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目的提高对先天性心脏病合并气道异常的认识,总结围术期处理经验。方法对中南大学湘雅二医院2006年1月至2008年1月收治的5例先天性心脏病合并气道异常患者的临床资料进行回顾性分析,其中男3例,女2例;年龄5个月~9岁,平均年龄27个月。气道异常包括:左主支气管狭窄2例,右侧气管化支气管、气管轻度软化和左主支气管狭窄、长段先天性气管狭窄各1例。行先天性心脏病矫治术加左主支气管成形术1例,仅行先天性心脏病矫治术3例,放弃手术治疗1例。结果围术期死亡1例,为先天性心脏病合并右侧气管化支气管,术后发生右肺上叶不张、肺部感染和呼吸衰竭死亡。1例先天性心脏病合并左主支气管狭窄,同期行左主支气管成形术后顺利出院;随访2年健康。1例行先天性心脏病矫治术后撤离呼吸机困难,发现合并先天性左主支气管狭窄伴轻度气管软化,经内科处理撤离呼吸机后仍有喘憋;随访6个月患者发育好,喘憋未再发作。1例混合型室间隔缺损致心脏扩大、肺动脉扩张压迫左主支气管导致狭窄,行先天性心脏病矫治术后左主支气管压迫症状缓解;随访1年健康。1例合并长段气管狭窄,家属放弃手术。结论先天性心脏病合并气道异常临床上很少见。应提高对该病的认识,做到术前确诊;采用最佳的手术方法,尽量与先天性心脏病同期手术纠治;术后加强呼吸道管理,以获得最佳的疗效。
Objective To improve the understanding of congenital heart disease with airway abnormalities and summarize the experience of perioperative management. Methods The clinical data of 5 patients with congenital heart disease complicated with airway abnormalities admitted to the Second Xiangya Hospital of Central South University from January 2006 to January 2008 were retrospectively analyzed. There were 3 males and 2 females, aged 5 Month ~ 9 years old, average age 27 months. Abnormal airways include left main bronchial stenosis in 2 cases, right tracheal bronchus, mild tracheal softening and left main bronchial stenosis, and 1 long-term congenital tracheal stenosis. Line congenital heart disease plus left main bronchoplasty in 1 case, only congenital heart disease in 3 cases, to give up surgery in 1 case. Results Perioperative death in 1 case, congenital heart disease with tracheal bronchus right after the occurrence of the right upper lobe atelectasis, lung infection and respiratory failure death. One case of congenital heart disease with left main bronchus stenosis, the same period of left main bronchoplasty successfully discharged; 2 years follow-up health. 1 routine treatment of congenital heart disease evacuation after ventilator difficulties, found with congenital left main bronchial stenosis with mild tracheal softening, evacuated by mechanical treatment after the respirator still breathing; follow-up 6 months of patients with good development, asthma Hold no further attack. 1 case of mixed ventricular septal defect caused by cardiac enlargement, left pulmonary embolism caused by expansion of pulmonary artery leads to stenosis, line left congenital heart disease correction of postoperative left bronchial symptoms relieved; follow-up 1 year health. 1 case of long-term tracheal stenosis, family members to give up surgery. Conclusions Congenital heart disease with airway abnormalities is clinically rare. Should raise the awareness of the disease, so that preoperative diagnosis; the best surgical methods, as far as possible with congenital heart disease surgical correction; postoperative respiratory tract management to get the best effect.