婴幼儿完全肺静脉异位连接的外科治疗

Surgical treatment for total anomalous pulmonary venous connection in infants and children

目的:探讨婴幼儿完全肺静脉异位连接的外科治疗方法及围手术期处理.方法:回顾性分析29例婴幼儿全肺静脉异位连接手术资料,年龄2~35mo(平均16.8±10.1)mo,体质量2.9~14.0kg(平均8.8±4.3)kg.均在中度低温体外循环下施行全肺静脉异位连接矫治术,其中5例为急诊或亚急诊手术.结果:全组手术死亡2例(死亡率6.9%),术后低心排血量6例,肺动脉高压危象1例,肺部感染5例,膈肌麻痹1例.27例随访1mo^8a(平均14.2±9.6)mo,失访2例,无远期死亡及明显肺静脉回流梗阻,心功能I级23例,I级4例.结论:全肺静脉异位连接疾病一旦发现,应尽早外科矫治,特别是回流梗阻症状严重者,必要时可实施急诊或亚急诊手术.术后要注意控制液体入量和避免过高的体循环后负荷,适当给予强心、利尿、控制肺动脉压治疗,尤其要重视呼吸道管理及营养支持.

AIM： To explore surgical operation and perioperative treatment for total anomalous pulmonary venous connection （ TAPVC ） in infants and children. METHODS ： Twenty-nine corrective operations for TAPVC were retrospectively analysed. All patients, aging 2 - 35 months and weighing 2.9 - 14.0 kg, underwent total corrective operations with moderate hypothermia and cardiopulmonary bypass, of which 5 cases were emergency or sub-emergency. RESULTS ： Two patients died （ mortality 6.9% ）. In the period of perioperative low cardiac output occurred in 6 patients, pulmonary hypertension crisis in 1, pulmonary infection in 5 patients and diaphragmatic paralysis in 1 patient. Follow-up varied from 1 month to 8 years in 27 patients. No later death or obvious obstruction of pulmonary venous drainage were found. Twenty-three patients were NYHA Ⅰ class and 4 were Ⅱ class. CONCLUSION： Corrective operations for TAPVC have to be performed as soon as diagnosis is confirmed. Particularly for severe obstruction of pulmonary venous drainage, emergent or subemergent operation is necessary. Postoperative treatment should limit fluid volume and avoid exorbitant afterload. Proper managements including cardiac, diuresis and controlling pulmonary artery pressure are helpful, especially respiratory administration and nutrition support.