先天性心脏病伴气管狭窄或受压的诊治探讨

目的 探讨先天性心脏病伴气管狭窄或受压的诊断和治疗措施,以提高对气管狭窄的认识.方法 收集本院先心病伴气管狭窄或受压患儿41例,平均年龄14.6个月,体重7.8 kg.主要病种:室间隔缺损/肺动脉高压;法乐四联症肺动脉闭锁、肺动脉瓣缺如;主动脉缩窄伴室间隔缺损;右室双出口;肺动脉吊带等.9例患儿伴双主动脉弓、迷走锁骨下动脉.经胸部CT、气管造影和纤维支气管镜确诊:气管弥漫性狭窄9例,局限性狭窄16例,支气管狭窄7例,多发性狭窄伴支气管桥4例,气管软化5例.38例行体外循环心脏修补术,6例同时行气管狭窄纠治术(端端吻合术3例;自身气管片移植扩大2例;补片扩大1例).5例行气管切开术,用套管支撑狭窄段.结果 住院、近期死亡8例(19.50%).随访22例,平均(2.9±2.4)年.气管端端吻合及自身气管补片扩大者,气管通畅;补片修复者,肉芽组织增生再狭窄.带套管支撑者存活4例,分别于术后3个月、1年、2年、8年拔除气管套管.狭窄未处理者,狭窄段内径与气管整体生长成比例狭窄.结论 先天性心脏病伴气管狭窄诊断需及时,重度气管狭窄与心脏手术Ⅰ期矫治,以滑片吻合术,自身气管片移植术为好.轻、中度狭窄则等待其成长是最好策略.     

先天性心脏病合并气管狭窄气管支架置入后取出指征探讨

方法 比较分析CHD合并气管狭窄气管支架置入后尚留和取出病例的临床资料。结果 9例置入气管支架患儿均为重度气道狭窄,置入气管支架为乐普（北京）公司生产的血管金属支架（PARTNER),其中尚留气管支架的4例患儿,平均年龄13.75个月,平均体重8.70 kg,随访支气管镜检查均未发现肉芽组织增生,镜下气管支架支撑良好;5例患儿于气管支架置入68～96 d 后取出,平均年龄48个月,平均体重4.96 kg。气管支架取出后发现支架支撑处并未塌陷,不影响患儿通气功能,取出气管支架完整,随访10～11个月,气道未见再阻塞及其他并发症,未出现需要再次置入气管支架的情况和死亡病例。在复习文献的基础上提出气管支架取出指征：①在良好的随访体系下气管支架置入2~3个月;②经支气管镜证实无肉芽组织增生,CT及其三维气道重建显示气道通畅;③临床无呼吸困难表现及感染征象,血气分析示无肺通、换气功能异常,X线胸片正常,超声心动图检查示心脏功能正常;④支气管镜下能清楚显示气管支架边界,且整个张开的支架位于气道表面并未上皮化;⑤支气管镜下钳夹住气管支架后,轻轻上下小幅推拉,镜下证实可以松动与气道紧密粘贴的支架。结论 对于小婴儿气管支架取出从长远角度来看是最佳选择,在良好的随访体系下,在不影响患儿生命质量的前提下,CHD合并气管狭窄气管支架置入2～3个月后取出安全可行。     

婴幼儿先天性气管狭窄伴复杂先心病的Ⅰ期纠治

目的：探讨婴幼儿先天性气管狭窄伴复杂先心病I期纠治的治疗效果。方法：2例手术年龄分别1.5岁和3岁的先天性气管狭窄伴法洛四联症患儿在体外循环下行气管狭窄纠治和四联症根治术。1例气管狭窄长约1.2cm，切除气管狭窄段，然后端端吻合；另1例气管狭窄长约6cm，切除3cm剪开形成两块补片，分别扩大剪开的气管前壁。总扩大气管狭窄段达6cm。结果：术后恢复良好。随访6个月-1年，呼吸平稳，CT示气管通畅，心脏超声检查无残余分流和梗阻。结论：纠治先天性气管狭窄半先心病，以I期手术治疗为好，不但术中在体外循环下纠治气管狭窄较安全，而且同时纠治先心病有利于手术后患儿康复。     

婴幼儿先天性心脏病合并气管狭窄的基层诊治策略

目的初步探讨婴幼儿先天性心脏病合并气管狭窄的基层诊治策略。方法回顾性分析泉州市儿童医院2014年5月至2015年11月间收治的25例先天性心脏病合并气管狭窄患儿的临床资料,对先天性心脏病并发或合并分支支气管轻中度狭窄的患儿采用术后早期拔管策略,合并严重气管狭窄的患儿建议转入上级医院接受进一步治疗,并对治疗结局进行评估。结果 18例合并轻中度气管狭窄的简单型先天性心脏病(室间隔缺损,房间隔缺损或动脉导管未闭等)患儿在我院接受先天性心脏病手术,术后均顺利撤离呼吸机; 7例先天性心脏病合并严重气管狭窄的患儿未在我院接受治疗,其中2例转入上级医院接受先天性心脏病联合气管狭窄手术,3例未及时接受手术而死亡。结论对于绝大多数合并节段性轻中度气道狭窄的简单型先天性心脏病婴幼儿,可采取保守治疗策略;而对于合并较为严重气管狭窄的患儿,在基层医院接受手术治疗存在较大风险,应尽早转上级医院接受心脏手术等一系列外科治疗。     

新生儿先天性心脏病的围手术期处理

先天性心脏病(简称先心病)是新生儿期常见的出生缺陷,发病率为活产婴儿的7‰～9‰,未经治疗者1/4于新生儿期死亡,是新生儿期死亡的主要原因之一。近年来,先心病的治疗趋向提早施行手术,手术水平向一期纠治的方向发展。随着胸外科手术水平的提高,新生儿期先心病手术成功率逐渐增高,为患有先心病的新生儿带来了更多的生存希望。掌握先心病的围手术期处理,使患儿在术前得到较好的治疗,生存到外科手术时,并减少手术并发症,对降低新生儿的死亡率十分重要。     

先天性心脏病合并气管狭窄的诊疗分析

先天性心脏病(先心病)合并气管狭窄较少见[1],术前诊断和治疗较困难,容易漏诊。但随着对该疾病的进一步认识,以及胸部CT气道三维重建、纤维支气管镜检查的广泛应用,对该疾病的术前诊断率明显提高。本研究对我院2009年4月至     

先天性心脏病合并气管狭窄的诊疗分析

先天性心脏病(先心病)合并气管狭窄较少见[1],术前诊断和治疗较困难,容易漏诊。但随着对该疾病的进一步认识,以及胸部CT气道三维重建、纤维支气管镜检查的广泛应用,对该疾病的术前诊断率明显提高。本研究对我院2009年4月至     

低体重危重先天性心脏病婴儿围手术期的监护与治疗

目的 探讨低体重危重小婴儿行先天性心脏病(先心病)外科手术治疗前后的监护与治疗特点.方法 采用回顾性研究,选择2002年1月～2005年12月间年龄≤11个月,体重≤6 kg的危重先心病婴儿69例,总结其围手术期的监护与治疗方法.结果 69例患儿经过治疗痊愈62例,死亡7例,病死率为10%.结论 低体重危重先心病婴儿术前常伴有较严重的肺炎、心力衰竭及肺动脉高压,当内科治疗效果不佳时应给予及时的手术治疗,术前的营养支持、术后严密监护、正性肌力药物及选择性肺血管扩张剂的使用等都是手术成功的关键.     

Development of a left atrial ball thrombus in a woman with complex congenital heart disease including congenital mitral valve stenosis

Summary A 20-year-old woman with complex cyanotic congenital heart disease, including severe congenital mitral stenosis and intact atrial septum, who developed a left atrial ball thrombus and an embolic phenomenon, is presented. Increased vigilance in this unique setting is necessary for the antemortem detection of this rare complication.     

The current state of congenital tracheal stenosis

Congenital tracheal stenosis (CTS) is an uncommon condition that has challenged pediatric surgeons for decades. Patients with CTS can present with a wide spectrum of symptoms and varying degrees of severity. In addition, a variety of techniques have been devised to repair this malformation. A review of these procedures and our suggestions for clinical standards and practice guidelines will be presented in this paper. A retrospective review of the literature on CTS from 1964 to 31 March, 2006. There is not one standard technique for the repair of CTS, as individualized approach to each patient and airway lesion is necessary to optimize patient management; nevertheless there is a consensus about segmental resection and anastomosis being best for short segment stenosis while slide tracheoplasty is most effective for the long-segment ones. Conservative management is also an option for select group of patients with careful and close follow up. Survival following surgery over the years has improved, but mortality remained high, particularly in a specific subset of patients presenting at the age less than 1 month with associated cardiac malformations. In conclusion, CTS remains a significant challenge for pediatric surgeons. Additional research is required to improve our understanding of the pathogenesis of CTS, and to develop evidence-based treatment protocols for the entire spectrum of presentation including conservative management.     

Perioperative extracorporeal membrane oxygenation for tracheal reconstruction in congenital tracheal stenosis

The management of a critical airway in infants and toddlers with congenital tracheal stenosis (CTS) continues to be an enormous challenge to the surgeon. Until recently, this condition often proved fatal. Improvements in surgical techniques, anesthetic management, and postoperative critical care have resulted in successful outcomes in children not long ago considered untreatable. However, issues such as the best operative approach and the optimal perioperative management are still unresolved. The diagnosis of CTS, often delayed, must be considered in any infant with stridor, wheezing, cyanosis, or recurrent episodes of pneumonia. Associated anomalies are the rule, including frequently vascular rings and rarely pulmonary agenesis. These defects can be repaired with conventional ventilatory support under cardiopulmonary bypass, or using extracorporeal membrane oxygenation (ECMO). We report our experience in which ECMO was used to support two patients with CTS during the perioperative period. ECMO proved to be both safe and practical, allowing unrushed, precise repair of the tracheal stenosis and providing brief postoperative support. Perioperative outcomes were excellent, although one of our patients died months after the repair. A review of the literature and our experience in which ECMO was used to provide cardiopulmonary support during repair of CTS showed uniformly successful perioperative outcomes. Accepted: 3 November 1998     

新生儿气管狭窄病因及诊疗进展

气管狭窄是由于不同病变所造成的气管腔狭窄,病因尚不清楚,但与先天性心脏病血管环压迫气道、气管软化及气管重塑等有着密切联系.影像学技术尤其是纤维支气管镜和CT对于诊断气管狭窄起到重要作用;手术是常用的治疗手段,气管置入支架是目前临床内科发展的方向.     

Slide方法在小儿先天性气管狭窄的应用

目的 总结Slide方法纠治长段气管狭窄的手术疗效.方法 2001年8月至2010年10月,手术纠治先天性气管狭窄21例.手术年龄28 d至8.6岁,平均(21.9±27.1)个月,体重4.2～22.5 kg,平均(9.65±4.24)kg,除2例分别为纵隔肿瘤和气管外伤所致气管短段狭窄外,其余19例均伴有先天性心脏病.根据气管狭窄长度,分为短段气管狭窄9例,长段气管狭窄12例.所有病例均在低温体外循环下行先天性心脏病纠治术,同时行气管狭窄处理.近期8例均采用Slide方法.结果 21例先天性气管狭窄手术死亡2例.1例早期采用人工材料修补,术后3个月出现肉芽,放弃治疗死亡.1例长段气管狭窄采用Slide方法,术后3周出现气管内肉芽,经多次球囊扩张、激光烧灼均无效,死亡.余19例长期随访2个月至8年,气管狭窄症状消失,CT复查效果满意,无一例再次手术.结论 Slide气管成形术采用自体的气管组织重建气道,吻合口牢固稳定,而且保持了正常的气管内膜,保证术后气管内壁的细胞功能和良好生长.是长段气管狭窄的最佳手术方法.由于本组病例较少,还需要定期随访,观察长期疗效.

Abstract：

Objective To evaluate slide tracheoplasty for congenital tracheal stenosis. Methods Between August 2001 and October 2010, twenty-one patients with congenital tracheal stenosis were repaired. The operative age was 28d～8. 6y (mean 21.9 ± 27. 1m) and the weight was 4. 2～22. 5kg (mean 9. 65 ± 4.24 kg) . Except for one case of mediasternal tumour and another one of tracheal trauma, all the restothers had congenital heart disease. There were 9 patients with short tracheal stenosis and 12 patients with long segment tracheal stenosis. The congenital heart disease were repaired underwent low temperature and cardiopulmonary bypass, and the tracheal stenosis were corrected simultaneously. Nine patients with long segment tracheal stenosis were repaired by slide tracheoplasty. Results There was two death in this group. One patient with right and left broncheal stenosis was repaired by synthetic patch. He succumbed 3 months later due todied of granulation tissue on the patch. Another patient with long segment tracheal stenosis was repaired by slide tracheoplasty, who also died from granulation tissue. All other 19 patients were uneventful. These patients were followed up from 2 month to 8 years, there were no complication. CT scan shows that the tracheal anastomosis was patent withand no any stenosis. Conclusions The slide tracheoplasty was used by autologous material,maintaining its contour and function of respiratory epithelium, and keeping its growth. The slide tracheoplasty is best way for correction of long segment tracheal stenosis. Long term follow up is required.     

Doty法矫治小儿先天性主动脉瓣上狭窄的疗效

目的 总结Doty法矫治小儿先天性主动脉瓣上狭窄的临床疗效.方法 2009年1月至2013年6月,Doty法矫治小儿先天性主动脉瓣上狭窄12例,其中合并Williams综合征5例.其中男9例,女3例;年龄9个月～12岁[(5.3±4.2)岁];体质量7.5～ 32.0(11.5±5.3)kg.心脏超声和增强CT明确诊断,局限型9例,弥散型3例;跨主动脉瓣上收缩压差6.86 ～16.23(11.07±3.68) kPa,压差＞13.33 kPa者3例;合并左、右肺动脉分支狭窄3例,主动脉瓣二瓣化2例,主动脉瓣轻度返流2例,动脉导管未闭2例,轻度狭窄1例,主动脉瓣下膈膜1例,肺动脉瓣狭窄1例.1例二尖瓣中-重度关闭不全.12例患儿均在全麻体外循环下行Doty法矫治术,并处理合并畸形.结果 体外循环时间68～ 129(85±26) min;主动脉阻断时间43 ～68(51±16) min.术后早期无死亡,无严重低心排出量综合征;5例轻度ST段改变,3例偶发期前收缩;呼吸机应用7～58 h,心脏重症监护室治疗时间1 ～5 d;超声心动图检查提示跨主动脉瓣上收缩压差1.01 ～3.76(2.18±1.09) kPa;有效随访患儿11例,随访6个月～3年,无死亡,无头晕、心悸.除2例患儿外,其余残余压差均低于3.33 kPa.结论 Doty法治疗小儿先天性主动脉瓣上狭窄可获得满意的疗效.     

小儿先天性心脏病介入治疗进展

从1966年Rashkind等进行球囊房间隔造口术至今,先天性心脏病介入治疗已经经过了50余年的发展,从最初的导管检查、姑息手术到现在的根治性封堵、支架的植入及瓣膜的替换等,介入性心导管技术已经成为治疗先天性心脏病的主要手段之一。我国先天性心脏病介入治疗已取得长足进步,但与发达国家相比仍有一定差距,现就间隔缺损性疾病、主肺分流性疾病、瓣膜性疾病及内外科镶嵌治疗等介入治疗进展进行梳理总结,以期对我国先天性心脏病的介入治疗提供借鉴。