Sultan Mehboob, Zulfiqar Zunaira, Khan Maryam, Ahmed Yashfeen
Paediatric Cardiology, Army Cardiac Centre, Lahore, PAK.
Paediatrics, Combined Military Hospital, Lahore, PAK.
Cureus. 2022 Jul 19;14(7):e27035. doi: 10.7759/cureus.27035. eCollection 2022 Jul.
Obstructed total anomalous pulmonary venous connection is a life-threatening pediatric cardiac emergency. Infants usually present in critical condition with marked respiratory distress, severe metabolic acidosis, and central cyanosis. Urgent cardiac surgical intervention, despite its high risk, is necessary in order to save the life of the patient. A two-month-old female infant presented to our tertiary care hospital with dense cyanosis and metabolic acidosis. She required mechanical ventilation, but her oxygen saturation did not improve. Her 2D transthoracic echocardiography revealed obstructed supracardiac total anomalous pulmonary venous connection with adequate interatrial communication and severe pulmonary hypertension. After discussion with the family and pediatric cardiac surgical team, it was decided to offer her transcatheter relief of obstructive ascending channel. She underwent successful balloon angioplasty of stenosed levoatrial cardinal vein (vertical vein) with remarkable improvement in blood flow and vessel caliber. She was extubated and her oxygen saturation rose from the high seventies to low eighties immediately after the procedure. She is scheduled for cardiac surgical repair within the next few days. Transcatheter angioplasty is a workable option in stabilizing very sick young infants with obstructed total anomalous pulmonary venous connection, especially supracardiac ones.
梗阻性完全性肺静脉异位连接是一种危及生命的小儿心脏急症。婴儿通常病情危急，伴有明显的呼吸窘迫、严重的代谢性酸中毒和中枢性发绀。尽管风险很高，但紧急心脏手术干预对于挽救患者生命是必要的。一名两个月大的女婴因重度发绀和代谢性酸中毒被送至我们的三级护理医院。她需要机械通气，但氧饱和度没有改善。她的经胸二维超声心动图显示心上型完全性肺静脉异位连接梗阻，伴有足够的房间交通和严重的肺动脉高压。在与患儿家属和小儿心脏外科团队讨论后，决定为她进行经导管解除梗阻性上升通道。她成功接受了狭窄的左房总静脉（垂直静脉）球囊血管成形术，血流和血管管径有显著改善。术后她立即拔管，氧饱和度从七十多岁升至八十岁出头。她计划在接下来的几天内接受心脏手术修复。对于病情严重的梗阻性完全性肺静脉异位连接的小婴儿，尤其是心上型的，经导管血管成形术是一种可行的稳定病情的选择。
