先天性心脏病伴气管狭窄或受压的诊治探讨

Diagnosis and management for congenital heart defects complicated with tracheal stenosis or compression

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7．8kg。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（19．5％）。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。

Objective To investigate the diagnosis and management for congenital heart defects complicated with tracheal stenosis or compression. Methods Forty-one children who had tracheal stenosis or compression combined with congenital heart defects were enrolled into the study. The age of the patients ranged from 1.5 to 60 months, with the mean age of 14. 6 month; the weight ranged from 3. 7 to 16. 5 kg, with mean weight of 7. 8 kg. The diagnosis for the patients included ventricular septal defect （VSD） with pulmonary hypertension, tetralogy of Fallot with pulmonary atresia or absence of pulmonary valve, coarctation of the aorta combined with VSD, double outlets of right ventricle, and pulmonary artery sling. Nine patients had double aortic arch and aberrant subclavian artery. The tracheal abnormality included long-segment stenosis in 9 cases, short-segment stenosis in 16, bronchial stenosis in 7, multiple stenosis with bronchial bridge in 4, and tracheal compression in 5, which were diagnosed by computed tomography scanning, tracheography and fibreoptic bronchoscopy. Thirty- eight patients underwent cardiac surgery by cardiopulmonary bypass, in whom, 6 patients underwent surgical correction for tracheal stenosis., end-end anastomosis for 3 cases, tracheobronchial reconstruction with autograft for 2 ; tracheobronchial reconstruction with Gore-Tax patch for 1. Five patients underwent tracheotomy. Results The mortality rate during hospitalization was 19. 5%. Twenty-two patients were followed-up for 2. 9 ± 2. 4 years. No tracheal stenosis was noted in patients who had endend anastomosis or tracheobronchial reconstruction with autograft. Re-stenosis was found in patients who underwent tracheobronchial reconstruction with Gore-Tax patches. Four patients who were supported by trachea cannulas survived, whose cannulas were pulled out at 3 month, 1, 2, and 8 years respectively after operations respectively. For those whose tracheal stenosis was not treated, the stenosis correlated with the internal diameter of developed trachea. Conclusions Tracheal stenosis should be treated at the same time with the correction of congenital heart defects. For one-stage correction of congenital heart defects with severe tracheal stenosis in infants, Gore-Tax patches and autograft for tracheobronchial reconstruction are both satisfactory alternatives. While for mild or moderate tracheal stenosis, expectance for the development of segment of stenosis may be the optimal strategy.