Unroofed coronary sinus,left-sided superior vena cava and mitral insufficiency:A case report and review of the literature

BACKGROUND Unroofed coronary sinus(UCS)is a rare subtype of atrial septal defect.It is frequently associated with a persistent left superior vena cava and is often part of a more intricate cardiac malformation.CASE SUMMARY This report describes a rare case of an adolescent patient with UCS featuring atrial situs solitus,absence of the right superior vena cava and a persistent left superior vena cava draining into the left atrium consistent with total unroofing of the coronary sinus.This was associated with concurrent severe mitral insufficiency secondary to redundant and prolapsing leaflets,and a substantial left-to-right shunt across the coronary sinus orifice.A comprehensive examination of the existing literature is included,shedding light on the diagnostic challenges of UCS and describing the available surgical options within the context of mitral valve surgery.CONCLUSION UCS is a complex condition requiring careful consideration of associated anomalies and a tailored surgical approach.

Persistent left superior vena cava in right hemiarch replacement under deep hypothermic circulatory arrest:A case report

BACKGROUND Persistent left superior vena cava(PLSVC),a relatively rare thoracic vascular malformation,can inconvenience perfusionists and operators when encountered during deep hypothermic circulatory arrest(DHCA).CASE SUMMARY Herein,we describe the case of a patient with concurrent giant aortic arch aneurysm,aortic stenosis,and PLSVC.To treat these conditions,we performed right hemiarch and aortic valve replacements under DHCA.Notably,we applied“bilateral superior vena cava retrograde cerebral perfusion(RCP)”for cerebral protection,which significantly optimized the surgical procedure and reduced the risk of postoperative complications.The patient was discharged 14 d after surgery with no complications.CONCLUSION Surgical intervention for PLSVC under DHCA can be performed using the bilateral superior vena cava RCP approach.

Integrated analysis of comorbidity, pregnant outcomes, and amniotic fluid cytogenetics of fetuses with persistent left superior vena cava

BACKGROUND Persistent left superior vena cava(PLSVC)is the most common venous system variant.The clinical characteristics and amniotic fluid cytogenetics of fetuses with PLSVC remain to be further explored.AIM To develop reliable prenatal diagnostic recommendations through integrated analysis of the clinical characteristics of fetuses with PLSVC.METHODS Cases of PLSVC diagnosed using prenatal ultrasonography between September 2019 and November 2022 were retrospectively studied.The clinical characteristics of the pregnant women,ultrasonic imaging information,gestational age at diagnosis,pregnancy outcomes,and amniocentesis results were summarized and analyzed using categorical statistics and the chi-square test or Fisher’s exact test.RESULTS Of the 97 cases diagnosed by prenatal ultrasound,49(50.5%)had isolated PLSVC and 48(49.5%)had other structural abnormalities.The differences in pregnancy outcomes and amniocentesis conditions between the two groups were statistically significant(P<0.05).No significant differences were identified between the two groups in terms of advanced maternal age and gestational age(P>0.05).According to the results of the classification statistics,the most common intrac-ardiac abnormality was a ventricular septal defect and the most common extrac-ardiac abnormality was a single umbilical artery.In the subgroup analysis,the concurrent combination of intra-and extracardiac structural abnormalities was a risk factor for adverse pregnancy outcomes(odds ratio>1,P<0.05).Additional-ly,all abnormal cytogenetic findings on amniocentesis were observed in the comorbidity group.One case was diagnosed with 21-trisomy and six cases was diagnosed with chromosome segment duplication.CONCLUSION Examination for other structural abnormalities is strongly recommended when PLSVC is diagnosed.Poorer pregnancy outcomes and increased amniocentesis were observed in PLSVC cases with other structural abnor-malities.Amniotic fluid cytogenetics of fetuses is recommended for PLSVC with other structural abnormalities.

Single lead catheter of implantable cardioverterdefibrillator with floating atrial sensing dipole implanted via persistent left superior vena cava

Persistent left superior vena cava(LSVC) is a congenital anomaly with 0.3%-1% prevalence in the general population. It is usually asymptomatic but in case of transvenous lead positioning, i.e., for pacemaker or implantable cardioverter defibrillator(ICD), may be a cause for significant complications or unsuccessful implantation. Single lead ICD with atrial sensing dipole(ICD DX) is a safe and functional technology in patients without congenital abnormalities. We provide a review of the literature and a case report of successful implantation of an ICD DX in a patient with LSVC and its efficacy in treating ventricular arrhythmias.

Peripherally inserted central catheter placement in neonates with persistent left superior vena cava: Report of eight cases

BACKGROUND Reports on peripherally inserted central catheter(PICC)placement in neonates with persistent left superior vena cava(PLSVC)are rare.The majority of PLSVC patients have no clinical symptoms or hemodynamic changes,which are usually detected during cardiac catheterization,cardiac pacemaker implantation,or PICC placement.However,in neonates with PLSVC,PICC placement can be challenging.Here,we report PICC placement in eight neonates with PLSVC.CASE SUMMARY This article introduces the concept of the“TIMB”bundle.After PICC implantation,we found PLSVC in all eight patients.The key points of care regarding PICC placement in neonates with PLSVC included“TIMB”,where“T”indicates a reasonable choice of the catheterization time,“I”refers to a retrospective analysis of imaging data before catheterization,“M”refers to correct measurement of the body surface length,and“B”indicates that the tip of the PICC is placed in the middle and lower 1/3 of the left superior vena cava under the guidance of B-ultrasound.CONCLUSION“TIMB”is a bundle for PICC placement in neonates,especially for those with PLSVC.Using this new approach can improve the first-attempt success rate of PICC placement,reveal cardiovascular abnormalities in advance,allow the selection of different measurement methods reasonably according to the puncture site,and finally,improve the accuracy of catheter positioning through the use of B-ultrasound guidance.

Transjugular intrahepatic portosystemic shunt with accidental diagnosis of persistence of the left superior vena cava

Transjugular intrahepatic portosystemic shunt (TIPSS) is considered a valid therapeutic option for the treatment of portal hypertension and its complications.The guidelines for this procedure have already been established on the basis of the normal vascular anatomy and of various technical radiological aspects.In some few rare cases,diagnosis of a congenital vascular anomaly can be made accidentally by interventional radiologists,making the procedure of the TIPSS placement extremely difficult or in some cases technically impossible.This report describes a rare vascular malformation characterized by the absence of the right superior vena cava and persistence of the left superior vena cava in a patient with a diagnosis of advanced liver cirrhosis who needed a TIPSS placement in order to control refractory ascites.

CRT-D Upgrading in a Patient with Persistent Left Superior Vena Cava and Right Superior Vena Cava Atresia Using the Novel Active-Fixation Quadripolar Left Ventricular Lead

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.

Accidental venous port placement via the persistent left superior vena cava:Two case reports

BACKGROUND Breast cancer poses a great threat to females worldwide.There are various therapies available to cure this common disease,such as surgery,chemotherapy,radiotherapy,and immunotherapy.Implantable venous access ports(IVAP,referred to as PORT)have been widely used for breast cancer chemotherapy.Venous malformations are possible conditions encountered during PORT implantation.Persistent left superior vena cava(PLSVC)is a common superior vena cava malformation.Most patients have normal right superior vena cava without affecting hemodynamics,so patients often have no obvious symptoms.CASE SUMMARY We incidentally found that two patients had PLSVC while a PORT was implanted via the internal jugular vein.Due to chemotherapy for breast cancer,PORT was successfully implanted under the guidance of ultrasound into these 2 patients.Positive chest X-ray examination after the operation showed that the catheter ran beside the left mediastinum and the end was located in the seventh thoracic vertebra.The patients had no catheter-related complications and successfully completed the course of chemotherapy.Ultrasonography found that the ratio of PORT outer diameter to PLSVC inner diameter was less than 0.45,which was in line with the recommendations of relevant literature and operating guidelines.The purpose of this article is to introduce two rare cases and review the relevant literature.CONCLUSION Correct assessment of PLSVC status and ultrasound-guided PORT placement generally does not affect breast cancer patients chemotherapy.

Transvenous defibrillator implantation in a patient with persistent left superior vena cava

Persistent left superior vena cava(LSVC) can be incidentally detected during pacemaker implantation through left pectoral side.There is technical difficulty of optimal site pacing and lead stability for right ventricle lead in such situation.We hereby report a case of successful single-chamber implantable cardioverter defibrillator(ICD) implantation in a 50 years-old male with LSVC.The practical issues related with right ventricle lead implantation and pacing/defibrillation parameters for ICD device are discussed.

PCI techniques to aid implantation of CRT-D in a senior patient with persistent left superior vena cava

Persistent left superior vena cava （PLSVC） is a rare con- genital anomaly of the vena cava, affecting about 0.5% of the general population, which poses a particular obstacle during transvenous electronic device implantation. Here, we report a successful cardiac resynchronization therapy-de-fibrillator device （CRT-D） implantation strategy in which techniques and devices for percutaneous coronary interven- tion were used via a PLSVC.

Combined Double Sleeve Lobectomy and Superior Vena Cava Resection for Non-small Cell Lung Cancer with Persistent Left Superior Vena Cava

A 65-year-old man with right central type of lung squamous carcinoma was admitted to our department.Bronchoscopy displayed complete obstruction of right upper lobe bronchus and infiltration of the bronchus intermedius with tumor.Chest contrast computed tomography revealed the tumor invaded right pulmonary artery,superior vena cava,and the persistant left superior vena cava flowed into the coronary sinus.The tumor was successfully removed by means of bronchial and pulmonary artery sleeve resection of the right upper and middle lobes combined with resection and reconstruction of superior vena cava(SVC)utilizing ringed polytetrafluoroethylene graft.To the best of our knowledge,this was the first report of complete resection of locally advanced lung cancer involving superior vena cava,right pulmonary artery trunk and main bronchus with persistant left superior vena cava.

Analysis and Prediction of Collateral Channels from Left Superior Vena Cava after Bilateral Bidirectional Cavopulmonary Anastomosis

Objectives: To analyze and predict collateral channels in bilateral bidirectional cavopulmonary anastomosisfocused on that from left superior vena cava. Methods: 41 consecutive patients who all underwent bilateral bidirectional cavopulmonary anastomosis were enrolled the study. The number, shape, size, origin and entry, and course of collateral vessels from left superior vena cava were referred for evaluation based on 41 cardiac angiographies. The influential factors on the formation of collateral vessels were identified by binary logistic regression analysis. Results: The number of the left superior vena cava with collateral vessels was larger than right one, 22 versus 8 (X2 = 10.303, P = 0.001). The medium-distal parts of left systemic vena were prone to development of collateral channel. The logic function with respect to the probability of left systemic venous collateral channel could be expressed as the linear expression of left systemic venous pressure x: 0.418x ?7.111 (X2 = 23.095, P Setting 17.0 mmHg of left systemic venous pressure as prediction cut-point to discriminate the occurrence of left collateral vessels, the predictive accuracy reached 85.7%. Conclusions: The left systemic venous pressure was an independent factor associated with left systemic venous collateral channel after bilateral bidirectional cavopulmonary shunt. It might be practically feasible to predict the development of collateral vessels according to systemic venous pressure.

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).

Side matters: An intriguing case of persistent left superior vena-cava

Persistent left superior vena cava, usually an incidental finding, is the most common thoracic vein anatomical variation draining into the coronary sinus. Central venous catheter procedures may be complicated secondary to the presence of a persistent left superior vena cava, leading to life-threatening complications such as arrhythmias, cardiogenic shock, and cardiac arrest. We present a case of persistent superior vena cava diagnosed on transthoracic echocardiogram(TTE) in a patient with congestive heart failure. A dilated coronary sinus was identified on TTE, followed by injection of agitated saline into the left antecubital vein resulting in filling of the coronary sinus prior to the right atrium-an indication of persistent left superior vena-cava. This also was confirmed on cardiac computed tomography. Such a diagnosis is critical in patients who may undergo central venous catheter procedures such as our patient's potential requirement for an implantable cardiovertor defibrillator due to severe global left ventricular systolic dysfunction. The presence of a persistent left superior vena cava should always be suspected when the guidewire takes a left-sided downward course towards the rightatrium at the level of the coronary sinus. Therefore, special attention should be paid to the imaging work-up prior to central venous catheter procedures.

复杂先天性心脏病术后残余左上腔静脉异位引流入左心房介入治疗1例

永存左上腔静脉(PLSVC)是一种较常见的先天性体静脉畸形,常常回流入右心房,无需特殊处理。PLSVC回流入左心房,形成右向左分流,导致血氧饱和度降低及矛盾栓塞,需要对其进行处理。传统外科手术结扎PLSVC是常规处理异常分流的方法,但创伤比较大,且具有损伤膈神经的风险。本病例报道介绍了1例因复杂先天性心脏病矫治术遗漏PLSVC-左心房交通未处理,术后产生左向右分流,导致右心功能不全的患者,采用国产Plug血管塞经房间隔入路成功介入封堵PLSVC。本病例报道经房间隔入路成功封堵复杂先天性心脏病矫治术后PLSVC-左心房通道伴左向右分流,表明介入封堵是这类疾病比较理想的处理方式。

肺癌患者合并永存左上腔静脉3例报道并文献复习

永存左上腔静脉(persistent left superior vena cava,PLSVC)是一种罕见的体循环血管变异,在人群中的发生率约为0.3%~0.5%[1],但是在先天性心脏病患者中,这一发生概率可达到约1.3%~11%[1-2]。PLSVC通常无血流动力学的异常,因为它多数经由冠状静脉窦回流入右心房,但由于该血管的特殊走行及其罕见性,胸外科医师可能会将其误认为主动脉旁肿大的淋巴结,并对肺癌进行错误的分期,抑或是增加手术误伤的风险。因此我们回顾了3例于我院行左侧肺癌手术的患者,在术前或术中发现存在这一变异的血管,本文对其进行详细描述,并结合文献复习,总结其发病原因、临床特点及影像学特征,以呼吁胸外科医生应对此提高认识,避免相关的临床不良事件发生。

经胸超声心动图可减少无顶冠状静脉窦综合征漏误诊率

目的分析经胸超声心动图诊断无顶冠状静脉窦综合征(UCSS)漏误诊原因,并提出相应的解决策略。方法回顾性分析2015年1月~2024年3月在中国科学技术大学附属第一医院接受手术治疗的12例UCSS患者,男性7例,女性5例,年龄3~74岁。将患者的超声心动图结果进行比较,将纳入患者分为超声确诊组和超声漏误诊组。分析患者术前超声心动图录像资料,记录并分析特异性征象,根据冠状静脉窦显示情况,分为显示清楚、可疑、模糊、未显示/显示不清4种,计算各切面的显示率,分析漏误诊原因,总结无顶冠状动脉窦的超声心动图特征。结果超声确诊组8例,诊断准确率为66.7%,超声漏诊1例,漏诊率为8%,误诊3例,误诊率为25%。在UCSS确诊患者中,显示CS的常用切面的显示率均高于漏误诊组,其中右室流入道非标准切面在所有TTE确诊的患者中均可显示,该切面在两组中差异有统计学意义(P<0.05)。结论超声医师对UCSS解剖、病理生理、血流动力学的理解以及对CS各切面的观察对于准确诊断该病至关重要。经胸超声心动图作为该病极佳的筛查和诊断方式,有助于减少该病的误诊和漏诊率。

单纯经食管超声心动图引导经皮封堵房间隔缺损合并永存左上腔静脉1例

单纯超声心动图引导经皮封堵房间隔缺损(ASD)技术成熟,解剖上要求ASD边缘距离冠状静脉窦、上下腔静脉及肺静脉开口≥5 mm,经食管超声心动图较经胸超声心动图能更准确评估缺口大小、残存边缘及解剖特点。本例患儿单纯使用经食管超声心动图引导经皮封堵ASD合并永存左上腔静脉且距离冠状静脉窦口残端缺乏成功,为此类患者的治疗提供新的思路和策略。

产前超声诊断部分型房室间隔缺损合并永存左上腔静脉1例

孕妇,31岁,孕24周,外院超声提示胎儿心脏异常;孕1产0,无特殊家族史。胎儿超声心动图(图1):右心增大;房间隔原发隔回声中断约6.2 mm,CDFI探及右向左分流,宽6.0 mm;二尖瓣少量反流,束宽2.3 mm;三血管切面见左头臂静脉于肺动脉左侧向下走行,经宽4.1mm的冠状静脉窦(coronary sinus,CS)汇入右心房;提示:胎儿房间隔缺损伴二尖瓣瓣叶裂可能,部分型房室间隔缺损?永存左上腔静脉。

左无名静脉在产前超声筛查胎儿上腔静脉先天变异中的价值

目的:探讨产前阶段左无名静脉在超声筛查胎儿上腔静脉先天变异中的价值。方法:选取2019年1月-2021年12月深圳市宝安区石岩人民医院收治的48例胎儿存在上腔静脉先天性变异的孕妇资料进行回顾性分析,48例孕妇接受系统全面的产前超声筛查,并应用彩色多普勒能量技术,对胎儿的上腔静脉先天性变异超声检查特征予以分析。结果:胎儿上腔静脉先天变异类型主要分为4类,永存左上腔36例,占75.00%;永存左上腔伴右上腔缺如1例,占2.08%;主动脉弓左后无名静脉8例,占16.67%;左无名静脉胸腺内走行3例,占6.25%。结论:在上腔静脉先天变异胎儿中,其三血管气管切面在左无名静脉超声产前筛查中图像表现的特异性较高,诊断准确率较为理想,可将其作为筛查胎儿先天上腔变异的关键评判指标和方法。