无关。存活３ 例中１ 例并发低心排出量和呼吸功能不全,２ 例术后恢复顺利。分别随访１１４、９６ 和３４ 个月,心功能均为Ⅰ级,１ 例术后８ 年死于外管道梗阻。　结论　儿童永存动脉干仍可有手术指征; 带自体心包瓣人工血管远期效果良好; 纠正动脉干瓣关闭不全,防止ＶＳＤ 残余漏,避免过大的跨外管道压差和缩短手术时间,可提高手术成功率和远期效果     

主动脉弓中断合并室间隔缺损及肺动脉高压的一期手术矫治

目的 总结主动脉弓中断合并室间隔缺损及肺动脉高压的一期手术矫治经验.方法 2003年2月至2007年8月,一期手术矫治主动脉弓中断合并室间隔缺损及肺动脉高压病婴6例,男、女各3例.手术年龄18 d～14个月,平均(8.3±4.5)个月;平均体重(8.5 ±2.2)kg.A型4例,B型2例.所有病例术前均有反复呼吸道感染史.手术均采用胸骨正中切口.结果 全组病例均生存.随访3～36个月,恢复良好.结论 一期手术矫治主动脉弓中断合并室间隔缺损及肺动脉高压效果良好,需同时加强对围术期肺动脉高压的管理.     

一期手术矫治主动脉弓中断及伴发畸形

目的总结主动脉弓中断(IAA)及伴发畸形的手术治疗经验。方法自2000年1月至2005年12月我科共收治48例IAA及伴发畸形患者(平均年龄1.1岁、平均体重7.0kg),合并畸形有室间隔缺损23例,完全性大动脉错位2例,主-肺动脉窗伴右肺动脉起源于主动脉3例,第5主动脉弓残存狭窄2例,右心室双出口2例,永存动脉干2例,迷走右锁骨下动脉伴降主动脉发育不良1例。48例中35例接受手术纠治,其中34例IAA及伴发畸形一期解剖纠治,1例伴右心室双出口患者行主动脉弓吻合及肺动脉环缩术纠治。结果手术死亡4例。术后26例随访3个月~4年,死亡2例。术后发生并发症7例,Ⅲ°房室传导阻滞和膈神经麻痹各1例,轻度吻合口梗阻5例,其余患者无残余解剖问题,心功能良好。结论在新生儿期施行IAA一期矫治手术成功率高,围术期合适的干预是治疗成功的关键;由于伴发畸形复杂,远期生存率不容乐观。     

永存动脉干的外科治疗

永存动脉干的外科治疗顾承雄周其文王盛宇陈长城杨俊峰永存动脉干较少见，占先天性心脏病１％～４％。１９９２年７月至１９９６年７月，我们共收治这类病儿３例，现报告如下：临床资料本组病儿均为男性，年龄４岁。术前都有明显发绀，经超声心动图和右心室造影检查确定Ｉ...     

主动脉弓中断二例

例１　女,３岁６个月。活动后心悸、气促２年。血压１２／８ｋＰａ（９０／６０ｍｍＨｇ）,胸骨左缘第３、４肋间可闻及Ⅲ级收缩期杂音（ＳＭ）,肺动脉瓣第二心音（Ｐ２）亢进。胸部Ｘ线片示肺动脉段突出,主动脉结不清晰;Ｂ型超声波示室间隔缺损（ＶＳＤ）,双向分流;右心导管提示降主动脉血氧分压与主肺动脉相同,导管可经肺动脉进入降主动脉。术前诊断为ＶＳＤ,动脉导管未闭（ＰＤＡ）合并严重肺动脉高压（ＰＨ）。因术前误诊,手术选用正中切口,常规主动脉、腔静脉插管。术中探查发现合并主动脉弓中断（ＩＡＡ）。将肺动脉前壁裁…     

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。     

完全性大血管错位和右心室双出口肺动脉瓣下室间隔缺损伴主动脉弓病变的一期矫治手术

目的评估一期纠治完全性大血管错位(TGA)和右心室双出口肺动脉瓣下室间隔缺损(Taussig-B ing)伴主动脉弓病变的手术疗效。方法2001年1月—2004年6月对8例伴主动脉弓病变的TGA(3例)和Taussig-B ing(5例)行一期手术治疗。3例TGA中,室间隔完整型1例,伴室间隔缺损2例;主动脉弓病变为7例主动脉缩窄、1例主动脉弓中断。手术年龄1例为8个月,7例为5 d~3个月,平均40 d,体重3.5~6.3 kg,平均(4.3±0.5)kg。均采用胸骨正中切口。手术先在深低温、停循环下矫治主动脉弓病变,然后在深低温、低流量下行大动脉转换术(Sw itch术)。体外循环转流时间107~159 m in,平均(126±23)m in,主动脉阻断时间63~118 m in,平均(92±16)m in,停循环14~45 m in,平均(30±12)m in。结果手术死亡1例,为8个月Taussig-B ing伴主动脉弓发育不良、冠状动脉畸形患儿,术后因低心排血综合征、Ⅲ度房室传导阻滞、肺高压危象死亡;1例3月龄患儿术后5 d喂奶时窒息死亡。6例随访5个月~2年,生长发育良好,1例Taussig-B ing主动脉弓中断出现吻合口狭窄,压差60 mm Hg;2例出现主动脉瓣轻微返流,1例肺动脉瓣轻度返流。结论一期纠治TGA和Taussig-B ing伴主动脉弓病变能取得较好手术效果,手术死亡原因为肺动脉高压和冠状动脉畸形。     

一期手术纠治主-肺动脉窗及伴发畸形

目的总结一期手术纠治主－肺动脉窗及伴发畸形的临床经验，以提高手术疗效。方法我科共收治26例主－肺动脉窗患者．男14例．女12例；年龄1．4±1．6岁；体重7．8±3．8kg。其中单纯主－肺动脉窗8例，合并主动脉弓中断、右肺动脉异常起源于主动脉、法洛四联症、房间隔缺损、室间隔缺损、二尖瓣反流和气管狭窄等18例。25例患者一期手术经主动脉切口补片关闭瘘口纠治主－肺动脉窗，同时纠治伴发的畸形；1例放弃治疗。结果全组无手术死亡。2例伴主动脉弓中断、动脉导管未闭和右肺动脉起源于升主动脉的新生儿术后3d延迟关胸；1例术后出血，3h后再次开胸止血。术后随访22例．随访时间1个月～4年。所有患者无明显的主动脉瓣上狭窄和肺动脉分支狭窄，2例残留轻度二尖瓣反流，1例残留轻至中度二尖瓣反流。结论主－肺动脉窗患者早期易发生肺动脉高压，一经诊断应立即手术。手术方式首选修补主－肺动脉窗及一期纠治伴发畸形。尽管主－肺动脉窗可合并各种心内外畸形，但早期手术纠治可获得较好的中长期疗效。     

主动脉弓中断的外科治疗

目的 总结主动脉弓中断及合并心脏畸形的外科治疗经验.方法 1997年1月至2008年1月,36例主动脉弓中断患者进行了外科手术治疗,其中男性22例,女性14例.儿童患者36例,年龄2个月～7岁,平均年龄2.8岁.成人患者1例,年龄31岁.33例合并心内畸形,其中31例正中开胸同时矫治主动脉弓中断和心内畸形;1例左侧切口矫治主动脉弓中断,正中开胸修补心内畸形;1例采用姑息手术.3例无心内畸形的患者2例采用左后外侧切口,1例采用正中胸部加上腹部切口.术式包括16例管道连接,9例直接吻合,9例直接吻合并补片成形,1例应用左锁骨下动脉翻转.在31例正中切口一期手术中,17例应用选择性脑灌注加下半身停循环,8例采用深低温低流量灌注,6例采用全身停循环.结果 住院死亡5例,3例死于肺部感染,1例死于肺动脉高压危象,1例死于术后低心排血量.术后早期有其他重要并发症7例.31例存活患者随访3个月～5年,无远期死亡,无需要再次手术的病例.结论 合并心内畸形的主动脉弓中断患者可采取选择性脑灌注加下半身停循环或深低温全身低流量下正中一期手术同时矫治.     

小儿永存动脉干外科诊治19例

目的 总结19例小儿永存动脉干(PTA)外科治疗的经验。方法 1989年1月至2002年6月外科手术纠治19例PTA。病儿2月龄～5岁；体重3．5—16．0kg。按Van Praagh分型，A1型8例，A2型9例，A3及A4型各1例。右室-肺动脉流出道重建用涤纶生物瓣管道和同种带瓣肺动脉各2例，同种带瓣主动脉11例，近1年的4例未用外管道。结果 手术死亡率10．5％(2，19例)。晚期死亡1例。结论 PTA易早期并发肺血管梗阻性疾病，应在1岁以内行纠治术。手术需防止室间隔缺损残余分流；离断肺动脉和修复动脉干缺损时，应避免损伤动脉干、瓣膜及冠状动脉口；如条件许可，A1型及A2型可不用外管道重建右室-肺动脉流出道，避免了因更换管道而再次手术。     

经胸骨正中切口一期矫治主动脉缩窄或主动脉弓中断合并心内畸形

目的 总结经胸骨正中切口一期矫治主动脉缩窄或弓中断合并心内畸形的经验.方法 2007年1月-2008年7月手术治疗24例.包括主动脉缩窄9例,主动脉缩窄合并主动脉弓发育不良12例,主动脉弓中断3例.4例合并右室双出口(Traussig-Bing型).22例合并非限制性室间隔缺损,2例不合并室间隔缺损病儿1例合并主动脉瓣下狭窄,另l例合并肺静脉狭窄.主动脉弓降部成形均在深低温低流量持续性选择性脑灌注下进行.3例主动脉弓中断及9例主动脉缩窄病儿采用端端吻合术.12例主动脉缩窄合并主动脉弓发育不良病儿中采用扩大端端吻合术8例,端侧吻合术2例,补片成形术2例.结果 死亡2例.全组病儿围术期未出现神经系统并发症及肾功能损害.术后反复呼吸道感染2例.除l例残存压差大于20mm Hg外,最长随访18个月,尚未发现再缩窄发生.结论 主动脉缩窄或弓中断合并心内畸形一经诊断即需尽早手术.经胸骨正中切口一期矫治是安全、有效的.充分切除动脉导管组织,广泛彻底游离松解胸部各血管进行无张力吻合以及选择恰当的组织一组织吻合术式是主动脉弓降部成形手术成功及减少再缩窄发生的关键.     

Staged repair of interrupted aortic arch with ventricular septal defect compared with primary repair in infancy

Interrupted aortic arch is a poor prognosis cardiac anomaly with nearly 100 percent mortality if not recognized and treated early. The associated intracardiac lesions often lead to death if only the arch defect is repaired. Several recent reports have described patients with interrupted aortic arch who were treated as infants by primary repair of the arch defect with simultaneous repair of the intracardiac lesions. The improved survival data from these series have been attributed to the simultaneous repair of both lesions. We report herein on nine patients with both interrupted aortic arch and ventricular septal defect seen at Children's Hospital and Medical Center in Seattle from 1979 to 1987. Three patients had partial expression of DiGeorge's syndrome. All patients underwent primary repair of the interrupted aortic arch with concomitant pulmonary artery banding during infancy (mean age 18 days, range 2 days to 4 months). Operative mortality was 11 percent (1 of 9 patients). Eight patients had eventual repair of the ventricular septal defect (mean age 18 months, range 6 to 29 months) with one death occurring at 5 months postoperatively (12 percent mortality). The overall mortality of these nine patients was 22 percent. Staged repair of interrupted aortic arch with associated ventricular septal defect can be performed with results comparable to simultaneous primary repair in infancy. The improved survival from either approach is more likely to be attributable to improved perioperative stabilization, particularly the use of prostaglandin E.     

Repair of persistent truncus arteriosus with interrupted aortic arch.

OBJECTIVE: The aim of our study was to analyse experience with repair of truncus arteriosus with interrupted aortic arch. METHODS: Between 1993 and 2004, eight consecutive patients underwent repair of truncus arteriosus with interrupted aortic arch. The median age was 6.5 days (range 1-85 days) and median weight was 3.2 kg (range 2.6-4.8 kg). Five patients had type A and 3 patients had type B aortic arch interruption. The repair was performed in deep hypothermia with circulatory arrest in 4 patients and isolated selective low-flow perfusion of the head and the heart in the last 4 patients. The repair consisted in aortic arch reconstruction by direct anastomosis between descending and ascending aorta, closure of ventricular septal defect and reconstruction of the right ventricular to pulmonary artery continuity using a valved conduit. RESULTS: One (12.5%) patient died from sepsis and hepato-renal failure 18 days after surgery. Seven (87.5%) patients were followed up for 2.0-11.7 years (median 2.6 years). No patient died after the discharge from hospital. In 4 patients 1-3 reinterventions were required 0.6-10.0 years after repair. Reoperations were performed for conduit obstruction in 2 patients, aortic regurgitation in 2 patients, right pulmonary artery stenosis in 2 patients and airway obstruction in 1 patient. In 2 patients concommitant aortic valve and conduit replacement was required. Balloon angioplasty for aortic arch obstruction was necessary in 1 patient, and for bilateral pulmonary branch stenosis in 1 patient. Five (28.6%) surviving patients are in NYHA class I and 2 (71.4%) patients are in NYHA class II. CONCLUSIONS: Primary repair of persistent truncus arteriosus with interrupted aortic arch can be done with low mortality and good mid-term results. Aortic arch reconstruction in isolated low-flow perfusion of the head and the heart influences favourably the postoperative recovery. The main postoperative problems are associated with conduit obstruction and aortic insufficiency.     

Repair of truncus arteriosus in infancy

Improvements in myocardial protection, surgical technique, and postoperative care have decreased operative mortality for neonatal repair of truncus arteriosus. Primary repair of truncus arteriosus in infancy without prior pulmonary artery banding is currently the preferred approach. During the period from 1982 to December 1990, 32 patients under the age of 12 months underwent surgical correction of truncus arteriosus at UCLA. The average age was 3.5 months (range, 12 days to 12 months). Three patients had interrupted aortic arch. Early mortality for the entire group was 15.6% (5/32); for those older than 1 month early mortality was 7% (2/28). In the past 4 years, early mortality has decreased to 8.3% (2/24); both of these patients had interrupted aortic arch. Excluding patients with interrupted aortic arch, there were no early deaths in the last 22 patients (1986 to 1990). Late mortality overall was 7.4% (2/27). In a mean follow-up of 73 months (range, 40 to 110 months), 71% (5/7) of the survivors with Dacron porcine-valved conduits required conduit replacement secondary to obstruction. In a mean follow-up of 36 months (range, 1 to 89 months), only 14% (3/21) of the patients with homografts required replacement secondary to obstruction.     

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.     

Primary Tumors of the Thoracoabdominal Aorta: Surgical Treatment of 5 Patients and Review of the Literature

The objectives of this study were to describe five cases involving primary tumors of the thoracoabdominal aorta and to review the pertinent literature. Between April 1990 and April 2000, we performed surgery on five patients with primary tumors of the aorta (PTA). There were three men and two women ranging in age from 37 to 65 years (mean, 49.8 years). The presenting manifestations were renovascular hypertension in four cases, including three associated with abdominal angina and lower extremity embolism in one case. In all patients aortograms identified atherosclerotic-like occlusive lesions in the thoracoabdominal aorta extending to the descending thoracic aorta in three cases, visceral arteries in four cases, and infrarenal aorta in one case. Preoperative histological diagnosis of PTA was achieved in two patients following open repair with placement of an aortoaortic graft in one case and peripheral embolectomy in one case. In two cases, diagnosis of PTA was strongly suspected before or during the procedure. In the remaining case, diagnosis was not achieved until the definitive histological report. In two patients surgical treatment was carried out with curative intent (aortic resection with graft replacement). In two cases surgical treatment was incomplete (endarterectomy of the aorta and visceral arteries). In the remaining case surgical treatment was purely palliative (aortic and superior mesenteric artery bypass). Histological findings demonstrated intimal-type sarcoma in two cases, leiomyosarcoma in one case, and angiosarcoma in one case. In the remaining case, histological analysis was unfeasible for technical reasons. One patient died due to massive cerebral embolism 2 days after surgical treatment involving revascularization of the aortic arch carried out with hypothermic circulatory arrest. One patient developed secondary paraplegia. All four patients who survived the immediate postoperative period died of tumor-related complications and cachexia at 5, 7, 16, and 24 months after the initial surgical procedure. The results of this small series as well as those of 130 previously reported cases confirm the extremely dismal prognosis of PTA. Mean overall survival for patients presenting PTA was less than 16 months. Survival at 5 years was 8%. Survival rates appear to be higher after surgical treatment and were significantly improved by adjuvant chemotherapy. The main factors correlated with poor prognosis were intimal type, involvement of the ascending aorta, aortic arch, or visceral aorta, and incomplete resection. Presented at the Seventeenth Annual Meeting of the Société de Chirurgie Vasculaire de Langue Française, May 29-31, 2002, Liege, Belgium.     

Primary definitive repair of interrupted aortic arch with ventricular septal defect

The optimal surgical management (primary or staged repair) of interrupted aortic arch (IAA) with ventricular septal defect (VSD) remains to be determined. A consecutive series of 14 neonates, aged 3-18 days (mean: 10 +/- 6 days) underwent primary complete repair. Mean weight was 3.3 +/- 0.4 kg. Eleven patients had IAA type B, 2 had type A and 1 had type C. Six infants had the Di George syndrome. Preoperative management (mean: 5 +/- 4 days) included prostaglandin E1 (14/14), intubation and ventilation (13/14), and inotropic support (11/14). Surgery was performed under deep hypothermia and circulatory arrest and involved resection of all ductal tissue, direct end-to-side aortic arch anastomosis and patch closure of the VSD. There were 2 early deaths (14%, 70% CL: 5%-31%): low cardiac output (1), residual VSD (1). Four patients (33%, 70% CL: 13%-52%) underwent reoperation for recurrent aortic obstruction (3 patients, 1 death) or left ventricular outflow tract obstruction (LVOTO) (1 patient). The results improved with time: no death and no recurrent aortic obstruction in the last 8 patients. At last follow-up (11 patient, mean follow-up = 24 +/- 9 months), all patients were free of cardiac symptoms; none had persistent aortic obstruction; 4 had LVOTO (gradient greater than 20 mm Hg) and 1 (with the Di George syndrome) had severe mental disorders. Primary complete repair provides satisfactory results in most infants born with IAA and VSD. An adequate direct aortic arch anastomosis should entail a low risk of recurrent obstruction. LVOTO develops in many cases and may require further surgery.