婴幼儿主动脉缩窄合并心内畸形的外科治疗

目的 探讨婴幼儿主动脉缩窄合并心内畸形的外科治疗经验.方法 2000年1月至2006年12月,84例主动脉缩窄合并心内畸形患儿接受了外科手术治疗,手术年龄1个月～3岁(平均13.5个月),体重3.3～15.0 kg(平均7.3 kg).12例合并复杂心内畸形,72例合并室间隔缺损和其他简单心内畸形,23例伴有主动脉弓发育不良.一期手术62例,49例正中开胸同时矫治主动脉缩窄和心内畸形,13例左侧开胸矫治主动脉缩窄,正中开胸修补心内畸形;分期手术22例.主动脉缩窄的手术方式包括补片成形42例,切除端端吻合30例,锁骨下动脉翻转6例,血管旁路3例,球囊扩张1例.在49例正中切口一期手术中,43例应用选择性脑灌注加下半身停循环,4例应用全身低流量灌注,2例应用深低温停循环.结果 围手术期死亡8例,死亡率9.5%,其中3例为术前漏诊主动脉缩窄.结论 婴幼儿主动脉缩窄合并心内畸形的外科治疗可获得良好的近期疗效,绝大部分患儿可采取正中切口一期手术.选择性脑灌注和下半身停循环可以有效地保护脑和重要脏器.     

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…     

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。     

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.     

婴儿主动脉缩窄合并心内畸形的一期矫治

目的总结婴儿主动脉缩窄（CoA）合并心内畸形一期矫治的手术方法和临床经验。方法2001年1月至2006年1月，对28例CoA合并心内畸形患者行一期手术矫治。CoA为导管前型18例，邻近或正对导管处10例；伴主动脉弓发育不良6例，动脉导管未闭22例。合并的心内畸形包括：室间隔缺损16例、室间隔缺损＋房间隔缺损5例、完全性房室间隔缺损3例，完全型大动脉错位伴室间隔缺损（D—TGA／VSD）2例，右心室双出口2例。采用左胸后外侧联合胸骨正中切口（双切口）径路手术12例，胸骨正中切口径路（单一切口）手术16例。采用缩窄段切除端端吻合术20例，Gore—Tex补片扩大成形术4例，左锁骨下动脉翻转扩大成形术4例。结果全组无手术死亡，术后呼吸机辅助呼吸时间7h～13d，住ICU时间3～18d。因术后心脏扩大延迟48～72h关胸4例，术后发生急性肾功能衰竭行腹膜透析3例，吸入一氧化氮（NO）治疗重度肺动脉高压3例。术后下肢收缩压高于上肢10～20mmHg18例（64．3％），收缩压上、下肢相差不大8例（28．6％），上肢分别高于下肢15mmHg和20mmHg2例（7．1％）。所有患者均得到随访，随访时间3～50个月，1例D—TGA／VSD患者因重度三尖瓣反流、肺部严重感染和呼吸功能衰竭于术后3个月死亡；其余27例患者恢复顺利。心脏超声心动图提示：1例采用端端吻合法、1例采用Gore～Tex补片扩大成形术的患者在CoA纠治处仍存在压差，分别为25mmHg、28mmHg，均未再次手术。结论一期手术矫治CoA合并心内畸形可以缩短疗程，有利于患者术后心、肺功能的恢复，避免二次手术的痛苦，降低治疗费用。可采用单一胸骨正中切口或胸骨正中联合左胸后外侧切口完成手术。     

一期手术纠治婴幼儿主动脉缩窄或离断合并心内畸形

目的 总结婴幼儿主动脉缩窄或离断合并心内畸形胸骨正中切口一期纠治的外科治疗策略.方法 2004年5月至2010年3月,采用胸骨正中切口一期纠治主动脉缩窄或离断合并心内畸形患儿52例.其中男性32例,女性20例;年龄25 d～7个月,平均(2.03±0.15)个月;体质量3.5～8.0 kg,平均(3.9±0.5)kg.采用自体心包片修补心内缺损;主动脉缩窄者40例,34例采用端侧吻合或扩大端侧吻合术,3例行纵切横缝术,3例假性主动脉缩窄行导管韧带切断主动脉松解;主动脉离断12例,均采用扩大端侧吻合.结果 所有病例心肺转流时间平均为(98±41)min,术中出血量(78±13)ml.1例主动脉离断合并右心室双出口患儿术后因左侧支气管狭窄,脱离呼吸机困难,于术后43 d死亡.其余病例术后生存良好,随访1～6年,术后再缩窄率为11%.结论 采用胸骨正中一期切口纠治主动脉缩窄或离断近期手术效果显著,可避免二次手术.

Abstract：

Objective To sum up one-stage complete correction of infantile aortic coarctation (CoA) or interrupted aortic arch (IAA) associated with intracardiac anomailes through median sternotomy.Methods The clinical data of 52 infants with CoA or IAA associated with intracardiac anomalies from May 2004 to March 2010 was analyzed. There were 32 male and 20 female, aged from 25 d to 7 months with a mean of (2.03 ±0. 15) months, weighted from 2.5 to 8.0 kg with a mean of (3.9 ±0.5) kg. All of intracardia defect were corrected by self-arcula cordisand. Forty cases with CoA were underwent by operative techniques, including resection with end to side anastomosis, extented end to side anastomosis (n = 34),and vertical incision and cross joint ( n = 3). Three cases of pseudo-CoA were cuted and ductus arterissus or ligamentum arteriosus and dissected aorch. Twelve cases of IAA were underwent by extented end to side anastomosis. Results The time of cardiopulmonary bypass was (98 ± 41 ) min, and all patients hemorrhaged (78 ± 13) ml during operation. One case of IAA associated with double outlet right ventricle died after 43 d post-operation because of left bronchinal stenosis. The other patients were in good condition.The rate of aneurysm formation was 11% in 1 to 6 years' follow-up. Conclusions One-stage complete correction of infantile CoA or IAA associated with intracardiac anomailes through median sternotomy yieldes excellent intermediate surgical results. This operative approach is beneficial, not only with shorten period of therapy and loss operative cost.     

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。     

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。     

1例先天性主动脉缩窄致胸主动脉破裂患者的护理

对1例先天性主动脉缩窄致胸主动脉破裂的患者,在深低温体外循环下实施胸主动脉人工血管置换术。术后患者出现心律失常、出血、切口感染、意识障碍和右侧肢体功能障碍等并发症,对其实施严密监护,采取相应的救治,做好心、肺、神经系统护理和基础护理,保证静脉营养供给。结果胸主动脉破裂所致危重状况得到有效控制,患者住院35d痊愈出院。     

介入诊断宫颈癌全盆腔切除术后并发髂动脉-输尿管瘘1例

患者，女，37岁．诊断宫颈癌（Ⅲb）1年．曾行化疗、放疗术、盆腔脏器全切术后3个月。术后患者定期更换肾盂输尿管支架管，曾因自行更换时出现血尿，入院治疗后缓解。术后2个月患者部分肠管坏死．形成肠瘘，粪便自阴道口排出。     

分期介入治疗主动脉缩窄合并右锁骨下动脉瘤1例

主动脉缩窄(CoA)是指主动脉局限狭窄,管腔缩小,造成血流量减少.主动脉缩窄可以单独出现,也可以合并二叶式主动脉瓣、室间隔缺损、动脉导管未闭、大动脉转位等病变.主动脉狭窄合并右锁骨下动脉瘤非常罕见,本文报导一例主动脉缩窄合并右锁骨下动脉及降主动脉瘤,一期应用定制的Armada 35球囊扩张导管及覆膜支架治疗主动脉狭窄,...     

栓塞治疗复杂责任血管引发肺结核咯血1例

患者男,40岁,主因＂肺结核10年,间断性咯血1个月、加重1天,累计咯血量约1000ml＂入院。RBC：2.5×1012/L,Hb：58g/L。胸部CT平扫示左上及左下肺结核（图1）。急行支气管动脉及其他责任血管造影,见左侧2支支气管动脉均增粗、扭曲,动脉晚期出现明显片状染色区（图2A）,用明胶海绵碎屑进行栓塞;右支气管动脉与右上肋间动脉共干,发出分支与左侧交通（图2B）,用同样的方法进行栓塞;逐个进行肋间动脉造影,发现左侧第7、8肋间动脉及左膈下动脉也为     

常温非体外循环下外科治疗主动脉缩窄15例

目的评价常温非体外循环下外科治疗先天性导管后型主动脉缩窄（CoA）的早中期效果。方法回顾性分析1999年1月至2004年12月间,15例在常温非体外循环下施行CoA外科手术患者的临床资料。全组男11例、女4例;年龄（18±10）岁。CoA为单纯性7例,合并动脉导管未闭（PDA）7例、合并PDA和室缺1例。在常温降主动脉部分阻断或主动脉弓-降主动脉临时旁路下施行人工血管旁路移植术9例;在常温降主动脉完全阻断施行腔内缩窄环切除加主动脉壁Gore—Tex补片成形术4例、缩窄段楔行切除端端吻合术2例。同期行结扎PDA8例,分期施行室缺修补术1例。结果全组无早期死亡,无声音嘶哑和下肢瘫痪。术后随访6个月～5年,下肢血压术后均较术前明显升高,术后有高血压者9例,其中需长期服用降压药者4例。无晚期死亡。术后人工血管通畅,降主动脉无再缩窄或假性动脉瘤形成。结论对于导管后型CoA,无论是单纯性、还是伴有PDA或合并其他心内畸形但准备分期手术者,常温非体外循环下施行CoA矫正手术是安全和有效的。     

Aortic valvular insufficiency and postductal aortic coarctation with small aorta syndrome: one-stage surgical management using extra anatomic bypass through median sternotomy

A 30-year-old man who had undergone repair for coarctation of the thoracic aorta at age 7 and mitral valve annuloplasty at age 9 was admitted for shortness of breath and claudication of both lower legs. The preoperative angiogram showed severe aortic regurgitation, moderate coarctation of the thoracic aorta beyond the left subclavian artery, a degree of hypoplasia of the infrarenal abdominal aorta, and total occlusion of both external iliac arteries. Aortic valve replacement, ascending-to-bilateral femoral arterial bypass, and end expanded polytetra fluoro ethylene (ePTFE) graft-to-descending aorta bypass was performed via a median sternotomy. Ascending-to-descending aortic bypass via the posterior pericardium allows simultaneous intracardiac repair or an alternative approach for the patient with complex coarctation.     

彩色多普勒超声诊断肾内静脉血栓形成1例

患者，男，15岁，主因“2天前无明显诱因出现发热，最高达39.4℃，左侧腰背部阵发性绞痛，拒按，伴血尿”来我院就诊。既往于4年前患有“肾病综合征”，治疗后好转。查体：腹膨降，软，左上腹压痛，反跳痛，左季肋区疼痛，拒按，左肾区叩痛，肠鸣音正常，双下肢对称性可凹陷性水肿。实验室检查：血常规：     

Multicenter experience with endovascular treatment of aortic coarctation in adults

ObjectiveThe objective of this study was to evaluate outcomes of endovascular treatment of aortic coarctation in adults.MethodsClinical data and imaging studies of 93 consecutive patients treated at nine institutions from 1999 to 2015 were reviewed. We included newly diagnosed aortic coarctation (NCO), recurrent coarctation, and aneurysmal/pseudoaneurysmal degeneration (ANE) after prior open surgical repair (OSR) of coarctation. Primary end points were morbidity and mortality. Secondary end points were stent patency and freedom from reintervention.ResultsThere were 54 (58%) male and 39 (42%) female patients with a mean age of 44 ± 17 years. Thirty-two patients had NCO (mean age, 48 ± 16 years) and 61 had endovascular reinterventions after prior OSR during childhood (mean, 30 ± 17 years after initial repair), including 50 patients (54%) with recurrent coarctation and 11 (12%) with ANE. Clinical presentation included asymptomatic in 31 patients (33%), difficult to control hypertension in 42 (45%), and lower extremity claudication in 20 (22%). Endovascular treatment was performed using balloon-expandable covered stents in 47 (51%) patients, stent grafts in 36 (39%) patients, balloon-expandable uncovered stents in 9 (10%) patients, and primary angioplasty in 1 (1%) patient. Mean lesion length and diameter were 64.5 ± 50.6 mm and 19.5 ± 6.7 mm, respectively. Mean systolic pressure gradient decreased from 24.0 ± 17.5 mm Hg to 4.4 ± 7.4 mm Hg after treatment (P < .001). Complications occurred in nine (10%) patients, including aortic dissections in three (3%) patients and intraoperative ruptures in two patients; type IA endoleak, renal embolus, spinal headache, and access site hemorrhage occurred in one patient each. The aortic dissections and ruptures were treated successfully by deploying an additional covered stent proximal to the site of dissection or rupture. Two patients died within 30 days of the index procedure. After a mean follow-up of 3.2 ± 3.1 years, nearly all patients (98%) were clinically improved and all stents were patent. Reintervention was needed in 10 (11%) patients. Freedom from reintervention at 5 years was 85%. Two additional patients died during follow-up of coarctation-related causes, including rupture of an infected graft and visceral ischemia. Patient survival at 5 years was 89%.ConclusionsEndovascular repair is effective with an acceptable safety profile in the treatment of NCO and postsurgical complications of coarctation after initial OSR. Aortic rupture is an infrequent (2%) but devastating complication with high mortality. Balloon-expandable covered stents are preferred for NCO, whereas stent grafts are used for ANE. The rate of reinterventions is acceptable, with high procedural and long-term clinical success.