先天性主动脉缩窄合并心内畸形的外科矫治

目的 探讨先天性主动脉缩窄(CoA)合并心内畸形的外科治疗方法。方法 1994～2001年共收治45例CoA合并心内畸形患者,其中一期手术组26例,分期手术组19例。23例合并有中～重度肺动脉高压(平均肺动脉压56mmHg)：心内畸形以室间隔缺损(VSD)最常见(36例,80％),一期组选择单纯正中切口21例,左外侧和正中双切口5例。分期手术组平均间隔105d行二期手术矫正心内畸形。结果 两组分别死亡2例。24例术后上下肢动脉压差消失,10例平均动脉收缩压差均小于10mmHg平均随访29个月。患者生活质量有明显提高。超声心动图检查提示无假性动脉瘤或主动脉再狭窄。结论 选择一期或分期手术矫治CoA合并心内畸形,手术结果无明显差异,一期手术中选择左外侧和止中双切口方法安全有效。     

主动脉缩窄合并心内畸形的一期手术治疗

１９９３年６月至１９９４年８月为５例主动脉缩窄合并先天性心内畸形病儿施行了一期手术治疗。男４例，女１例，年龄１．５～１３岁，体重７．５～４５ｋｇ。除主动脉缩窄外，合并的心内畸形有室间隔缺损、主动脉瓣及瓣下狭窄、主动脉瓣关闭不全等。手术采用左后外侧第４肋间切口，矫治主动脉缩窄后，同期行胸骨正中切口体外循环下心内畸形矫治。本组无手术死亡和并发症，取得了满意的治疗效果。作者认为，对主动脉缩窄合并心内畸形施行一期手术矫治是完全可行的。     

一期手术矫治主动脉弓缩窄合并心内畸形

目的：探讨主动脉弓缩窄合并心内畸形病人的一期手术矫治方法及治疗效果。方法：自１９８９年１２月至１９９８年２月,运用一期手术方法为１０例主动脉弓缩窄合并心内畸形病人进行外科矫治。合并的心内畸形有室间隔缺损７例,二尖瓣关闭不全２例,主动脉瓣狭窄１例;６例同时合并动脉导管未闭。采用左后外侧切口矫治主动脉弓缩窄,正中切口行心内畸形矫治６例、正中切口采用主肺动脉内隧道同时矫治主动脉弓缩窄及合并心内畸形３例;     

主动脉缩窄合并心内畸形的治疗进展

主动脉缩窄 (coarctation ,CoA)占先天性心脏病的 5 %～10 % ,主要合并心内畸形包括室间隔缺损 (VSD)、房间隔缺损(ASD)、房室隔缺损 (AVSD)等。其中VSD发生率最高 ,可达5 5 % 〔1,2〕。目前对其治疗原则的争论主要在于手术方法和手术时机的选择。手术时机的选择目前 ,随着手术技术、体外循环及重症监护的进步 ,主动脉缩窄合并心内畸形的治疗效果已非常满意 ,但对一期根治或者二期纠治的选择仍存在很大争论〔3〕。1.分期手术的特点和结果传统的分期手术指在第一次手术时纠治主动脉缩窄或同时行肺动脉环缩术 ,后期再行心内畸形的治疗…     

心内畸形合并降主动脉缩窄一期矫治1例

病儿 男，13岁。自幼发现心脏杂音，活动后心悸、胸闷2个月。查体口唇无发绀；血压：右上肢134／71mmHg（1mmHg=0．133kPa），左上肢109／63mmHg，右下肢99／56mmHg，左下肢91／67mmHg。胸骨左缘3、4肋间可闻及3／Ⅵ级收缩期喷射样杂音，主动脉瓣听诊区可闻及3／Ⅵ级舒张期哈气样杂音。心电图示左心室肥大伴劳损。心脏彩色超声和大血管磁共振（MRA）检查见干下型室间隔缺损（VSD）1．0cm，主动脉瓣右冠瓣脱垂伴中度关闭不全，降主动脉起始处内径缩窄，最窄处约0．6cm。     

小儿主动脉缩窄合并心内畸形的治疗

2003年1月至2009年12月我院共收治小儿主动脉缩窄（CoA）合并心内畸形患者21例,取得了良好的效果。现总结该病围术期处理经验,以期降低围术期病死率。1临床资料与方法1.1一般资料本组共21例,男13例,女8例;年龄5~96个月（12.5±8.2个月）,其中〈6个月4例,6个月~1岁4例,1~3岁8例,〉3岁5例;体重5.5~18.0 kg（6.5±2.8kg）,其中〈6.0 kg 3例。     

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.     

降主动脉-升主动脉吻合术矫治婴儿期主动脉缩窄合并心内畸形15例

目的探讨降主动脉-升主动脉吻合术治疗婴儿期主动脉缩窄合并主动脉弓发育不良及心内畸形的疗效。方法选取2011-05—2015-05间治疗的主动脉缩窄合并主动脉弓发育不良、心内畸形的患儿15例。患儿均采用胸骨正中切口、开胸后先游离出主动脉弓、头臂干、动脉导管、弓降部等血管。建立体外循环、选择性脑灌注下行降主动脉-升主动脉端侧吻合术,恢复全身灌注后完成心内畸形的矫治。体外循环时间86~132 min,主动脉阻断时间51~94 min。结果术后早期死亡2例,低心排出量综合征6例,室上性心动过速6例,肺炎7例。13例患儿随访2个月~3 a,无死亡及再次主动脉狭窄。结论降主动脉-升主动脉吻合术治疗婴儿期主动脉缩窄合并主动脉弓发育不良及心内畸形的临床效果满意。     

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.     

端-侧吻合主动脉重建技术在婴儿主动脉缩窄合并心内畸形一期矫治术中的应用

目的比较婴儿主动脉缩窄(CoA)合并心内畸形一期矫治手术中采用端-侧吻合技术与扩大的端-端吻合技术的临床效果。方法 2008年1月至2011年7月广州市妇女儿童医疗中心共63例婴儿CoA合并心内畸形行一期矫治手术,按主动脉重建时的技术方法不同将63例患者分为两组,端-端吻合组:24例,男17例,女7例;年龄(4.6±2.9)个月,应用扩大的端-端吻合技术行手术治疗;端-侧吻合组:39例,男24例,女15例;年龄(3.4±2.6)个月,应用端-侧吻合技术行手术治疗。比较两组患者术后早期上、下肢动脉收缩压压差和围术期相关并发症发生情况。结果端-侧吻合组新生儿比率(23.1%vs.4.2%;χ2=3.979,P=0.045)、术前24 h内酸中毒比率(15.4%vs.0%;χ2=4.080,P=0.048)均高于端-端吻合组。术后端-端吻合组无死亡,端-侧吻合组死亡1例(2.6%)。端-侧吻合组术中停循环时间明显短于端-端吻合组[(18.6±2.7)min vs.(23.4±3.7)min,F=14.617,P=0.000]。端-端吻合组入心脏监护室(CICU)时上、下肢动脉收缩压压差<5 mm Hg、5～15 mm Hg、>15 mm Hg患者分别占20.8%、45.8%和33.3%,端-侧吻合组分别占97.4%、2.6%和0%,两组间差异有统计学意义(χ2=40.380,P=0.000)。术后24 h端-端吻合组上、下肢动脉收缩压压差<5 mm Hg、5～15 mm Hg、>15 mm Hg患者分别占45.8%、41.7%和12.5%,而端-侧吻合组占100%、0%和0%,两组间差异有统计学意义(χ2=26.620,P=0.000)。随访62例,随访时间2～36个月,所有患者均无血管瘤形成,无需再次手术处理的主动脉再狭窄。结论在婴儿CoA合并心内畸形的一期矫治手术中,与扩大的端-端吻合技术相比较,应用端-侧吻合技术可以安全、有效地显著降低术后早期出现的残余梗阻。     

婴幼儿主动脉缩窄合并心脏畸形的外科治疗

目的分析婴幼儿主动脉缩窄合并心脏畸形外科治疗的近远期疗效，探讨此类患者适宜的外科处理方法。方法回顾分析1998年5月至2006年11月期间在我院治疗的29例婴幼儿主动脉缩窄合并心脏畸形的临床资料，其中3例应用锁骨下动脉片翻转法，6例应用狭窄段切除和端侧吻合法，20例应用狭窄段切除和端端吻合法进行主动脉缩窄的矫治；并通过电话或书信对患者远期效果进行随访。结果手术死亡3例，其中1例死于低心排血量综合征，1例死于败血症，1例死于多器官功能衰竭。随访2个月～8年，无晚期死亡，无严重的神经系统并发症。术后发生再狭窄2例，患者均无自觉症状，继续随诊观察。全组未发现体循环高血压、动脉瘤形成和主动脉瓣反流等并发症。结论婴幼儿主动脉缩窄矫治术手术死亡率较低，术前心功能差和术后长时间机械呼吸是手术死亡的高危因素。术后再狭窄是婴幼儿主动脉缩窄患者主要的晚期并发症。     

主动脉缩窄的诊断和外科矫正

外科矫正主动脉缩窄２１例。术前漏诊３例。手术分别在浅低温、左心转流、体外循环或常温下进行，共采用８种术式。结果：ｌ８例术后上、下肢压差消失。２１例均痊愈出院，随访１～８年，仅２例成年病人仍有高血压。结论：上肢高血压而下肢低血压或存在差异性发绀者应疑诊本病。病人应于学龄前接受手术。多数可常温阻断主动脉行矫正术，术后应药物控制高血压。     

主动脉弓缩窄合并主动脉弓发育不良的外科治疗

目的探讨主动脉弓补片加宽成形术和主动脉弓扩大端侧吻合术治疗主动脉弓缩窄合并主动脉弓发育不良的优缺点,为临床选择更合理的手术方法。方法回顾性分析2008年6月至2013年6月北京安贞医院45例主动脉弓缩窄合并主动脉弓发育不良患者的临床资料,根据手术中处理主动脉弓发育不良的方法不同,将45例患儿分为两组,I组：26例,其中男15例,女11例;年龄0．5～6．8（0．9±2．5）岁;体重5．0～20.3（9．5±7-3）kg;采用主动脉弓补片加宽技术;术前右侧上下肢压差（38-3±15．6）mmHg。Ⅱ组：19例,其中男14例,女5例;年龄0．6～7．5（1．0±2．7）岁;体重5．5～21．5（10．2±6．6）kg;采用扩大端侧吻合技术;术前右侧上下肢压差（40．7±16．1）mmHg。检测两组患儿手术后上下肢压力差的变化,并与手术前进行比较。结果全组共死亡2例（4．4％）,其中l例死于低心排血量综合征,1例死于呼吸道感染。两组患儿均无肾功能衰竭及神经系统并发症。术后下肢收缩压高于上肢10～20mmHg28例,收缩压上、下肢相差小于10mmHg13例,上肢高于下肢20mmHg4例。右侧上下肢压差（3．2±13．5）mmHg,显著低于术前上下肢压差（P〈0．05）。组内比较：两组手术后上下肢压差明显低于术前（P〈0．05）。组间比较：两组之间上下肢压差术前、术后差异无统计学意义（P〉0．05）。随访38例,随访率88．4％,随访时间3个月至5年。随访期间1例患者出现主动脉弓降部血流速度进行性增快,主动脉弓压力阶差〉40mmHg,电子计算机断层扫描显示：主动脉弓再狭窄,于术后8个月再次行手术治疗;3例患者主动脉弓压力阶差〉20mmHg,仍在继续随访中;其余患者主动脉弓压力阶差均〈20mmHg。结论两种手术方式都是治疗主动脉弓缩窄合并主动脉弓发育不良的理想手术方法,应根据患儿具体病情选择合适的手术方法。     

Bifurcated Abdominal Aorta,with a Coarctation over the Right Branch of the Bifurcated Abdominal Aorta and Aberrant Renal Arteries Originating from the Left Branch of the Bifurcated Abdominal Aorta

This study describes an unusual case of secondary hypertension in a young female patient presenting with severe hypertension and abdominal bruits. Gadolinium‐enhanced MRA revealed a clearly bifurcated abdominal aorta, with a coarctation over the right branch of the bifurcated abdominal aorta and aberrant renal arteries originated from the left branch of the bifurcated abdominal aorta. Of interest is conventional angiography had failed to reveal these vascular abnormalities. This study mentions both embryologic and clinical aspects of this developmentally abnormal bifurcated abdominal aorta, coarctation of abdominal aorta and aberrant renal arteries.     

Repair of Coarctation of the Aorta in Neonates and Young Infants

Abstract In repair of coarctation in neonates or young infants, inadequate removal of ductal tissue, failure to address hypoplasia of the aortic arch, and suture line tension have been reported to be important factors of residual or early recurrent stenosis at the coarctation repair site. In a consecutive series of neonates and young infants with coarctation, who were all operated without delay with extended resection, the clinical outcome regarding the development of restenosis and hypertension was studied. In addition, the resected specimens were investigated regarding the completeness of resection of ductal tissue. Twenty-five consecutive neonates and young infants (median age 22 days, range 5 to 39 days) who underwent surgical correction of coarctation were reviewed; the resected specimens were examined histologically to document the extent of ductal tissue in the aortic wall. Fifteen patients had a preductal coarctation with associated cardiovascular anomalies including a hypoplastic aortic arch (n = 11). The remaining 10 patients had a paraductal coarctation without associated intracardiac anomalies. In all patients, the isthmus was bypassed and an end-to-side anastomosis was constructed between the descending aorta and the undersurface of the proximal aortic arch (n = 13) or the distal ascending aorta (n = 12). In 13 patients without marked hypoplasia of tbe aortic arch, the coarctation repair was performed through a left thoracotomy. In the remaining 12 patients, the coarctation was repaired through a median sternotomy with CPB and hypothermic circulatory arrest, on the basis of an associated hypoplastic aortic arch (n = 4), hypoplastic aortic arch with intracardiac anomalies (n = 7), or a “bovine” innominate artery (n = 1). There was no perioperative or late mortality. At a median follow-up of 15 months, 1 patient (4%) developed a recurrent stenosis at the coarctation repair site; in the remaining 24 patients, echocardiograpby showed a widely patent anastomosis with no evidence of a hemodynamically significant gradient. None of the patients had hypertension. Histologic examination of the resected specimens demonstrated the presence of ductal tissue in the descending aorta with maximal extension into its lateral wall (mean 5.2 mm). In all specimens of the paraductal subtype, there was also extension of ductal tissue into the lateral wall of tbe isthmus (mean 3.9 mm). We conclude that: (1) in the absence of marked hypoplasia of the proximal aortic arch, coarctation can be repaired with low mortality and morbidity via a left thoracotomy; (2) in the presence of marked hypoplasia of the proximal aortic arch and/or if associated intracardiac defects also need to be repaired, we advocate repair of the coarctation and associated defects through a median sternotomy with circulatory arrest; (3) in view of the absence of postoperative hypertension in this series, early repair of aortic coarctation is recommended; and (4) because ductal tissue may extend not only into the descending aorta but also into the isthmus, complete excision of the coarctation and bypass of the isthmus are valuable techniques to avoid secondary constriction of the aorta by ductal tissue.     

Coarctation of the descending aorta with aneurysm of the anterior communicating artery

A case of a 19-year-old woman who had coarctation of the descending aorta associated with an aneurysm of the anterior communicating artery is reported. The aneurysm was successfully clipped during the acute phase of subarachnoid hemorrhage.     

Coarctation of the Aorta in Infants and Children: 25 Years of Experience

Repair of coarctation of the aorta was performed in 334 patients ranging from less than a year to 16 years of age at Children's Hospital of Pittsburgh from 1953 to 1977. Resection and end-to-end anastomosis were performed in 310, interposition grafts in 7, and other procedures in 17. There were 41 operative deaths among the 95 infants less than 1 year old (43% mortality); all of these infants had associated cardiac anomalies. Only 1 operative death occurred in patients older than a year (0.4% mortality). No hospital deaths occurred in patients with isolated coarctation of the aorta. Eleven late deaths were due to associated anomalies (7) and unrelated or unknown causes (4). Postoperatively, hemorrhage occurred in 7, paradoxical or persistent hypertension in 128, postcoarctectomy syndrome in 32, neurological problems in 14, pulmonary complications in 53, and infections in 12 patients. Restenosis occurred in 26 patients (8%); only 10 have required reoperation. Residual hypertension was present in 28 of 264 patients (11%). Mortality from coarctation resection remains high in infants, but is minimal in children older than 1 year. Based on minimizing the risk of restenosis and residual hypertension, elective repair of coarctation is recommended when the patient is about 6 years of age.