Hayashi Taiyu, Hirata Yoichiro, Inuzuka Ryo, Hirata Yasutaka
Department of Pediatrics, Graduate School of Medicine, University of Tokyo, Tokyo, Japan.
Department of Cardiothoracic Surgery, Graduate School of Medicine, University of Tokyo, Tokyo, Japan.
Pediatr Int. 2015 Aug;57(4):714-6. doi: 10.1111/ped.12580. Epub 2015 Mar 25.
We describe a rare case of infracardiac total anomalous pulmonary venous connection (TAPVC), associated with congenitally corrected transposition of the great arteries (ccTGA) and ventricular septal defect, in which the patient had undergone pulmonary artery banding (PAB) at 16 days of age. She began to have episodes of severe cyanosis while crying, 2 weeks after PAB. Cardiac catheterization at 34 days of age showed severe pulmonary hypertension and a transhepatic pressure gradient of 7 mmHg. The infant underwent TAPVC repair and conventional repair for ccTGA at 35 days of age. Although PAB might have the provisional effect of delaying the manifestation of pulmonary venous obstruction (PVO), it is unable to prevent the development of PVO due to the high resistance of the hepatic sinusoids. Signs of PVO should be closely monitored so that TAPVC can be repaired in a timely fashion.
我们描述了一例罕见的心下型完全性肺静脉异位连接(TAPVC)病例,该病例合并先天性矫正型大动脉转位(ccTGA)和室间隔缺损,患者在16日龄时接受了肺动脉环扎术(PAB)。在PAB术后2周,她开始在哭闹时出现严重青紫发作。34日龄时的心脏导管检查显示严重肺动脉高压,经肝压力梯度为7 mmHg。该婴儿在35日龄时接受了TAPVC修复及ccTGA的传统修复。尽管PAB可能有暂时延缓肺静脉梗阻(PVO)表现的作用,但由于肝血窦阻力高,它无法预防PVO的发生。应密切监测PVO的体征,以便及时修复TAPVC。