Poffley Emma, Mortensen Kristian, Starling Luke, Mangat Jasveer, Marek Jan
Echocardiography Department, Great Ormond Street Hospital for Children, Great Ormond Street, London WC1N 3JH, UK.
Institute of Cardiovascular Sciences, University College London, London WC1E 6DD, UK.
Eur Heart J Case Rep. 2024 Dec 24;9(1):ytae693. doi: 10.1093/ehjcr/ytae693. eCollection 2025 Jan.
Superior caval vein obstruction is a rare complication of endocardial pacing lead implantation that can result in a right to left shunt.
A 3-year-old child with type 2 Brugada syndrome presented with mild cyanosis post-endocardial pacing implantation due to evolutionary right superior caval vein obstruction. This obstruction resulted in a right to left shunt across a previously unrecognized patent levo-atrial cardinal vein associated with partial anomalous pulmonary venous drainage. The patient underwent endocardial pacing explantation, balloon dilation and stenting of the right superior caval vein, banding of the levo-atrial cardinal vein to left upper pulmonary vein venous channel, and implantation of an epicardial pacing system.
Levo-atrial cardinal vein and partial anomalous pulmonary venous drainage with dual drainage can go undetected on cardiac imaging and may not ever cause symptoms (high left-to-right shunt or cyanosis). The levo-atrial cardinal vein and associated partial anomalous venous drainage with dual drainage was missed on multiple occasions and with multiple imaging modalities in our patient. Blood flow may not be detected in a small calibre or collapsed levo-atrial cardinal vein with pulmonary venous connection when the pulmonary vein remains widely patent and connected to the left atrium. Detailed comprehensive echocardiography of any child referred for cardiac intervention should include pulsed wave Doppler and colour flow mapping interrogation of the innominate vein from the suprasternal approach.
上腔静脉梗阻是心内膜起搏导线植入术罕见的并发症，可导致右向左分流。
一名患有2型Brugada综合征的3岁儿童，在心内膜起搏植入术后出现轻度发绀，原因是逐渐发展的右上腔静脉梗阻。这种梗阻导致血液通过一条之前未被识别的与部分肺静脉异位引流相关的左房静脉从右向左分流。患者接受了心内膜起搏导线取出术、右上腔静脉球囊扩张和支架置入术、将左房静脉结扎至左上肺静脉静脉通道，并植入了心外膜起搏系统。
左房静脉和伴有双引流的部分肺静脉异位引流在心脏成像时可能未被发现，且可能从未引起症状（大量左向右分流或发绀）。在我们的患者中，左房静脉及相关的伴有双引流的部分肺静脉异位引流多次被多种成像方式漏诊。当肺静脉保持广泛开放并与左心房相连时，在小口径或塌陷的与肺静脉相连的左房静脉中可能检测不到血流。任何因心脏介入治疗而就诊的儿童，详细全面的超声心动图检查应包括从胸骨上切迹对无名静脉进行脉冲波多普勒和彩色血流图检查。
