【作者】 葛振伟；

【导师】 赵文增；

【作者基本信息】 郑州大学 ， 心胸外科学， 2011， 博士

【摘要】 背景与目的先天性心脏病是胎儿时期心脏血管发育异常所致的心血管畸形,是小儿最常见的心脏病。其发病率约占出生婴儿的4%o-10‰[1-5],其中60%于<1岁死亡。发病可能与遗传尤其是染色体易位与畸变、宫内感染、大剂量放射性接触和药物等因素有关。随着心血管医学的快速发展,许多常见的先天性心脏病得到准确的诊断和合理的治疗,病死率已显著下降。随着人们公众卫生意识的提高及临床诊疗技术水平的飞速发展,先天性心脏病(先心病)的临床治疗逐步转向低龄化、复杂畸形矫治的早期化。与此同时临床上又发现相当部分的先天性心血管畸形合并有气管、支气管狭窄,这就为本已非常棘手的小儿心脏手术后的呼吸道管理又增加了极大的困难,影响预后,甚至直接导致围手术期死亡[6,7]。先天性心脏病合并气管狭窄的发病率很少,并且很难处理。对先心病合并气管狭窄的认识最早始于心脏畸形矫治良好循环稳定后患儿因呼吸窘迫不能停机械通气的支持及拔出气管插管后反复再插管,进一步行胸部CT、MRI和／或纤维支气管镜检查后才诊断。后来,随着对气管狭窄认识的不断加深,通过对术前即合并有气促、喘鸣、吸凹征的患儿及小婴儿常规进行胸部CT气道重建检查,临床检出率渐增加,据文献报道占先心病发病率的0.15%[8]。先天性心脏病合并气管狭窄是一种在临床上少见的疾病,目前对于其发病机制还不清楚。气管狭窄的原因有很多种,狭窄可能是先天性畸形；气管的外压性改变如血管环和肺动脉吊带压迫气管限制了管腔的发育造成的继发性狭窄；长期气管插管和气管切开的并发症等。Backer等定义气管狭窄：管腔内径不足正常气管的50%。另外,把气管狭窄分为：先天性完全性气管环、气管插管后狭窄、外伤性狭窄、先天性气管蹼和其他类型狭窄。狭窄段在气管总长的50%以下者称为局限性狭窄,超过50%者为长段狭窄。Grillo等[10]描述了长段气管狭窄几乎均合并有完全性气管环,狭窄的长度在超过半数的病例中均超过气管总长度的50%,不管何种原因,临床表现主要是气道狭窄梗阻。先天性心脏病合并气管狭窄由于存在复合畸形,病情严重,如不做出及时、准确的诊断和治疗,将会严重地威胁到患儿的生命,据文献报道,死亡率可高达41.7%[11]。先天性心脏病引起的血流动力学的改变和气道狭窄以及由此引起的气道高反应性引起的气道阻塞意味着患者的心肺储备功能差,尤其在呼吸道感染存在的情况下。由于缺乏临床经验和针对气管狭窄的研究,大多心脏外科医生只是处理心脏病,或者分期处理心脏病和气管狭窄。结果是没处理的疾病在术后管理期间造成严重的后果,术后患者需要呼吸机辅助呼吸,通常会引起呼吸道感染,呼吸衰竭,甚至死亡[13]。目前对于此类复合畸形的临床治疗,大家比较推崇的策略是同期行先天性心血管畸形和气管狭窄的矫治[14,15,16],因为对于同时具有胸内段气管狭窄所致的气道梗阻及先天性心脏畸形的病人行手术治疗时,任何分期处理计划的实施均可能导致极其不良的后果,给术后管理制造极大的困难[7]：如果先处理心内畸形,那么气道狭窄会在术后引起严重的而且经常是致命的呼吸道问题如呼吸道梗阻、撤机困难、下呼吸道感染、呼吸衰竭、败血症等；若先手术纠治气管狭窄而遗留心内畸形不予处理,由于先心病的严重低氧血症,慢性心功能不全或肺动脉高压,致组织微循环差和／或缺氧、营养不良将严重影响气管狭窄纠治术后的愈合,增加术后感染、创口裂开的发生率[6]本文主要探讨先天性心脏病合并气管狭窄患儿的手术治疗策略及不同气管成形技术的临床应用。材料与方法分期治疗组(分期组)共3例患儿,男2例、女1例,年龄：8月、14月、5岁,体重：8.0kg、9.5kg、20.5kg。心血管畸形分别是室间隔缺损2例、法洛三联症1例；2例是心脏手术后分别通过尸检和纤维支气管镜检查发现合并气管狭窄,1例术前行CT三维气道重建诊断气管狭窄。同期治疗组(同期组)共6例患儿,男5例、女1例,年龄：5月-7岁(15.6±14.2月),体重：7.0-24.0(10.5±7.5)kg。均患有先天性心脏病,其中室间隔缺损1例,房间隔缺损2例其中1例伴左肺动脉异常起源于右肺动脉(肺动脉吊带),单纯肺动脉吊带1例,肺动脉瓣狭窄伴卵圆孔未闭1例,法洛四联症1例。手术前发现有声嘶、喘鸣、呼吸困难症和／或运动能力下降者5例。全部病例均先经超声心动图行心内畸形诊断,除两例单纯房、室间隔缺损外,其余均经双源64排CT增强造影检查进一步明确心内畸形及相关指标发育情况评估。全部病例进行了胸部CT气道三维重建,3例同时进行了纤维支气管镜检查,以明确气管狭窄的部位和程度,并行术后观察随访。心脏畸形矫治：均采用胸骨正中开胸,建立体外循环转流,心脏停跳下行心血管畸形矫治术。气管狭窄的手术矫治：分期组一例患儿先期行气管狭窄矫治：标准后外侧切口,右侧第5肋间进胸,肝素化后股动静脉转流并行循环保证氧供,狭窄段切除后继以端-端吻合。同期组病例气管狭窄矫治：均心脏畸形矫治后开放升主动脉心脏复跳、并行循环下进行。2例狭窄长度在2cm以内者,采用狭窄段切除后端-端吻合法；3例仅涉及主气管的长段狭窄病例,采用滑动法气管成形；1例合并左右支气管狭窄的长段气管狭窄病例,术中把狭窄段的气管和支气管前壁切开,采用同种冷藏主动脉壁作为补片材料加宽狭窄的气管和支气管管腔。结果分期组2例术后才诊出合并气管狭窄者均因并发严重呼吸道合并症院内死亡,1例先期行气管狭窄矫治者术后发生吻合口瘘,家属拒绝再次手术自动出院。手术死亡率几近100%同期组全组病例术后带气管插管时间6h-18d(1.5±1.2d)。一例肺动脉瓣狭窄(PS)合并长段气管狭窄病例行PS矫治并滑动法气管成形术,术后8周又出现气促复诊,检查为吻合口肉芽组织增生,后不得不气管插管呼吸机辅助,多次经气管插管腔内电灼治疗,但肉芽组织生长迅速,后发现向双侧支气管腔内蔓延,在一次电灼治疗中气管出血窒息死亡。死亡率约16.7%。其余病例均效果良好,随访4月-4.5年,无气促症状,临床查体无喘鸣。行气道CT重建检查气管、主支气管均通畅,无明显狭窄；行心脏超声检查,无残余心内畸形,心功能评估良好。结论1对于先心病合并先天性气管狭窄病例的临床治疗我们认为同期行心血管畸形和气管狭窄的矫治是比较合理的手术策略,临床观察效果良好。2对于心内畸形应根据具体情况尽量予一期根治,必要时也可分期治疗。3而对于气管狭窄者,则需根据狭窄的程度和部位不同而采取不同的方法才能取得良好的效果：狭窄段切除后再吻合和滑动法气管成形方法均是用自身气管组织进行矫治的方法,临床效果确切。4对于不得不采用补片法成形的气管狭窄,我们认为同种异体冷藏主动脉材料可是一种新的选择,但需要进一步的临床印证。更多还原

【Abstract】 Background and ObjectiveCongenital tracheal stenosis (CTS) in infants is an underdiagnosed, life-threatening respiratory anomaly. Associated congenital cardiac malformations are diagnosed often in this group of patients. They are represented mostly by ventricular septal defect (VSD) and atrial septal defect (ASD). Some of the more complicated congenital heart malformations can also accompany CTS:atrioventricular septal defects (AVSDs) and tetrelogy of Fallot (TOF). CTS often is associated with pulmonary vascular sling or bronchus suis, an anomalous right upper bronchus branching from the trachea. From a pathophysiologic point of view, it seems more reasonable to repair both the tracheal and cardiac pathology simultaneously. In this article, through to describe our experience, we analyzes and summarizes the effect of simultaneously surgical treatment to congenital heart disease(CHD) with concomitant congenital tracheal stenosis(CTS) and the different surgical alternatives of tracheal repair.Object and method3 infants underwent twe-stage surgical repair and 6 infants underwent simultaneous surgical repair of CTS and CHD. All patients were operated on through a median sternotomy and with cardiopulmonary bypass. After associated cardiac anomalies corrected, the tracheal repairs were performed,including tracheal resection with direct end-to-end anastomosis to localized tracheal stenosis in 2 patients, slide tracheoplasty to long-segment CTS in 3 patients, and cryopreserved aortic allograft (CCA) patch tracheoplasty to CTS with main bronchus involved in 1 patient.ResultsIn the twe-stage repair group,2 patients died of asphyxia and lung infection respectively. In the One-stage repair group, One patient performed with slide tracheoplasty died of granulation tissue on stoma 8 weeks postoperatively.5 patients were extubated between 6 hours and 2 days after operation. Postoperative CT examination showed adequate airway dimensions and patency in every case. The midterm results after a mean follow-up of 28 months (range,4～54 months) of the entire group demonstrate a stabile and complication-free clinical outcome.ConclusionWe advocate the strategy of simultaneously surgical treatment to CHD with concomitant CTS for infants. With cardiopulmonary bypass, intracardiac anomalies can be repaired, and tracheal reconstruction can be reassured.Different surgical alternatives of tracheal repair should be apt to the situs and extent of CTS. Management of congenital tracheal stenosis by mean of slide tracheoplasty or resection and reconstructions gives excellent short- and long-term results because the lesion is reconstructed with native tracheal tissue and is therefore immediately stable and lined with normal epithelium.If CTS involves a more long segment and/or main bronchus and a program of patch tracheoplasty has to be applied, CAA may be considered as a valuable tracheal substitute. Prolonged clinical application will be probably mandatory to authenticate the more long-time result of the procedure.更多还原