Fontan术后罕见并发症三例报道

Three cases report of rare complications after Fontan operation

回顾性分析3例Fontan术后比较罕见的并发症病例,其中2例为蛋白丢失性肠病,术前诊断1例为无脾综合征合并单心室和肺动脉狭窄,1例为功能性单心室合并肺动脉高压;另1例为塑型性支气管炎,术前诊断为纠正性大血管错位合并室间隔缺损和肺动脉狭窄。3例患儿Fontan术后至并发症出现的间隔时间分别为3周、3个月和5个月。术后死亡1例,失随访1例。Fontan术后腔静脉压力增高、心功能不全和高肺血管阻力,可能是蛋白丢失性肠病及塑型性支气管炎的发病原因。

Three cases of rare complications after Fontan operation were retrospectively analysed. Two cases experienced complication of protein-losing enteropathy, among whom one was diagnosed as asplenia with single ventricle and pulmonary stenosis, and the other was diagnosed as functional single ventricle with pulmonary hypertension. The other case had complication of plastic bronchitis, who was diagnosed as congenital corrected transposition of the great arteries with ventricular septal defect and pulmonary stenosis. Time intervals between Fontan operation and occurrence of complications were 3 weeks, 3 months, and 5 months respectively. One patient died after operation, and 1 patient lost in follow up. The increased systemic venous pressure, low cardiac output and high pulmonary resistance may be possible mechanisms of protein-losing enteropathy and plastic bronchitis.