Jonsson H, Ivert T, Jonasson R, Wahlgren H, Holmgren A, Björk V O
Department of Thoracic Surgery, Karolinska Hospital, Stockholm, Sweden.
J Thorac Cardiovasc Surg. 1994 Dec;108(6):1002-9.
Lung function was evaluated in 68 patients 13 to 26 (median 19) years after repair of tetralogy of Fallot. Age at repair was 7 years (9 months to 42 years) and 51% had a palliative shunt. An outflow patch was inserted in 56%. Median vital capacity was 84% of predicted, forced expiratory volume in 1 second 83%, maximal voluntary ventilation at 40 breaths/min 70%, and diffusing capacity for carbon monoxide 77% of predicted. Scintigraphy demonstrated abnormal pulmonary perfusion in 86%. Average right lung perfusion was 57% (predicted 52%). Regional hypoperfusion could in some patients be explained by previous palliative shunt, pulmonary artery obstruction, or presence of aortopulmonary collaterals. Median symptom-limited work capacity was 82% (95% confidence limits 78% to 90%) of predicted. Twenty-eight physically active patients had high values for symptom-limited work capacity, vital capacity, forced expiratory volume in 1 second, and maximal voluntary ventilation at 40 breaths/min compared with those of inactive patients. Lung function variables were related to physical exercise and previous palliative shunt. Moderate or severe pulmonary valve incompetence had negative but not significant influence on lung function. There was no significant influence of acyanosis before repair, use of transannular patch, duration of follow-up, or smoking. We found moderately reduced work capacity and lung function late after repair of tetralogy of Fallot that did not cause symptoms. Lung function variables were high in young active male patients and low in patients with previous palliative shunt. A better lung function in active patients indicates that physical activity should be encouraged after repair of tetralogy of Fallot.
在法洛四联症修复术后13至26岁（中位年龄19岁）的68例患者中评估了肺功能。修复时的年龄为7岁（9个月至42岁），51%的患者有姑息性分流。56%的患者植入了流出道补片。肺活量中位数为预测值的84%，第1秒用力呼气量为83%，40次/分钟时的最大自主通气量为70%，一氧化碳弥散量为预测值的77%。闪烁扫描显示86%的患者肺灌注异常。右肺平均灌注为57%（预测值为52%）。在一些患者中，局部灌注不足可由先前的姑息性分流、肺动脉梗阻或主肺动脉侧支的存在来解释。症状限制下的工作能力中位数为预测值的82%（95%置信区间为78%至90%）。与不活动的患者相比，28名身体活跃的患者在症状限制下的工作能力、肺活量、第1秒用力呼气量和40次/分钟时的最大自主通气量方面的值较高。肺功能变量与体育锻炼和先前的姑息性分流有关。中度或重度肺动脉瓣关闭不全对肺功能有负面影响，但不显著。修复前无青紫、使用跨环补片、随访时间或吸烟对肺功能无显著影响。我们发现法洛四联症修复术后晚期工作能力和肺功能有中度降低，但未引起症状。年轻活跃男性患者的肺功能变量较高，而有先前姑息性分流的患者较低。活跃患者更好的肺功能表明，法洛四联症修复术后应鼓励体育活动。
