Fontan类手术后20年远期心肺功能评价

摘要

目的 通过随访研究,评价Fontan术后远期心肺功能.方法 随访我院10例Fontan类手术后生存20年以上患者,其中女3例,男7例;最大年龄60岁,最小年龄20岁,平均(30.7±13.2)岁.术后随访(26±4)年.全组患者均经超声心动图、CT三维重建、24 h动态心电图、心导管及肺血管造影、核素肺血灌注扫描、心肺运动试验等检查.结果 超声心动图示左心室射血分数0.49±0.04,房室瓣膜反流中或中度以上7例.24 h动态心电图示窦性心律7例,频发短阵房速1例,心房扑动、心房颤动各1例.CT三维重建示全组未见吻合口狭窄,其中疑似动静脉瘘4例.右心导管及肺动脉造影示肺动静脉瘘患者1例,全组肺动脉压(17.8±2.0)mmHg(1 mmHg=0.133 kPa)、阻力(4.17±0.56)Woods.心肺运动试验示肺通气功能筛查减低,运动心脏储备功能中-重度下降.核素肺血灌注示双肺肺血出现不均衡分布特点.生化结合临床诊断蛋白丢失肠病1例.结论 Fontan类术后20年远期心肺功能均有不同程度下降,心功能下降重于肺功能.房室瓣反流较重、较多,早期手术应重视房室瓣病变处理.远期肺血呈不均衡分布.心外管道Fontan手术心律失常发生率低于其他术式.%To evaluate the long term cardiopulmonary function after Fontan operation. Methods 10 patients who received Fontan operation were followed for(26 ±4) years in our hospital. 7 males and 3 females,(30. 7 ± 13. 2) years old(20 -60 years). All patients were diagnosed by echocardiography, CTA, 24 h ECG, cardiac catheterization, pulmonary angiography, radionuclide pulmonary blood perfusion and cardiopulmonary exercise test. Results Echocardiography shows: LVEF(0. 49 ± 0. 04), more than moderate atrioventricular valve regurgitation in 7 cases. CTA shows: there was no anastomotic stenosis, arteriovenous fistula in 4 cases. 24 h ECG shows: sinus rhythm in 7 cases, frequent paroxysmal atrial tachycardia in 1 case, atrial flutter in one and atrial fibrillation in one. Cardiac catheterization and pulmonary angiography show: the average pulmonary artery pressure(17. 8 ±2. 0)mmHg, average pulmonary resistance(4. 17 ±0. 56) woods. Radionuclide pulmonary blood perfusion shows: pulmonary blood of upper lung was reduced significantly with unbalanced distribution. Cardiopulmonary exercise test shows: both of lung ventilation function and heart reserve function decreased moderately or severely. Protein losing enteropathy was found in 1 case. Conclusion The decrease of cardiopulmonary function is found after Fontan operation during the long-term follow up for 20 years, especially for cardiac function. More attention should be paid to the early postoperative atrioventricular valve regurgitation. Unbalanced distribution of pulmonary blood is Found in long term. The incidence of arrhythmia after extra conduit Fontan operation is lower than other methods.