Lung perfusion scintigraphy is a cornerstone for evaluating pulmonary embolism but may reveal unexpected vascular anomalies. Persistent left superior vena cava, a congenital anomaly, can cause a right-to-left shunt when draining into the left atrium, bypassing pulmonary circulation. We report the incidental detection of persistent left superior vena cava during lung perfusion scintigraphy in a patient with post-COVID-19 hypoxia. Dual-site radiotracer injections and SPECT/CT confirmed the diagnosis, highlighting the need for adaptive imaging techniques to accurately interpret atypical findings in nuclear medicine.

Circular shunt is a very rare, albeit critical, condition. We present a case of a post-surgical circular shunt that resulted after correction of an anomalous total pulmonary venous connection to the coronary sinus with a persistent left superior vena cava. A left cavo-pulmonary anastomosis was performed; however, she developed heart failure during follow-up. The anastomosis was closed by interventional cardiac catheterisation without complications that led to resolution of heart failure symptoms.

Abstract: Objective: To explore the evidence-based nursing optimization strategy for catheter tip positioning during peripherally inserted central catheter (PICC) insertion in patients with persistent left superior vena cava (PLSVC). Methods: For one ovarian cancer patient with PICC malposition in the coronary sinus (CS) due to PLSVC, multi-modal imaging techniques were integrated to accurately locate the catheter tip. The catheter position was adjusted based on evidence (withdrawing 5 cm), and a standardized nursing process was established, including personalized health education, catheter fixation and displacement monitoring, complication monitoring, establishment of a specialized disease information archive system, and formulation of a follow-up plan. Results: The catheter tip was successfully withdrawn from the coronary sinus (at the T8 level) to the middle and lower part of the PLSVC (at the T6 vertebral level), and the catheter functioned normally after adjustment. No complications such as arrhythmia or thrombosis occurred during the 332-day chemotherapy period. Conclusion: The PICC tip in PLSVC patients should be positioned in the middle and lower part of the PLSVC (at the T5–T7 vertebral level). This new standard can effectively avoid CS-related complications. The integration of multi-modal imaging techniques and evidence-based nursing management are key to ensuring safe infusion.

BackgroundApproximately half of the patients with Turner syndrome (TS) have congenital or acquired cardiovascular diseases. The objectives of this study were to improve the early diagnosis of TS and to predict the risk of severe cardiovascular diseases in patients with TS by analyzing the main features of cardiovascular diseases in these patients.MethodsThis study included 107 patients with TS who underwent echocardiography and were admitted to the Shanghai Children’s Medical Center between November 2019 and November 2024. In this study, the height, weight, age, karyotype, cardiac imaging data, and electrocardiograms of the patients were collected. The main features of the cardiovascular diseases in patients with TS were assessed, and the correlations between the height, body mass index (BMI), karyotype, and cardiovascular diseases were analyzed.ResultsOverall, 107 patients with TS were included, with an average age of 9.68 ± 4.23 years at diagnosis. Bicuspid aortic valve (BAV), aortic coarctation (CoA), and persistent left superior vena cava (PLSVC) were the most prevalent cardiovascular diseases, with prevalence rates of 11.2% (12/107), 8.4% (9/107), and 9.3% (10/107), respectively. The partial anomalous pulmonary venous return (PAPVC) was of the ‘supra cardiac type’ in all cases. Patients with CoA had lower BMI-for-age Z-score (BMIAZ) (-0.81 ± 1.17 vs. 0.61 ± 0.99; P = 0.003) than those without CoA. The best cutoff for predicting CoA was calculated using a sensitivity of 0.857 and specificity of 0.697 with a BMIAZ of 0.115 and an area under the curve of 0.825 (P = 0.004). Moreover, CoA (63.3%, 7/11) was the leading cause for surgical and interventional procedures in children and adolescents with TS. The 45,X karyotype was found to be associated with congenital heart disease (CHD) (47.4% vs. 24.6%; P = 0.016).ConclusionsGirls with BAV or CoA should undergo karyotyping to facilitate the early diagnosis of TS. CoA is the leading cause for surgical and interventional procedures in children and adolescents with TS. The BMIAZ is a reliable predictor of CoA. Cardiovascular magnetic resonance (CMR) or computed tomography (CT) should be performed at the time of diagnosis for girls with TS, especially those with a 45,X karyotype, BMIAZ < 0.115, or CHD detected by echocardiography.

Ventricular septal defect (VSD) is a common congenital heart defect that often requires surgical closure. We present the case of a seven-month-old infant with a large perimembranous VSD, moderate pulmonary valve stenosis, and hypertrophied right ventricular muscle bundles who failed to wean from cardiopulmonary bypass due to severe cyanosis after VSD closure and muscle bundle resection. An intraoperative transesophageal echocardiogram was performed with an agitated saline test, and a large atrial-level right-to-left shunt was seen. Return to cardiopulmonary bypass revealed an unroofed coronary sinus defect, which had not previously been identified. There was no evidence of a persistent left superior vena cava. Closure of the orifice of the coronary sinus eliminated the shunt, enabling successful weaning from cardiopulmonary bypass, and the patient was subsequently discharged home.

This article aims to emphasize the importance of considering invasive thymoma in the differential diagnosis of mediastinal masses and highlights the critical role of timely surgical and oncological management in improving patient outcomes. We present the case of a 70-year-old woman who presented with signs of superior vena cava syndrome, including dyspnea, facial swelling, and fatigue. Advanced imaging and intraoperative findings revealed a large anterior mediastinal mass infiltrating the left brachiocephalic vein and superior vena cava, extending into both the right atrium and right ventricle. Surgical intervention was performed, and histopathological analysis confirmed B2 thymoma with a high Ki-67 proliferation index. Despite surgical intervention, the patient's condition deteriorated, and she ultimately succumbed to the disease. To the best of our knowledge, this is the first reported Bosnian case of B2 thymoma invading the brachiocephalic vein and superior vena cava and infiltrating both the right atrium and ventricle, causing superior vena cava syndrome. Despite their rarity, thymomas should always be considered in patients presenting with an enlarged mediastinum.

Background: The incidence of persistent left superior vena cava (PLSVC) in the general population is estimated to be between 0.3% and 0.5%, which equates to approximately 1 in every 200 to 325 healthy individuals. Aims: To highlight the technical challenges posed by this congenital venous anomaly during cardiac pacing procedures, particularly in venous access and lead positioning. To raise awareness of the need for a multidisciplinary approach, involving cardiology, cardiovascular imaging, and cardiothoracic surgery, to ensure safe and effective management of such patients. Case Presentation: A 67-year-old male with a history of symptomatic bradycardia presented with episodes of severe sinus bradycardia at 40 bpm, sinus pauses lasting up to 2.9 seconds, and isolated ventricular extrasystoles, as documented on a two-month Holter monitor. He was admitted to hospital following a nocturnal syncopal episode and an electrocardiogram (ECG) showing a heart rate of 20 bpm. During initial management, there was difficulty obtaining venous access and advancing a temporary pacemaker lead via the jugular vein. As a result, a temporary pacemaker was implanted via the right femoral vein, and the patient was evaluated for permanent pacemaker implantation. Decision-Making: During the procedure in the cardiac catheterisation laboratory, difficulties were encountered in advancing the guidewire towards the subclavian vein. Consequently, bilateral subclavian vein angiography was performed, which revealed a persistent left superior vena cava with anomalous drainage into the coronary sinus. The procedure was therefore deferred. A cardiac CT angiogram confirmed the diagnosis. The cardiothoracic surgery team was subsequently consulted, and an epicardial pacemaker was successfully implanted via a subxiphoid approach in DDDR mode. Conclusion: Persistent left superior vena cava is a rare venous anomaly that can pose significant technical challenges during pacemaker implantation, particularly in patients with atrioventricular block. A thorough pre-procedural assessment of venous anatomy is essential for selecting the most appropriate implantation strategy and minimising procedural risks.

Background: Persistent left superior vena cava (PLSVC) is a rare congenital venous anomaly that can complicate the anatomy of the right atrium and coronary sinus, posing technical challenges during catheter ablation of atrioventricular nodal reentrant tachycardia (AVNRT). Optimal ablation strategies in this subset remain poorly defined. Methods: We retrospectively analysed 11 patients with PLSVC who underwent typical AVNRT ablation at our centre between 2015 and 2024, identified from a cohort of 2,030 ablations (0.54%). PLSVC was suspected by dilated coronary sinus on transthoracic echocardiogram and confirmed by coronary sinus catheter manipulation or coronary sinus venography. All procedures were performed using the Claris EP Workmate system with right femoral vein access. Due to unstable His catheter positioning from distorted anatomy, fluoroscopic landmarks in the left anterior oblique (LAO 30°) view were used in review screen during radiofrequency ablation. Radiofrequency ablation (RFA) was delivered using a 4-mm non-irrigated Blazer™ catheter (max 40W, 60°C) targeting the slow pathway, guided either by low-amplitude electrograms or anatomical landmarks—primarily the anterior lip of the mid coronary sinus ostium (CSOS). A long sheath (SR0) was used in 5 patients for better catheter stability. Results: The mean age was 38 ± 11 years; 8 were female. Typical AVNRT was inducible in all 11 patients. Immediate procedural success was achieved in 100%, defined by non-inducibility of AVNRT post-ablation with and without isoproterenol. Junctional ectopy during energy delivery was used as a surrogate marker when slow pathway potentials were absent (8/11 patients). Long-term success at 24-month follow-up was 91%, with one recurrence. There were no major complications. In 3 patients, the slow pathway was localized to the posteroinferior septum; in the remainder, ablation was successful within the coronary sinus ostium. Conclusion: In patients with PLSVC, right-sided ablation of typical AVNRT remains effective, despite anatomical distortion. Atypical anterior ablation targets mostly within the Coronary sinus ostium, fluoroscopic guidance, and long sheath support facilitate successful outcomes. Awareness of these adaptations may reduce recurrence and procedural risk in this challenging subset.

Persistent left superior vena cava (PLSVC) is a rare congenital anomaly, present in &lt;1% of the population, typically draining into the coronary sinus and right atrium. When an anomalous drainage occurs into the left atrium—directly or via an unroofed coronary sinus—a right-to-left shunt may result, detected by an agitated saline (“bubble”) study from a left-sided vein. We present a rare case mimicking such a shunt. A 33-year-old man with ESRD on hemodialysis was admitted with subacute cough and weight loss, diagnosed with necrotizing MSSA pneumonia. His hospitalization was complicated by respiratory failure, septic shock, and transient effusive-constrictive pericarditis. TTE revealed an EF of 50–55%, normal RV function, small pericardial effusion, mild aneurysmal interatrial septum, and an unexpected bubble study finding: agitated saline injected via a left-arm IV resulted in bubbles first appearing in the left atrium via pulmonary veins (Figure 1), with robust left-sided opacification and delayed right-sided filling. No interatrial transit of bubbles was observed. CT angiography revealed chronic right SVC occlusion with extensive venous collaterals draining into the pulmonary veins (Figure 2), mimicking a PLSVC with unroofed coronary sinus. The SVC occlusion likely resulted from years of central venous catheterizations for dialysis. Thus, common sequelae of chronic illness produced a pattern resembling rare congenital anomalies. This case highlights the importance of careful interpretation of bubble studies, awareness of PLSVC mimickers, and the diagnostic value of multimodality imaging to avoid misdiagnosis, unnecessary testing, and patient burden. A high index of suspicion, combined with correlation across imaging modalities, is critical for accurate diagnosis and optimal patient care.

Atrial fibrillation occurs in a growing number of adults with congenital heart disease (ACHD); however, recommendations for procedural considerations are lacking. A 43-year-old woman with surgical repair of sinus venosus ASD and partial anomalous pulmonary venous return and history of atrial arrhythmias and sinus node disease presents for repeat ablation procedure for persistent atrial fibrillation. Echocardiogram two days prior to ablation procedure demonstrated no SVC stenosis, known left SVC with dilated coronary sinus (CS), and moderate LV systolic dysfunction, presumed due to persistent atrial fibrillation with tachycardia. The AF ablation was performed with intracardiac echo guidance of trans-septal puncture. All four pulmonary veins were confirmed to be isolated from prior ablation. Right and left atria were mapped during atrial fibrillation using Volta Medical to identify dispersion areas, which identified areas of spatial dispersion in the posterior wall and coronary sinus, for which posterior wall isolation was performed with CS ablation set. AF was converted to AFL with ablation and patient was cardioverted to junctional rhythm, her known baseline rhythm. At conclusion of the study no pericardial effusion was noted by ICE. Post-procedure, the patient continued to have junctional rhythm and hypotension requiring vasoactive support; limited bedside echo demonstrated no effusion. Due to persistent hypotension, additional echo imaging was performed which demonstrated no pericardial effusion, normal LV systolic function; however, an echo lucency posterior to aorta with compression of left atria was seen. CT scan confirmed a hematoma surrounding LA, 7.2 x 3.3 cm, with compression on left superior vena cava. No surgical intervention was performed due to contained hematoma and clinical stability, as surgical evacuation of the hematoma would increase the risk of bleeding. Repeat CT at 7 days demonstrated stable mediastinal hematoma of 7.2 x 3.3 cm. The patient continues to be monitored clinically with serial imaging. In ACHD patients, ablation complicated by perforation may be underrecognized due to prior surgical scarring, which may contain bleeding and obscure findings on limited imaging due to lack of circumferential effusion. Heightened vigilance and tailored procedural and post-procedural imaging strategies are necessary for ablation procedure in ACHD patients, considering their unique anatomy and surgical history

Background: Local anesthesia is considered safe and commonly used in the pacemaker implantation procedure. However, it carries a risk of local anesthetic systemic toxicity (LAST). Although rare, LAST occurrence can be fatal. Case Presentations: We reported a case of a 69-year-old woman with fatigue and dizziness. The patient's ECG indicates atrial fibrillation with total AV block. Pacemaker implantation was performed under local anesthesia. Within minutes of lidocaine injection, the patient developed a seizure followed by cardiac arrest. Immediate administration of intravenous midazolam and cardiopulmonary resuscitation was done, resulting in the patient's stability. A permanent pacemaker was successfully implanted using a modified hand-shaped stylet to accommodate the presence of a persistent left superior vena cava (PLSVC). Brain computed tomography scan showed only mild brain atrophy. No clinical symptoms were documented during the outpatient visit after discharge. Conclusions: Local anesthetic systemic toxicity during pacemaker implantation is a rare but potentially catastrophic. Prompt recognition of its signs and appropriate management are crucial. In this case, a persistent left superior vena cava posed additional challenges to the pacemaker implantation. Keyword : Lidocaine toxicity; Local anesthesia systemic toxicity; Pacemaker implantation; Persistent left superior vena cava; Venous anomaly.

Congenital heart defects (CHD) are among the most frequent congenital anomalies and represent a significant source of morbidity and mortality in infants. Both genetic and environmental factors are involved, and variants in the MYH6 are potential major genetic factors. The objective of the present study was to describe cardiac phenotypes in individuals with CHD and MYH6 variants. We included individuals with CHD and a MYH6 variant identified in four French genetics laboratories. Clinical and family data were collected. Of the 29 individuals included, 20 (68.9%) had a principal phenotype of left heart defect: hypoplastic left heart syndrome (n = 11, 37.9%), left heart obstruction at multiple sites (n = 6, 20.7%), and coarctation of aorta (n = 3, 10.3%). Nine individuals (31%) had other CHD, such as tetralogy of Fallot, pulmonary stenosis, and septal defects. Prevalence of persistent left superior vena cava (LSVC) was much higher here (n = 11, 37.9%) than in literature (0.31-5.9%). Most of the 19 MYH6 variants were heterozygous, missenses, and inherited. Family screening demonstrated incomplete penetrance and variable phenotypic expressivity. MYH6 variants are associated with various CHD with a predominance of left heart defects and persistent LSVC. Our findings support broad indication for the molecular screening of MYH6 in CHD-particularly when familial recurrence is uncertain.

While meso/dextrocardia, a cardiac axis abnormality, is associated with various complications and a poor prognosis, few studies have been reported. We aimed to identify and review patients at our hospital who had been diagnosed with fetal meso/dextrocardia. The medical records of 29 patients diagnosed with fetal meso/dextrocardia between April 1, 2014 and March 31, 2024 were reviewed. We identified eight cases of mesocardia and 21 cases of dextrocardia (17 dextropositions and four dextroversions). Right lung hypoplasia (including 3q trisomy, esophageal bronchopleural fistula, and left pulmonary artery sling) was identified in three cases. Five cases of persistent left superior vena cava (PLSVC) were identified [isolated PLSVC (n = 2), VACTERL association (n = 1), trisomy 13 (n = 2)]. Dextroposition was linked to congenital pulmonary airway malformation (eight cases), left pulmonary sequestration (one case), congenital diaphragmatic hernia (six cases), right lung hypoplasia (one case), and VACTERL association with right lung aplasia and esophageal atresia (one case). Dextroversion was associated with asplenia syndrome (two cases), single-ventricle (one case), and Temple syndrome with PLSVC and bilateral hypoplastic pulmonary arteries (one case). Among 29 newborns, six (20.7%) died during the early neonatal period and seven (24.1%) required postnatal multidisciplinary treatment, highlighting a poor prognosis in many cases. While some patients, such as those with isolated PLSVC, had favorable outcomes, several cases involved severe complications requiring intensive perinatal management. When fetal meso/dextrocardia is detected, it is critical to evaluate fetal anomalies comprehensively and not limit assessment to the heart and lungs.

ABSTRACTIn patients with vascular anomalies (e.g., isolated persistent left superior vena cava [IPLSVC]), real‐time ultrasound guidance during central venous catheter (CVC) placement reduces immediate procedural risks but cannot prevent delayed vascular perforation. Post‐procedural imaging remains critical to verify catheter tip position and mitigate complications.

Double-outlet right ventricle is a complex congenital cardiac anomaly in which both great arteries arise predominantly from the right ventricle. Accurate anatomical evaluation is critical for surgical planning. While transthoracic echocardiography is commonly used for intracardiac assessment, its limitations in visualising extracardiac structures highlight the need for additional imaging modalities like CT angiography. To compare the diagnostic accuracy and clinical utility of transthoracic echocardiography and CT angiography in the pre-operative evaluation and surgical planning of infants with double-outlet right ventricle. This retrospective, single-centre study included 78 infants diagnosed with double-outlet right ventricle. All patients underwent both transthoracic echocardiography and CT angiography before surgical intervention. Imaging findings were compared with intraoperative surgical results to assess sensitivity, specificity, positive predictive value, negative predictive value, and overall accuracy for each modality. CT angiography was significantly more effective than transthoracic echocardiography in identifying extracardiac anomalies such as coronary artery anomalies (p = 0.014), aortic arch hypoplasia (p = 0.024), aortopulmonary collateral vessels (p = 0.039), anomalous pulmonary venous connections (p = 0.001), and persistent left superior vena cava (p = 0.001). In contrast, transthoracic echocardiography was more sensitive in detecting small ventricular septal defects (p = 0.035). Transthoracic echocardiography remains the first-line modality for intracardiac evaluation in double-outlet right ventricle, but CT angiography provides superior visualisation of extracardiac structures. The combined use of both imaging techniques improves diagnostic accuracy and surgical planning, supporting better outcomes in the management of CHD.

Successful isolation of persistent left superior vena cava using very high-power short-duration radiofrequency ablation: Case Report

Intracardiac Echocardiography-Guided CS Lead Placement via Left Axillary Vein Access in a Patient with Persistent Left SVC and Absent Right SVC

The vestigial fold in humans: Characterization of a potential novel target for selective cardiac sympathetic denervation.

Left superior vena cava draining into left atrium with atresia of right superior vena cava: A case report

BackgroundBicuspid aortic valve (BAV) represents a prevalent form of congenital heart disease. The NOTCH1 gene is implicated in the pathogenesis of BAV, and congenital valve anomalies caused by variants in this gene are classified as Aortic Valve Disease 1 (AOVD1), which is inherited in an autosomal dominant pattern.MethodsEchocardiographic data and peripheral blood samples were collected from a Chinese family manifesting a clinical phenotype characterized by various cardiovascular abnormalities, including BAV. Exome sequencing target enrichment technology was performed to identify candidate genes and variants. This was followed by family segregation analysis using Sanger sequencing.ResultsA novel nonsense variant, c.2266G > T (p.Glu756Ter), was identified in the NOTCH1 gene (NM_017617.5) within this family. Individuals harboring the p.Glu756Ter pathogenic variant (III4, II7, II9, and I2) exhibited BAV – associated phenotypes. Conversely, the proband carrying p.Glu756Ter pathogenic variant (IV1) presented with distinct clinical phenotypes, including a persistent left superior vena cava (PLSVC) and a widened coronary sinus. Family members without the p.Glu756Ter pathogenic variant showed no cardiovascular abnormalities. This variant is located in exon 14 of the NOTCH1 gene, resulting in a premature stop codon. Bioinformatics analysis predicted that p.Glu756Ter induces nonsense-mediated mRNA decay or produces a truncated protein, impairing NOTCH1 receptor function.ConclusionsThe NOTCH1 variant is a prevalent genetic factor in BAV etiology, exhibiting both interfamilial and intrafamilial phenotypic variability with incomplete penetrance. PLSVC and a widened coronary sinus represent common anatomical variations within the general population. Nevertheless, the potential role of NOTCH1 variants in the development of these vascular anomalies should be considered.