The persistence of left superior vena cava (PLSVC) is the most common congenital anomaly of the venous return system to the heart. Because of the increasing number of patients referred for cardiac resynchronization th...The persistence of left superior vena cava (PLSVC) is the most common congenital anomaly of the venous return system to the heart. Because of the increasing number of patients referred for cardiac resynchronization therapy (CRT) devices implantations, it is expected to encounter this venous anatomic variation. Left ventricular lead placement at an appropriate site is an integral and technically challenging part of successful CRT. In case of cardiac abnormalities could be difficult to achieve an optimal cardiac rhythm management devices implantation. Previous reports in patients with PLSVC highlighted the challenges to achieve an optimal cardiac rhythm device implantation. Recently, a new quadripolar active fixation left ventricular lead is available for CRT device implantation. Hereby we report a case of a device upgrading from dual-chamber pacemaker to CRT with defibrillator backup using the active fixation left ventricular quadripolar lead in a patient with PLSVC and right superior vena cava atresia.展开更多
As a special focus in initiating and maintaining atrial fibrillation (AF), cardiomyocytes in superior vena cava (SVC) have distinctive electrophysiological characters. In this study, we found that comparing with the r...As a special focus in initiating and maintaining atrial fibrillation (AF), cardiomyocytes in superior vena cava (SVC) have distinctive electrophysiological characters. In this study, we found that comparing with the right atrial (RA) cardiomyoctyes, the SVC cardiomyoctyes had longer APD90 at the different basic cycle lengths; the conduction block could be observed on both RA and SVC cardiomyoctyes. A few of SVC cardiomyoctyes showed slow response action potentials with automatic activity and some others showed early afterdepolarization (EAD) spontaneously. Further more, we found that there are nonselective cation current (INs) in both SVC and RA cardiomyocytes. The peak density of INs in SVC cardiomyocytes was smaller than that in RA cardiomyocytes. Removal of extracellular divalent cation and glucose could increase INs in SVC cardiomyocytes. The agonist or the antagonist of INs may in-crease or decrease APD. To sum up, some SVC cardiomyocytes possess the ability of spontaneous activity; the difference of transmembrane action potentials between SVC and RA cardiomyocytes is partly because of the different density of INs between them; the agonist or the antagonist of INs can in-crease or decrease APD leading to the enhancement or reduction of EAD genesis in SVC cardiomyo-cytes. INs in rabbit myocytes is fairly similar to TRPC3 current in electrophysiological property, which might play an important role in the mechanisms of AF.展开更多
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...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。展开更多
永存左上腔静脉(persistent left superior vena cava,PLSVC)是由左前主干静脉退化失败所致,是上腔静脉最常见的畸形,也是最常见的先天性胸腔静脉异常,总患病率为0.35%~0.5%[1-3]。PLSVC伴右上腔静脉缺如(absent right superior vena ca...永存左上腔静脉(persistent left superior vena cava,PLSVC)是由左前主干静脉退化失败所致,是上腔静脉最常见的畸形,也是最常见的先天性胸腔静脉异常,总患病率为0.35%~0.5%[1-3]。PLSVC伴右上腔静脉缺如(absent right superior vena cava,ARSVC)是一种非常罕见的静脉畸形,称为孤立性PLSVC,发生率为0.09%~0.13%[3]。本文报道1例罕见的儿童孤立性PLSVC,早期诊断为窄QRS波心动过速,经心脏电生理检查明确诊断为房室结功能异常,完全性房室分离伴交界性自主节律、室性心动过速。展开更多
BACKGROUND Unroofed coronary sinus syndrome(UCSS)is a rare congenital heart disease,which has variable morphologic features and is strongly associated with persistent left superior vena cava(PLSVC).However,it is often...BACKGROUND Unroofed coronary sinus syndrome(UCSS)is a rare congenital heart disease,which has variable morphologic features and is strongly associated with persistent left superior vena cava(PLSVC).However,it is often difficult to visualize the left-to-right shunt pathway through the CS by transthoracic echocardiography(TTE).CASE SUMMARY A 37-year-old female was admitted to the hepatological surgery department of a hospital with complaint of subxiphoid pain that had started 1 wk prior.Physical examination revealed a grade 3/6 systolic murmur at the left margin of the sternum,between the 2nd and 3rd intercostal cartilage.The patient underwent echocardiography and was diagnosed with ostium primum atrial septal defect(ASD);thus,she was subsequently transferred to the cardiovascular surgery department.A second TTE evaluation before surgery showed type IV UCSS with secundum ASD.Right-heart contrast echocardiography(RHCE)showed that the right atrium and right ventricle were immediately filled with microbubbles,but no microbubble was observed in the CS.Meanwhile,negative filling was observed at the right atrium orifice of the CS and right atrium side of the secundum atrial septal.RHCE identified UCSS combined with secundum ASD but without PLSVC in this patient.CONCLUSION This rare case of UCSS highlights the value of TTE combined with RHCE in confirming UCSS with ASD or PLSVC.展开更多
The authors report a case of Large Anterior mediastinal mass in which Transoesophageal Echocardiography (TEE) revealed a large mass that was compressing the right atrium, SVC, henceforth, TEE helped in guid</span&g...The authors report a case of Large Anterior mediastinal mass in which Transoesophageal Echocardiography (TEE) revealed a large mass that was compressing the right atrium, SVC, henceforth, TEE helped in guid</span><span style="font-size:10.0pt;font-family:"">ing</span><span style="font-size:10.0pt;font-family:""> aspiration and surgical resection of the cystic mass. Therefore, through this case report, <span>authors emphasize the importance of performing intraoperative real-time</span> TEE in the mediastinal mass. This imaging technique helps in visualizing the external compression of cardio-vascular structure and aid in their management, obviating the possibility of large vascular structures from being injured by the surgeon during intraoperative manipulation.展开更多
文摘The persistence of left superior vena cava (PLSVC) is the most common congenital anomaly of the venous return system to the heart. Because of the increasing number of patients referred for cardiac resynchronization therapy (CRT) devices implantations, it is expected to encounter this venous anatomic variation. Left ventricular lead placement at an appropriate site is an integral and technically challenging part of successful CRT. In case of cardiac abnormalities could be difficult to achieve an optimal cardiac rhythm management devices implantation. Previous reports in patients with PLSVC highlighted the challenges to achieve an optimal cardiac rhythm device implantation. Recently, a new quadripolar active fixation left ventricular lead is available for CRT device implantation. Hereby we report a case of a device upgrading from dual-chamber pacemaker to CRT with defibrillator backup using the active fixation left ventricular quadripolar lead in a patient with PLSVC and right superior vena cava atresia.
文摘As a special focus in initiating and maintaining atrial fibrillation (AF), cardiomyocytes in superior vena cava (SVC) have distinctive electrophysiological characters. In this study, we found that comparing with the right atrial (RA) cardiomyoctyes, the SVC cardiomyoctyes had longer APD90 at the different basic cycle lengths; the conduction block could be observed on both RA and SVC cardiomyoctyes. A few of SVC cardiomyoctyes showed slow response action potentials with automatic activity and some others showed early afterdepolarization (EAD) spontaneously. Further more, we found that there are nonselective cation current (INs) in both SVC and RA cardiomyocytes. The peak density of INs in SVC cardiomyocytes was smaller than that in RA cardiomyocytes. Removal of extracellular divalent cation and glucose could increase INs in SVC cardiomyocytes. The agonist or the antagonist of INs may in-crease or decrease APD. To sum up, some SVC cardiomyocytes possess the ability of spontaneous activity; the difference of transmembrane action potentials between SVC and RA cardiomyocytes is partly because of the different density of INs between them; the agonist or the antagonist of INs can in-crease or decrease APD leading to the enhancement or reduction of EAD genesis in SVC cardiomyo-cytes. INs in rabbit myocytes is fairly similar to TRPC3 current in electrophysiological property, which might play an important role in the mechanisms of AF.
文摘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。
文摘永存左上腔静脉(persistent left superior vena cava,PLSVC)是由左前主干静脉退化失败所致,是上腔静脉最常见的畸形,也是最常见的先天性胸腔静脉异常,总患病率为0.35%~0.5%[1-3]。PLSVC伴右上腔静脉缺如(absent right superior vena cava,ARSVC)是一种非常罕见的静脉畸形,称为孤立性PLSVC,发生率为0.09%~0.13%[3]。本文报道1例罕见的儿童孤立性PLSVC,早期诊断为窄QRS波心动过速,经心脏电生理检查明确诊断为房室结功能异常,完全性房室分离伴交界性自主节律、室性心动过速。
文摘BACKGROUND Unroofed coronary sinus syndrome(UCSS)is a rare congenital heart disease,which has variable morphologic features and is strongly associated with persistent left superior vena cava(PLSVC).However,it is often difficult to visualize the left-to-right shunt pathway through the CS by transthoracic echocardiography(TTE).CASE SUMMARY A 37-year-old female was admitted to the hepatological surgery department of a hospital with complaint of subxiphoid pain that had started 1 wk prior.Physical examination revealed a grade 3/6 systolic murmur at the left margin of the sternum,between the 2nd and 3rd intercostal cartilage.The patient underwent echocardiography and was diagnosed with ostium primum atrial septal defect(ASD);thus,she was subsequently transferred to the cardiovascular surgery department.A second TTE evaluation before surgery showed type IV UCSS with secundum ASD.Right-heart contrast echocardiography(RHCE)showed that the right atrium and right ventricle were immediately filled with microbubbles,but no microbubble was observed in the CS.Meanwhile,negative filling was observed at the right atrium orifice of the CS and right atrium side of the secundum atrial septal.RHCE identified UCSS combined with secundum ASD but without PLSVC in this patient.CONCLUSION This rare case of UCSS highlights the value of TTE combined with RHCE in confirming UCSS with ASD or PLSVC.
文摘The authors report a case of Large Anterior mediastinal mass in which Transoesophageal Echocardiography (TEE) revealed a large mass that was compressing the right atrium, SVC, henceforth, TEE helped in guid</span><span style="font-size:10.0pt;font-family:"">ing</span><span style="font-size:10.0pt;font-family:""> aspiration and surgical resection of the cystic mass. Therefore, through this case report, <span>authors emphasize the importance of performing intraoperative real-time</span> TEE in the mediastinal mass. This imaging technique helps in visualizing the external compression of cardio-vascular structure and aid in their management, obviating the possibility of large vascular structures from being injured by the surgeon during intraoperative manipulation.
