孤立性永存左上腔静脉畸形的临床研究

Clinical study of the isolated persistent left superior vena cava

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。

Objective A systematic analysis on the clinical features, natural history, diagnosis and treatment experience of isolated persistent left superior vena cava（PLSVC） was done. Methods Collected the symptoms succession and nearly 21 years of examination and detection data of one 78-year old case with isolated PLSVC, then did a comparative analysis. Results The case was without positive symptoms until 57-year-old, which was sinus bradycardia. Dual-chamber pacemaker was implanted due to the sick sinus syndrome 11 years ago, before implantation, examinations showed leukocytes, and reduction of platelets, PLSVC was found during the surgery. Seven years ago, the performances of abdominal venous congestion were visualized, and 5 years ago, he was presented to clinics due to severe tricuspid regurgitation and right ventricular dysfunction. Three months ago, his chest enhanced CT＋ 3D cardiovascular reconstruction cleared the abnormalities of isolated PLSVC. Conclusion The delayed occurrence of isolated PLSVC symptoms motivates misdiagnosis of this malformation, whose first manifestation is often kinds of arrhythmias. Tricuspid systolic murmur could be found quite early. Echocardiography is the preferred screening method, cardiac catheterization or enhanced CT ＋ 3D cardiovascular reconstruction could manifested it. The treatments of cardiac arrhythmias include pacemaker implantation and coronary sinus target ablation. Some patients required early surgical treatment, at advanced stage, heart transplantation may be considered.