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.

Accidental placement of venous return catheter in the superior vena cava during venovenous extracorporeal membrane oxygenation for severe pneumonia: A case report

BACKGROUND Venovenous extracorporeal membrane oxygenation(V-V ECMO)has become an important treatment for severe pneumonia,but there are various complications during the treatment.This article describes a case with severe pneumonia success-fully treated by V-V ECMO,but during treatment,the retrovenous catheter,which was supposed to be in the right internal vein,entered the superior vena cava directly in the mediastinum.The ECMO was safely withdrawn after multidiscip-linary consultation.Our experience with this case is expected to provide a reference for colleagues who will encounter similar situations.CASE SUMMARY A 64-year-old man had severe pulmonary infection and respiratory failure.He was admitted to our hospital and was given ventilation support(fraction of inspired oxygen 100%).The respiratory failure was not improved and he was treated by V-V ECMO,during which the venous return catheter,which was supposed to be in the right internal vein,entered the superior vena cava directly in the mediastinum.There was a risk of massive mediastinal bleeding if the catheter was removed directly when the ECMO was withdrawn.Finally,the patient underwent vena cava angiography+balloon attachment+ECMO with-drawal in the operating room(prepared for conversion to thoracotomy for vascular exploration and repair at any time during surgery)after multidiscip-linary consultation.ECMO was safely withdrawn,and the patient recovered and was discharged.CONCLUSION Patients may have different vascular conditions.Multidisciplinary cooperation can ensure patient safety.Our experience will provide a reference for similar cases.

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.

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.

Balloon venoplasty for disdialysis syndrome due to pacemakerrelated superior vena cava syndrome with chylothorax postbacteraemia: A case report

BACKGROUND Although superior vena cava(SVC)syndrome has also been reported as a late complication of pacemaker(PM)implantation,acute onset of SVC syndrome caused by disdialysis syndrome in patients with PM implantation is very rare.There are no specific therapies or guidelines.CASE SUMMARY A 96-year-old woman receiving dialysis was implanted with a PM due to sick sinus syndrome.She was referred to our facility for chest discomfort experienced during dialysis.Upon examination,unilateral pleural effusion on the right side was cloudy with a foul odour.The patient was diagnosed with pyothorax and treated with antibiotics.After the effusion was reduced,it gradually reaggravated and remained cloudy.In this case,SVC syndrome,which is generally considered a late complication after PM implantation,rapidly developed following the bacteraemia,resulting in impaired venous return,chylothorax,and disdialysis syndrome.After catheter intervention for SVC stenosis,the patient’s symptoms promptly improved.The patient has been recurrence-free for a year.CONCLUSION Acute SVC syndrome can cause dysdialysis in PM-implanted patients.Catheter intervention alone has improved this condition for a traceable period.

Simultaneous lateral and subxiphoid access methods for safe and accurate resection of a superior vena cava aneurysm:A case report

BACKGROUND Superior vena cava(SVC)aneurysms are a relatively uncommon disease that has not been widely reported.The conventional surgical approach for treating SVC aneurysms includes open thoracotomy and mid-sternotomy.However,in this case,the aneurysm could be safely resected by thoracoscopic simultaneous lateral and subxiphoid access methods.CASE SUMMARY A 58-year-old male presented with intermittent chest pain and persistent discomfort in the chest area.A chest computed tomography scan revealed a 6.2 cm aneurysm in the left innominate vein and SVC junction.For surgical resection,simultaneous lateral and subxiphoid access were planned to achieve optimal proximal and distal aneurysm control.The approach site was 1 cm below the xiphoid process,the fifth mid-axillary line and the seventh anterior axillary line on the right side.The aneurysm was resected using a stapler.The patient was discharged on the third day after chest tube removal on the second postoperative day with no particular issues.CONCLUSION Aneurysms located within the mediastinum can be accessed through thoracoscopic approach without open surgery and safely resected using vascular staples.

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.

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.

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.

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).

Gallbladder cancer with tumor thrombus in the superior vena cava

BACKGROUND:Gastrointestinal cancers,especially pancreatobiliary cancers,are frequently associated with or are complicated by thromboembolic phenomena due to hypercoagulability and/or altered venous drainage,especially of the abdomen and lower limbs.This report describes an unusual and interesting case of gallbladder carcinoma developing a viable tumor thrombus in the superior vena cava(SVC)with resultant SVC obstruction,while on gefitinibbased anti-epidermal growth factor receptor(EGFR)therapy.METHODS:A 60-year-old woman was incidentally diagnosed to have gallbladder cancer on cholecystectomy.She had disease recurrence and received systemic chemotherapy followed by gefitinib-based anti-EGFR therapy.Subsequently,while on gefitinib-based therapy,she presented with clinical signs and symptoms suggestive of SVC thrombosis.RESULTS:A whole body PET scan revealed a metabolically active tumor thrombus in the SVC,besides other sites of metabolically active disease inclusive of the lung parenchyma, lymph nodes and abdomen.She was treated with antithrombotics and external beam radiotherapy directed to the SVC thrombus leading to symptomatic relief.She continues to survive on the day of writing this report.CONCLUSIONS:This rare complication,though theoretically possible,is unreported because of the short overall survival of advanced gallbladder cancer patients.This highlights that with the availability of better chemotherapeutic/biotherapeutic agents for increasing in the lifespan of cancer patients,we may come across such cases more frequently in the future.

Budd-Chiari syndrome: A case with a combination of hepatic vein and superior vena cava occlusion

We here report a recent, rare case of Budd-Chiari syndrome, associated with a combination of hepatic vein and superior vena cava occlusion. A young female, who had been ingood health, was admitted to our hospital because of massive ascites. The patient had used no oral contraceptives. Tests for coagulation disorders, hematological disorders, and antiphospholipid syndrome were all negative. BuddChiari syndrome was diagnosed by radiographic examination. The patient was suffering from a combination of hepatic vein and superior vena cava occlusion. In particular, the venous flow returned from the liver mainly through a right accessory hepatic vein, and stenosis was recognized at the orifice of this collateral vein into the vena cava.Subsequently, the patient underwent percutaneous balloon dilatation therapy for this stenosis. After this treatment, the massive ascites was gradually reduced, and she was discharged from our hospital. It has now been one year since discharge, and the patient has been doing well. If deteriorating liver function or intractable ascites occur again, a liver transplantation may be anticipated. This is the first case report of Budd-Chiari syndrome associated with a superior vena cava occlusion.

Effectiveness of sharp recanalization of superior vena cava-right atrium junction occlusion

BACKGROUND Conventional recanalization techniques may fail in patients with completely occluded superior vena cava(SVC).AIM To analyze the effectiveness and complications of sharp recanalization for completely occluded SVC.METHODS This was a retrospective study of patients that underwent puncture and recanalization of the SVC between January 2016 and December 2017 at our hospital.Sharp recanalization was performed using the RUPS-100 system.The patients were followed for 12 mo.The main outcomes were the patency rate of SVC and arteriovenous fistula flow during dialysis.RESULTS The procedure was successful in all 14 patients(100%).Blood pressure in the distal SVC decreased in all 14 cases(100%)from 26.4±2.7 cmH2O to 14.7±1.3 cmH2O(P<0.05).The first patency rates of the SVC at 24 h and at 3,6,9 and 12 mo after sharp recanalization were 100%,92.9%,85.7%,78.6%and 71.4%,respectively.There were two(14.3%)severe,one(7.1%)moderate and one(7.1%)minor complication.The severe complications included one case of pericardial tamponade and one case of hemothorax.CONCLUSION The results suggest that sharp recanalization can be an additional tool to extend or renew the use of an occluded upper extremity access for hemodialysis.This could be of use in patients with long-term maintenance hemodialysis in whom the maintenance of central venous access is often a challenge.

Pulmonary hypertension concurrent with pericardial effusion and superior vena cava syndrome: who is the initiator?

The diagnosis of pulmonary hypertension(PH) should be made by combining clinical manifestations and echocardiographic probability.[1] Following the confirmation of PH, the classification should begin with the more common groups [group 2(PH due to left heart disease) and group 3(PH due to lung diseases and/or hypoxia)], then group 4(chronic thromboembolic PH and other pulmonary artery obstructions) and finally group 1(pulmonary arterial hypertension) and group 5(PH with unclear and/or multifactorial mechanisms).[1] In this case, we demonstrate a rare scenario of obstruction-caused group 4 PH.

Epicardial adipose tissue around the superior vena cava: A single center study of factors related to atrial fibrillation

The superior vena cava(SVC)is the main component of non-pulmonary vein(PV)ectopy in patients with atrial fibrillation(AF).Researchers have found that epicardial adipose tissue(EAT)volume is related to the AF substrate,which can be defined by the low voltage area(LVA).This study aimed to investigate the relationship between SVC-EAT and SVC-AF.Twenty-six patients with SVC-AF triggers were identified as the SVC-AF group.Other three groups were defined and included as the LVA-AF group(LVA>5%),non-LVA-AF group(LVA<5%),and physical examination(PE)group.EAT around left atrium(LA-EAT)and SVC-EAT volumes were obtained using a cardiac risk assessment module.According to the SVC/LA-EAT ratio,there are significant differences between the SVC-AF group and the three control groups(the SVC-AF group 0.092±0.041 vs.the LVA-AF group 0.054±0.026,the non-LVA-AF group 0.052±0.022,and the PE group 0.052±0.019,all P<0.001).Receiver operating characteristic curve analysis suggests the optimal cut-off point of SVC/LA-EAT ratio is 6.8%for detecting SVC-AF patients,with 81.1%sensitivity,73.1%specificity,and an area under the curve of 0.83(95%confidence interval,0.75-0.91).Those with SVC-AF have a higher SVC/LA-EAT ratio and empirical SVC isolation could be considered if the SVC/LA-EAT ratio was over 6.8%.

Termination of Recurrent Atrial Fibrillation by Superior Vena Cava Isolation: A Case Report

Background:Paroxysmal atrial fibrillation can be triggered by non-pulmonary vein foci,such as the superior vena cava.Here,we report the case of a patient with a 6-year history of paroxysmal atrial fibrillation who received cryoballoon ablation in 2012 but relapsed in 2014.He then received cardiac radiofrequency ablation,which successfully isolated the left pulmonary vein and superior vena cava,but the arrhythmia recently relapsed again.The tachycardia was finally successfully terminated by ablation on the free wall without recurrence during a 2-year following up.Conclusion:Superior vena cava isolation may not require ablation isolation with a full circle way and can be accomplished by ablating several connection points between the superior vena cava and the right atrium.

Giant saphenous vein graft pseudoaneurysm to right posterior descending artery presenting with superior vena cava syndrome

Saphenous vein grafts(SVG) pseudoaneurysms,especially giant ones,are rare and occur as a late complication of coronary artery bypass grafting. This condition affects both genders and typically occurs within the sixth decade of life. The clinical presentation ranges from an asymptomatic incidental finding on imaging studies to new onset angina,dyspnea,myocardial infarction or symptoms related to compression of neighboring structures. An 82-year-old woman presented with acute onset back pain,dyspnea and was noted to have significantly engorged neck veins. In the emergency department,a chest computed tomographic angiogram with intravenous contrast revealed a ruptured giant bilobed SVG pseudoaneurysm to the right posterior descending artery(RPDA). This imaging modality also demonstrated compression of the superior vena cava(SVC) by the SVG pseudoaneurysm. Coronary angiogram with bypass study was performed to establish the patency of this graft. Endovascular coiling and embolization of the SVG to RPDA was initially considered but disfavored after the coronary angiogram revealed preserved flow from the graft to this arterial branch. After reviewing the angiogram films,a surgical strategy was favored over a percutaneous intervention with a Nitinol self-expanding stent since the latter would have not addressed the superior vena cava compression caused by the giant pseudoaneurysm. Intraoperative transesophageal echocardiogram demonstrated SVCcompression by the giant pseudoaneurysm cranial lobe. Our patient underwent surgical ligation and excision of the giant pseudoaneurysm and the RPDA was regrafted successfully. In summary,saphenous vein grafts pseudoaneurysms can be life-threatening and its therapy should be guided based on the presence of mechanical complications,the patency of the affected vein graft and the involved myocardial territory viability.

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.

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.

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.