孤立性永存左上腔静脉畸形的临床研究

目的对孤立性永存左上腔静脉畸形(persistent left superior vena cava,PLSVC)的临床特点、自然病程及诊治经验进行系统性分析,以期减少临床漏诊率。方法采集、整理北京积水潭医院1例78岁孤立性PLSVC患者的症状体征演变史及其近21年历次就诊的检查资料,进行比较分析。结果该例患者57岁出现心慌的首发症状,11年前因"病态窦房结综合征"安装双腔起搏器,术前检查外周血白细胞、血小板减低,术中发现PLSVC,7年前开始出现腹腔静脉淤血表现,5年前因重度三尖瓣反流、右心功能不全而就诊,3个月前胸部增强CT加血管重建明确为孤立性永存左上腔静脉畸形。结论孤立性永存左上腔静脉阳性症状出现晚,首发症状常为各种心律失常,临床极易漏诊误诊,心脏查体可于早期闻及三尖瓣收缩期杂音,超声心动图为首选筛查手段,心导管检查或增强CT加血管重建可明确诊断。其心律失常治疗包括起搏器植入术及冠状窦靶点消融。部分患者需早期手术治疗,疾病晚期可考虑心脏移植术。

体外循环心内直视手术中永存左上腔静脉的处理探讨

目的探讨体外循环手术时永存左上腔静脉(PLSVC)的发生率,总结手术处理经验。方法回顾性分析体外循环手术中所见PLSVC22例,占同期心内直视手术患者的2.3%(22/977),手术在全麻和中度低温体外循环下进行,根据不同情况分别对PLSVC采取套带阻断(8例)、插管引流(8例)、直接结扎(5例)或不做特殊处置(1例)等方法,保证手术的顺利实施。结果本组手术无死亡患者。16例患者术毕放置心表起搏电极,其中3例术后短期应用起搏器(1～3d)。1例法乐氏四联症患者发生左侧急性硬膜外血肿,清除后顺利恢复。所有患者术后随访1～10年,均无神经并发症,无远期死亡。结论 PLSVC发生率低,体外循环手术应重视。根据术中不同情况处置PLSVC既有利于手术操作,又不会对患者造成伤害,术毕应放宽心表起搏电极应用指征。

造影超声心动图诊断永存左上腔静脉合并先天性心脏病(附10例报告)

本文总结了10例永存左上腔静脉(PLSVC)合并先天性心脏病。10例中9例经手术证实,8例经二维超声心动图及造影超声心动图诊断。本文认为先天性心脏病手术前,常规进行二维超声心动图和造影超声心动图检查是很有必要的。

产前心超诊断永存左上腔静脉引流入左房的价值

文章回顾性分析5例经产前超声诊断为永存左上腔静脉引流入左房的超声资料及随访结果,总结其超声声像图特点,探讨产前超声诊断永存左上腔静脉引流入左房的价值。

A Case Report about Persistent Left Superior Vena Cava: Is it Always Asymptomatic?

A persistent left superior vena cava (PLSVC) is a rare malformation which affects approximately 0.3% - 0.5% of the population and it is presented along with a right-sided superior vena cava in 82.2% of the cases reported [1]. Clinicians diagnose it incidentally by difficulties with pacemaker implantation, central venous catheterization or screening for another etiologies when it is not accompanied by other anomalies it is typically asymptomatic. W. Schummer et al. described the embryogenesis and the anatomic variations of persistent LSVC according to the positioning of a central venous catheter on the chest radiograph: type I, normal;type II, only PLSVC;type IIIa, right and left superior vena cava with connection;type IIIb, right and left superior vena cava without connection [2]. In 92% of individuals with PLSVC, the PLSVC drains into a dilated coronary sinus (CS) and rest 8% drain directly into the left atrium. PLSVC is caused by a failure in the closure of the left anterior cardinal vein during embryogenic development [3]. The coronary sinus (CS) is a vein that transmits venous blood to the right atrium though atrioventricular groove. The CS wall contains atrial myocardium. Thus, its size extensively depends on variability of blood flow and pressure. We present a variant PLSVC with unknown prevalence and a mild platypnea-orthodeoxia syndrome after recovery of COVID-19 related acute respiratory distress syndrome (ARDS).