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Background : Unroofed coronary sinus is a congenital cardiac anomaly usually associated with persistent left superior vena cava. Premature restriction or closure of foramen ovale is described in association with hypoplastic left heart syndrome. Abdominal peritoneal bands when present manifest clinically. Case report: A 27 years, gravida 2, presented with intrauterine fetal death at 24 weeks gestation due to fetal congestive cardiac failure, cardiomegaly and hydrops. Perinatal autopsy showed absent coronary sinus with cardiac veins draining directly into the heart. There was no persistent left superior vena cava. The foramen ovale was restricted prematurely. The ductus arteriosus was present and non-restrictive. Abdomen showed a cysto-colic peritoneal band. Conclusion : This is the first report showing a triad of (1) complete absence of coronary sinus without left superior vena cava (type-II); (2) premature restriction of foramen ovale without hypoplastic left heart; and (3) a cysto-colic peritoneal band between the gall bladder and colon.

Background: Wolff-Parkinson-White syndrome is characterized by a short PR interval (delta-wave), long QRS complex, and the appearance of paroxysmal supraventricular tachycardia. Patients with Wolff-Parkinson-White syndrome usually have one accessory pathway, whereas cases with multiple accessory pathways are rare. Persistent left superior vena cava is a vascular anomaly in which the vein drains into the right atrium through the coronary sinus at the junction of the left internal jugular and subclavian veins due to abnormal development of the left cardinal vein. The simultaneous presence of multiple accessory pathways and persistent left superior vena cava has not been reported before., Case Presentation: A 56-year-old Japanese man with a 5-year history of palpitations was referred for radiofrequency catheter ablation due to increased frequency of tachycardia episodes in the previous 2 months. Persistent left superior vena cava was confirmed by transthoracic echocardiography and computed tomography. An electrophysiological study revealed that the accessory pathways were located in the left lateral wall, anterolateral wall, and posteroseptal region. They were completely ablated with radiofrequency energy application., Conclusions: We reported an extremely rare case of a patient with multiple accessory pathways and persistent left superior vena cava. Our case may suggest a potential embryological relationship between the multiple accessory pathways and persistent left superior vena cava., (© 2023. The Author(s).)

A 21-month-old boy was diagnosed with partial anomalous pulmonary venous connection, with the right upper pulmonary veins draining to the superior vena cava (SVC). Intraoperatively, it became evident that the right upper pulmonary veins connected to the azygous vein before draining to the SVC.

Introduction: Surgery is considered as the first-line therapeutic strategy of partial anomalous pulmonary venous connection. The Warden technique has very good short-term results. The aim of this study is to evaluate the stability of these good results over a long period of follow-up., Materials and Methods: We reviewed all patients who underwent a Warden procedure for partial anomalous pulmonary venous connection between 1997 and 2017 in our centre. A total of 73 patients were included. The median age was 14 years (5 months-72 years). Post-operative data were obtained through our hospital network. Late follow-up data were obtained through referrals, cardiologist letters and directly from the patient., Results: The mean length of follow-up was 8 years and the longest time was 22 years (range, 1-22). Twenty-five percent of our cohort had more than 10 years of follow-up. There were no cardio-vascular deaths. Eight (11.6%) patients suffered from post-operative rhythm disturbances, 5 (7.2%) of which were permanent. Two (2.9%) patients required a pacemaker implantation. At the end of the follow-up period, only one patient remained pacemaker dependent. There were no pulmonary vein obstructions. Two (2.9%) caval vein stenosis were detected, one at 7 months and the other at 7 years. These patients were treated by angioplasty alone. Five (6.8%) patients were lost to follow-up., Conclusions: The good short-term results of the Warden procedure for right partial anomalous pulmonary venous connection appear to persist in the long term, with excellent freedom from pulmonary and caval stenosis in adolescence through to adulthood.

Coronary anomalies encompass different conditions in terms of anomalous origin, coursing, and branching, which are occasionally associated with congenital heart disease. This study aimed to explore coronary anomalies associated with ventricular septal defect that was a stereotypical congenital heart disease. We retrospectively reviewed angiographic findings in patients with ventricular septal defect who required corrective surgery, and identified coronary abnormalities, including anomalous origin, coursing, and branching of coronary arteries based on angiography. We studied the prevalence and types of coronary anomalies among them. A total of 998 patients with ventricular septal defect was studied. Age and weight were 2.0 (2.1-15.2) months and 5.7 (4.1-8.7) kg, respectively. There were 115 patients (12%) with syndrome or genetic disorder, and 34 patients (3%) with extracardiac major organ anomalies. The overall prevalence of coronary anomalies was 6.2%. All coronary anomalies were anomalous origin and coursing, among 3 patients accompanied anomalous intrinsic coronary anatomy. Originating within aortic root above the Valsalva sinus was the most common coronary anomaly. Coronary anomalies were independently correlated with bicuspid aortic valve (odds ratio [OR]: 8.02, 95% confidence interval [CI]: 2.34-23.4) and persistent left superior caval vein (OR: 5.02, 95% CI: 1.93-11.7). We showed the possibility that minor cardiac variants, such as bicuspid aortic valve and persistent left superior caval vein, contribute to higher prevalence of coronary anomalies in patients with ventricular septal defect., (© 2022. Springer Japan KK, part of Springer Nature.)

Dilation of the coronary sinus is often a result of excessive volume overload from congenital anomalies of systemic venous return to the heart. These abnormalities are often discovered incidentally later in life when a patient requires cardiac imaging, cardiac catheterization, or thoracic surgery. The most common abnormality is a persistent left superior vena cava. Inferior vena cava malformation is less common, yet several different anomalies can arise. The presence of persistent left superior vena cava or inferior vena cava anomalies requires further evaluation to rule out congenital heart disease in infants. Knowledge of technically challenging systemic venous anatomy is beneficial prior to procedures necessitating central venous access such as a central line, cardiac catheterization, and intracardiac device implantation. We present an unusual case of persistent LSVC and IVC both draining directly into a severely dilated coronary sinus that was diagnosed by fetal echocardiogram and later confirmed postnatally by transthoracic echocardiogram and computed tomography angiography. To our knowledge this is the second reported case of IVC drainage into the CS and the first case that reports this as a prenatal diagnosis., (© 2022. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

A 2-month-old girl who had supracardiac total anomalous pulmonary venous connection (Darling classification type 1b) was referred to our institution. Computed tomography showed that multiple right upper pulmonary veins drained into the vertical vein, near the entry to the superior vena cava. The common pulmonary venous chamber was located lower right than usual, and right upper pulmonary veins were far from the common chamber. We successfully performed primary sutureless repair concomitant with the Warden procedure. Postoperative computed tomography showed unobstructed pulmonary veins and superior vena cava routes, and the vertical vein between right upper and lower pulmonary veins shrank slightly.

Vein of Galen aneurysmal malformation (VGAM) is a rare intracranial vascular malformation which typically presents in a critically ill newborn with intractable heart failure, severe pulmonary hypertension, and right heart dilation. We report two unusual cases of neonates with VGAM and anomalous connection of right superior vena cava to the left atrium. Both neonates were diagnosed with VGAM in utero and were clinically stable after birth with dilation of the left atrium and left ventricle and no evidence of pulmonary hypertension. One case with hydrocephalus underwent transcatheter embolization at 1 week of age. The other case without hydrocephalus underwent elective transcatheter embolization at 4 months. We postulate that the presence of a right superior vena cava to the left atrium provides a physiological advantage and counters the left-to-right shunt from the arteriovenous malformation. This provides insight to a potential treatment strategy to improve outcomes in patients with severe heart failure and pulmonary hypertension secondary to VGAM., (© 2022. This is a U.S. Government work and not under copyright protection in the US; foreign copyright protection may apply.)

Partial anomalous pulmonary venous return (PAPVR) is a rare congenital cardiovascular condition in which some of the pulmonary veins drain into the systemic circulation. We report on the cadaveric dissection of a 71-year-old Caucasian male donor who died of chronic obstructive pulmonary disease with hypertension. We noted a faint incisional scar on the thorax extending from the parasternal region at the 4th intercostal level to the midaxillary line. Since the straight-line incision followed the ribs and the scar was quite faint, surgery probably occurred when the donor was young. We also observed numerous surgical interventions of the heart, lungs, and vasculature to correct various defects. The morphology of the heart was normal, but was shifted more to the right side. An atrial septal defect (ASD) was closed with sutures. The right superior pulmonary vein that drained into the superior vena cava (SVC) was ligated close to the SVC and the right inferior, left superior, and inferior pulmonary veins all drained directly into the left atrium. We noticed a dilated coronary sinus entering the right atrium adjacent to the ASD; the ostium of the coronary sinus noticeably lacked the normal valve-like structure. We initially thought the right lung was a "horseshoe" lung, but realised that it was a "hypogenetic" lung with PAPVR and an accessory diaphragm. Compared to the left, the right secondary bronchi were much narrower and branched uncharacteristically, as seen in hypogenetic lung syndrome. The inferior lobe was highly disorganised, severely hypoplastic, and exhibited uncharacteristic morphology. The superior bronchopulmonary segment was markedly hypoplastic. The posterior and medial basal segments were not only hypoplastic and slender, but also extended like a tail to the left pulmonary cavity behind the heart/pericardium and in front of the oesophagus and aorta. The right lung, though hypoplastic, demonstrated patent bronchi and the lobes were inflatable. Based on the hypogenetic lung and PAPVR, we conclude that the donor exhibited 'scimitar' lung.

The relevance of the study is conditioned by the problem of implantation of an artificial cardiac pacemaker in atrial fibrillation in patients with tachy-brady syndrome according to the standard scheme related to the presence of a congenital anomaly, such as persistent left superior vena cava. The purpose of the study is to develop an operative method of implantation of a permanent two-chamber pacemaker in patients with tachy-brady syndrome with concomitant pathology of the persistent left superior vena cava. Research methods are the generally accepted clinical and instrumental examination of the patient, including taking anamnesis and a standard cardiological examination, electrocardiography, transthoracic echocardiography, plain radiography, angiocardiographic examination, and multispiral computed tomography, which, along with a general analysis, confirm the presence of tachy-brady syndrome with atrial fibrillation and congenital anomaly in the form of persistent left superior vena cava in patients. The study presents a developed model of surgical implantation of a permanent two-chamber pacemaker to stabilise the condition of patients with atrial fibrillation related to tachy-brady syndrome with concomitant persistent left superior vena cava; the standard implantation mechanism included the introduction of a radiopaque agent to clarify the anatomical structure of the vascular bed, further, its entry from the subclavian veins into the persistent left superior vena cava and into the cavity of the right atrium through the venous coronary sinus was detected, and then a gradual introduction of an endocardial right ventricular electrode was performed into the subclavian vein through the tricuspid valve along with its further positioning in the apex of the right ventricle; therefore, a permanent two-chamber pacemaker can be successfully installed, creating conditions for restoring sinus rhythm in this group of patients, which is of practical importance for the field of medicine (Tab. 3, Fig. 4, Ref. 20). Keywords: atrial fibrillation, persistent left superior vena cava, sick sinus syndrome, pacemaker implantation, cardiac surgery.

Exclusive drainage of superior vena cava (SVC) into the left atrium (LA) is one of the rare causes of right-to-left connection. Usually, this anomaly occurs in association with other cardiac defects, and is diagnosed in early childhood. But if it remains an isolated anomaly, this connection may be underdiagnosed and could lead to systemic manifestations. We describe a case of anomalous connection of SVC to LA with drainage of right upper pulmonary vein into SVC and its implications for the perioperative physician. The patient presented with dyspnea on exertion, cyanosis, and a past history of cerebral abscess., Competing Interests: The authors declare no conflicts of interest., (Copyright © 2022 International Anesthesia Research Society.)

The purpose of this review is to raise the index of suspicion for paradoxical embolism among generalists. The review is based solely on anecdotal reports compiled from EMBASE, MEDLINE, Googlescholar and Pubmed. Search terms were 'paradoxical embolism', 'pulmonary embolism' and 'pulmonary arteriovenous malformations'. What emerged was that right-to-left paradoxical embolism could occur with or without concurrent pulmonary embolism, and also with and without proof of the presence of an 'embolus-in-transit'. Potential sites of single or multiple systemic involvement included the central nervous system, the coronary circulation, renal arterial circulation, splenic circulation, the mesenteric circulation and the limbs. In many cases, the deep veins of the lower limbs were the source of thromboembolism. In other cases, thrombi originated from an atrial septal aneurysm, from a central venous line, from a haemodialysis-related arterio-venous shunt, from a popliteal vein aneurysm, internal jugular vein, superior vena cava, from a pulmonary arteriovenous malformation, from tricuspid valve endocarditis (with and without pulmonary embolism) and from the right atrium, respectively. Stroke was by far the commonest systemic manifestation of paradoxical embolism. Some strokes were attributable to pulmonary arteriovenous malformations with or without coexistence of intracardiac shunts. Clinicians should have a high index of suspicion for paradoxical embolism because of its time-sensitive dimension when it occurs in the context of involvement of the intracranial circulation, coronary circulation, mesenteric circulation, and peripheral limb circulation., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2022. No commercial re-use. See rights and permissions. Published by BMJ.)

Background: Partial anomalous pulmonary venous connection (PAPVC) occurs when at least one pulmonary vein drains into the right atrium or its tributaries rather than the left atrium, most commonly connecting with the superior vena cava (SVC). The Warden procedure involves transecting the SVC proximal to the uppermost connection of the pulmonary vein followed by proximal SVC reattachment to the right atrial appendage. However, descending thoracic aortic homograft replacement for SVC translocation has recently been introduced as a modified technique., Aims: This commentary aims to discuss the recent study by Said et al. who reported their experiences with six PAPVC cases undergoing a modified Warden procedure using thoracic aortic homograft SVC translocation., Methods: A comprehensive literature search was performed using multiple electronic databases to collate the relevant research evidence., Results: The Warden procedure is associated with a 10% incidence of SVC obstruction with many requiring reintervention. Meanwhile, using the aortic homograft for SVC translocation, Said et al. observed no SVC obstructions. In addition, this modified technique does not require anticoagulation and has demonstrated an improvement in long-term SVC patency. Nevertheless, it can be considered an expensive procedure. Moreover, since the thoracic aortic homograft utilized is biological tissue, only long-term follow-up will determine whether calcification and graft degeneration is an issue., Conclusion: It can be concluded that the modified Warden procedure is a safe and effective method to reconstruct the systemic venous drainage into the right atrium when a direct anastomosis under tension might be prone to re-stenosis., (© 2022 Wiley Periodicals LLC.)

Background: Warden procedure has been described for repair of partial anomalous pulmonary venous connections inserted high into the superior vena cava. In the current report, we describe a new modification., Methods: A total of six patients (five females, 83%) with a mean age of 18.8 ± 10.8 years, underwent a modified Warden procedure with a descending thoracic aortic (DTA) homograft connected to the free wall of the right atrium, rather than to the right atrial appendage in the period between June 2021 and July 2022., Results: All patients had evidence of enlarged right-sided cardiac chambers. Two patients had intact interatrial septum (33.3%), while three patients had sinus venosus (50%) and one had secundum atrial septal defects (16.7%). The procedure was performed via minimally invasive right thoracotomy in half of the patients. Patients were extubated in the operating room or soon after. No early mortality or reoperation. One patient required concomitant aortic root replacement and one needed repair of anomalous left upper pulmonary vein connection to the left innominate vein. Follow-up was complete in all patients with a mean of 6.2 ± 4.6 months. No late mortality or reoperation. All patients were discharged on antiplatelet therapy. Follow-up imaging studies showed satisfactory repair with widely patent superior vena caval and pulmonary venous pathways., Conclusions: The use of DTA homograft to facilitate superior vena caval translocation in the modified Warden procedure appears to be beneficial as it decreases tension on the anastomosis and avoids the need for anticoagulation., (© 2022 Wiley Periodicals LLC.)

Double drainage of the confluence of all four pulmonary veins is extremely rare. We present the image findings in a child with double drainage of the pulmonary venous confluence into the coronary sinus and left superior caval vein with co-existent right superior caval venous stenosis.

Background: Operative repair of partial anomalous pulmonary venous connection (PAPVC) remains challenging due to risks of sinus node dysfunction, baffle obstruction, and superior vena cava (SVC) obstruction., Methods: Traditional or modified Warden procedures were performed in 75 of 318 consecutive patients (24%) with PAPVC repaired surgically at our institution during 1993 to 2021. Clinical characteristics, echocardiography data, operative details, and early and late outcomes were collected. Cumulative incidence of reintervention and Kaplan-Meier survival analysis are reported., Results: Median age was 39 years (interquartile range, 21-57 years). Fifty-nine (79%) had normal sinus rhythm preoperatively. Seventeen (23%) had intact atrial septa. Traditional and modified Warden procedures were performed in 15 (20%) and 60 (80%), respectively. Frequent concomitant procedures included 15 (20%) tricuspid valve repairs and 12 (16%) atrial fibrillation procedures. There were no early deaths. Postoperative complications included atrial fibrillation in 17 (23%), sinus node dysfunction in 15 (20%), pneumothorax in 3 (4%), pleural effusion in 2 (3%), and pacemaker implantation in 1 (1%). At hospital discharge, sinus node dysfunction persisted in 8 (11%). Over a median follow-up of 6 years (interquartile range, 4-10 years), baffle obstruction developed in 1 patient and SVC obstruction developed in 7. None required reoperation and 6 were treated with SVC stents. At 1, 5, and 10 years, the cumulative incidence of reintervention was 5%, 7%, and 14%, and survival was 99%, 94%, and 94%, respectively., Conclusions: Traditional and modified Warden procedures can be performed with satisfactory early and late survival. Persistent sinus node dysfunction and need for permanent pacing are low. Late SVC obstruction is uncommon and can often be managed nonoperatively., (Copyright © 2022 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Persistent left superior vena cava (LSVC) that drains into the left atrium (LA) via the left superior pulmonary vein (LSPV) is a rare systemic venous drainage anomaly. It can cause cyanosis and unexplained recurrent strokes. Undiagnosed, it can seriously disrupt the conduct of the cardiopulmonary bypass (CPB), causing sudden air lock and/or flooding of the operative field with venous blood. Its connection with the LSPV outside the pericardium makes its intraoperative diagnosis difficult. We report here the case of a 48-year-old man operated for mitral and aortic valve endocarditis, complicating a Laubry-Pezzi syndrome. The opening of the LA was followed immediately by the entrance of high volume of air bubbles into the superior vena cava cannula which resulted in sudden air lock of the venous outflow line. After multiple lowerings and cessations of pump flow, partial clamping of this cannula resulted in flooding of the LA with venous blood coming from the LSPV. The heart luxation did not allow us to find the LSVC in its usual intrapericardial location, between the LSPV and the left appendage. We had to widely open the left pleura to expose its completely extrapericardial path and its communication with the LSPV. The LSVC was temporally clamped during the remainder of the surgical procedure, then ligated at both ends. The patient underwent mitral valve repair, closure of the infundibular septal defect, aortic valve replacement and tricuspid annuloplasty. He was discharged 10 days later., (© 2022 The Author(s). This is an open-access article distributed under the terms of the Creative Commons Attribution License (https://creativecommons.org/licenses/by/4.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.)

The persistent left superior vena cava is the most common venous anomaly in the systemic drainage in adults and tends to be asymptomatic. The persistent left superior vena cava causes rhythm disorders such as tachyarrhythmias or bradyarrhythmias. We report a case of persistent left superior vena cava diagnosed in a 53-year-old female patient admitted due to an acute coronary syndrome associated with unstable bradycardia. A transvenous peacemaker impressed the left atrium; therefore, a transthoracic echocardiogram was required to diagnose persistent left superior vena cava. The patient needed management with percutaneous intervention; she had an adequate evolution and subsequent discharge from the intensive care unit.

Objective To investigate fetal anomalies and pregnancy outcomes in pregnancies with persistent left superior vena cava (PLSVC) to provide assistance in prenatal counseling. Methods Cases diagnosed with PLSVC between January 2015 and January 2020 were obtained from the hospital's electronic system and were analyzed retrospectively. Results Twenty-seven cases were analyzed. The prevalence of PLSVC among congenital heart diseases (CHD) was 6.9%. Conotruncal anomalies and renal anomalies were the most common accompanying cardiac and extracardiac anomalies, respectively. Chromosomal abnormality was detected in one fetus.In the postpartum period coarctation of aorta (CoA) was found in one fetus. Conclusions When PLSVC is detected during prenatal ultrasonography, fetal anatomy should be carefully examined because of the anomalies that may accompany it. Prenatal genetic counseling should be given especially to cases with additional anomalies. In isolated cases, cardiac anatomy should be evaluated with repeated echocardiography because of the risk of CoA.

Background: Persistent left superior vena cava (PLSVC) is the most common congenital thoracic venous anomaly. It is usually found incidentally on examination or during invasive procedures. In most cases, the blood flows back to the right atrium through the coronary sinus without hemodynamic abnormalities and it is usually asymptomatic. There is some controversy regarding the clinical use of PLSVC. In a few cases, a PLSVC has been used for hemodialysis or large-bore intravenous access., Case Report: A 62-year-old woman with a previous hepatectomy for hepatocellular carcinoma and liver cirrhosis developed hepatic failure. Owing to her worsening condition, she needed liver transplantation (LT). However, a superior vena cava thrombus was found between the right atrium and proximal superior vena cava on preoperative transesophageal echocardiography. Usually, right-sided central venous catheterization is performed for LT preparation, but the embolic risk was very high in our patient. Fortunately, she had already been diagnosed with PLSVC. Therefore, we decided to perform fluoroscopy-guided catheterization through the PLSVC. For the safe use of a PLSVC catheter during surgery, the rapid infusion system pressure, coronary sinus inflow pressure, and intraoperative transesophageal echocardiography were monitored. The patient successfully underwent LT., Conclusions: Based on a literature review and this case, PLSVC can be used clinically when accompanied by a detailed history, preoperative imaging examination, and close intraoperative monitoring. We suggest that a PLSVC is a feasible alternative to central venous access for LT., (Copyright © 2022 Elsevier Inc. All rights reserved.)

We present an alternative technique for the repair of a high partially anomalous pulmonary venous connection to the superior vena cava.

Partial anomalous pulmonary venous connection is a rare congenital abnormality. We present images from an atypical case of isolated anomalous right superior pulmonary venous return to the inferior vena cava without other cardiac abnormalities.

Background: Diagnosis of sinus venosus defects, not infrequently associated with complex anomalous pulmonary venous drainage, may be delayed requiring multimodality imaging., Methods: Retrospective review of all patients from February 2008 to January 2019., Results: Thirty-seven children were diagnosed at a median age of 4.2 years (range 0.5-15.5 years). In 32 of 37 (86%) patients, diagnosis was achieved on transthoracic echocardiography, but five patients (14%) had complex variants (four had high insertion of anomalous vein into the superior caval vein and three had multiple anomalous veins draining to different sites, two of whom had drainage of one vein into the high superior caval vein). In these five patients, the final diagnosis was achieved by multimodality imaging and intra-operative findings. The median age at surgery was 5.2 years (range 1.6-15.8 years). Thirty-one patients underwent double patch repair, four patients a Warden repair, and two patients a single-patch repair. Of the four Warden repairs, two patients had a high insertion of right-sided anomalous pulmonary vein into the superior caval vein, one patient had bilateral superior caval veins, and one patient had right lower pulmonary vein insertion into the right atrium/superior caval vein junction. There was no post-operative mortality, reoperation, residual shunt or pulmonary venous obstruction. One patient developed superior caval vein obstruction and one patient developed atrial flutter., Conclusion: Complementary cardiac imaging modalities improve diagnosis of complex sinus venosus defects associated with a wide variation in the pattern of anomalous pulmonary venous connection. Nonetheless, surgical treatment is associated with excellent outcomes.

Rationale: Intramedullary spinal cord abscess (ISCA) is a rare but treatable bacterial infection of the central nervous system, and the etiology in no less than 40% of the cases is cryptogenic. Although a few cases of ISCA in individuals with a right-to-left shunt (RL shunt) have been reported, only few arguments focused on the association between RL shunt and ISCA have been provoked. The right superior vena cava (RSVC) draining into the left atrium (LA) is an uncommon systemic venous anomaly that results in an RL shunt, and this anomaly causes several types of neurological complication such as stroke or brain abscess. We report the first case of ISCA associated with RSVC-LA RL shunt., Patient Concerns: A 36-year-old man developed progressive paraparesis, dysuria, and spontaneous pain in the lumbar region and lower extremities. Spinal magnetic resonance imaging revealed an intramedullary lesion extended from Th12 to L2 with ring-shaped gadolinium enhancement. Cerebrospinal fluid (CSF) study exhibited a marked pleocytosis, and CSF culture grew Streptococcus intermedius. Cardiovascular computed tomography angiography identified RSVC-LA RL shunt, which caused transient acute cardiac syndrome due to air embolus., Diagnoses: The patient was diagnosed with ISCA associated with an RSVC-LA RL shunt., Interventions: The patient was treated with a combination of intravenous administration of meropenem and vancomycin in a daily dose of 6 and 2.5 g, respectively, followed by intravenous administration of ampicillin in a daily dose of 750 mg. The intravenous antibiotic therapy was continued for 37 days., Outcomes: A favorable neurological outcome was obtained by the intravenous antibiotic therapy, and recurrence of infection was prevented by continuous oral antibiotic therapy for 18 months., Lessons: With a literature review of ISCA associated with RL shunt, we insist that screening for RSVC-LA is beneficial to patients who are diagnosed with cryptogenic ISCA as its identification leads to appropriate preventive therapy., Competing Interests: The authors have no conflicts of interest to disclose., (Copyright © 2022 the Author(s). Published by Wolters Kluwer Health, Inc.)

We present a case of left sided inferior vena cava with hemiazygous continuation draining into the coronary sinus via the left persistent superior vena cava. This was incidentally found in an individual referred to our centre for evaluation of palpitations. These caval anomalies are rare, and are often associated with no clinical manifestations. However, it is necessary to recognize them during routine workup to avoid diagnostic and procedural pitfalls.

Aims: To investigate the abnormalities of the coronary venous system in candidates for cardiac resynchronization therapy (CRT) and describe methods for circumventing the resulting difficulties., Methods: From four implanting institutes, data of all CRT implants between October 2008 and October 2020 were screened for abnormal cardiac venous anatomy, defined as an anatomical variation not conforming to the accepted 'normal' anatomy. Patient demographics, procedural detail, and subsequent left ventricle (LV) lead pacing indices were collected., Results: From a total of 3548 CRT implants, 15 (0.42%) patients (80% male) of 72.2 ± 10.6 years in age with an LV ejection fraction of 34 ± 10.3% were identified to have had an abnormal cardiac venous anatomy over the study period. There were 13 cases of persistent left side superior vena cava (pLSVC), five of which had coronary sinus ostium atresia (CSOA) including two with an "unroofed" coronary sinus (CS); one patient had a unique anomalous origin of the CS and one patient had an isolated CSOA. In total 14 patients (60% repeat attempt) had successful percutaneous implant under general anesthesia (46.7%) via the cephalic vein (59.1%), using the femoral approach (53.3%) for levophase venography and/or pull-through, including one case of endocardial LV implant. Pacing follow-up over 37.64 ± 37.6 months demonstrated LV lead threshold between 0.62 and 2.9 volts (pulsewidth 0.4-1.5 ms) in all cases; five patients died within 2.92 ± 1.6 years of a successful implant., Conclusion: CRT devices can be implanted percutaneously even in the presence of substantial abnormalities of coronary venous anatomy. Alternative routes of venous access may be required., (© 2022 Wiley Periodicals LLC.)

Background: Prenatal diagnosis of persistent left superior vena cava is increasing, but little is known about outcomes of infants with prenatally diagnosed isolated persistent left superior vena cava., Objective: To assess the outcomes of infants with isolated persistent left superior vena cava diagnosed prenatally compared with infants with associated malformations., Methods: All cases of persistent left superior vena cava confirmed by specialized fetal echocardiography in pregnant women were included from a single-centre prospective registry. Unfavourable outcome was defined as termination of pregnancy, in utero death, postnatal death or severe genetic syndrome missed prenatally., Results: A total of 256 infants were included: 113 cases (44.1%) with isolated persistent left superior vena cava and 143 cases (55.9%) with associated malformations; respectively, 111 (98.2%) and 101 (70.6%) had a live birth. The median postnatal clinical follow-up was 3.6 years. Five-year postnatal survival with good outcome was estimated at: 100% (95% confidence interval 90.7% to 100%) in infants with isolated persistent left superior vena cava; 91.0% (74.0% to 98.1%) in infants with associated cardiac anomalies; 87.5% (51.8% to 97.3%) in infants with associated extracardiac anomalies; 81.0% (52.6 to 94.6%) in infants with both cardiac and extracardiac anomalies; and 78.9% (36.7% to 95.9%) in infants with non-structural anomalies. All genetic findings and syndromes were detected in fetuses or infants with non-isolated persistent left superior vena cava., Conclusion: Infants with isolated persistent left superior vena cava have good short-term outcomes postnatally, but persistent left superior vena cava is frequently associated with other malformations that have an effect on outcomes, which should be thoroughly searched for prenatally., (Copyright © 2022. Published by Elsevier Masson SAS.)

Background and Aim: On the basis of previously published accounts, coupled with our own experience, we have assessed the surgical approaches to patients with isomeric atrial appendages., Methods: We reviewed pertinent published studies on surgical treatment of individuals with isomeric atrial appendages, with the pertinent surgical details provided by most of the manuscripts., Results: Half of patients with right isomerism, and two-thirds of those with left isomerism have bilateral superior caval veins. Azygos extension of the inferior caval vein is reported in three-quarters of those with left isomerism. The coronary sinus is universally absent in right isomerism, along with totally anomalous pulmonary venous connection, and is absent in two-fifths of those with left isomerism. Univentricular atrioventricular connections are expected in up to three-quarters of those with right isomerism. Atrioventricular septal defect is reported in up to four-fifths, more frequently in right isomerism, with such patients typically having discordant ventriculoatrial connections or double outlet right ventricle. Reported mortalities extend to 85% for those with right, and 50% for those with left isomerism. In right isomerism, mortality is up to 54% for systemic-to-pulmonary arterial shunting, up to 75% for univentricular repair, and up to 95% for repair of totally anomalous pulmonary venous connection itself. No more than one-quarter had undergone Fontan completion, with reported mortalities of 21%., Conclusion: Early surgical results are satisfactory in patients with left isomerism, but disappointing for those with right. Recent advances in cardiac and liver transplantation may offer improved survival., (© 2022 Wiley Periodicals LLC.)

Aims: The aim of our study was to investigate the persistent left superior vena cava (PLSVC) cases diagnosed in the prenatal period in our clinic and to compare the obstetric and genetic outcomes of isolated PLSVC cases with cases accompanied by other cardiac or extracardiac anomalies., Methods: The cases diagnosed as PLSVC between January 2015 and January 2019 in our perinatology clinic were evaluated retrospectively. Patients were divided into two subgroups as isolated PLSVC and PLSVC accompanied by another anomaly. Furthermore, patients with extra anomalies were divided into three groups which are cardiac anomaly, extracardiac anomaly and those with both. The groups were compared in terms of genetic results and obstetric outcomes., Results: 89 patients were included in our study. Cases with positive pregnancy outcomes were significantly higher in the isolated PLSVC group than with extra anomaly group (p < 0.001). No karyotype anomaly was observed in the isolated group. Pregnancy results were significantly worse (postpartum demise, termination of pregnancy, in utero demise) in with both cardiac and extracardiac anomalies group (p < 0.001). There was no significant difference between the groups in terms of karyotype results (p = 0.535)., Conclusion: The diagnosis of PLSVC has gained importance and it can be made easier due to the fact that anatomic imaging can be performed in more detail. The isolated PLSVC cases have a very good prognosis. Obstetric outcomes vary according to the accompanying anomaly., Competing Interests: Declaration of Confict of interest All authors declare no conficts of interest., (Copyright © 2022. Published by Elsevier Masson SAS.)

A persistent left superior vena cava (LSVC) represents a challenging congenital abnormality for transvenous cardiac device implantation. In the current case a secondary prophylactic VDD implantable cardioverter-defibrillator (ICD) implantation was planned in a 75-year-old woman presenting with ischemic cardiomyopathy and elevated stroke risk. Since no venous communication to the right side was identified intraoperatively, the lead was placed via the persistent LSVC. The far-field signal on the floating atrial dipole could be successfully blanked out, and appropriate device function with high and stable atrial sensing was demonstrated at follow-up., (© 2022. The Author(s).)

Persistent left superior vena cava, the most common thoracic venous anomaly, may complicate transvenous lead extraction (TLE). We report a successful case of TLE in a patient with persistent left superior vena cava, despite a long dwelling time and several pocket revisions due to pocket infection. The lead was removed via a hand-powered mechanical extraction sheath, and postoperative complications did not occur. Complicated TLE cases may have a better outcome if performed in a high-volume center with experienced specialists.

An 85-year-old woman presenting with symptomatic pulmonary hypertension (PH) with a history of dextrocardia was referred to our facility for treatment of heart failure. Significant shunting was detected by measuring oxygen saturation during right heart catheterisation (RHC). CT with contrast revealed sinus venosus atrial septal defect (SVASD) and partial anomalous pulmonary venous connection (PAPVC), in which the left upper and middle lobar pulmonary veins returned to the superior vena cava and right atrium. Despite medical treatment, the patient died, and an autopsy was performed. SVASD and PAPVC are rare congenital anomalies. RHC with measurement of oxygen saturation and CT with contrast should be considered in patients with unexplained right atrial and ventricular enlargement or suspected PH., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2022. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.)

Abnormal systemic vein development produces anomalous veins, which - in the case of persistent left superior vena cava and/or left brachiocephalic vein - exhibit considerable topographic and morphometric differences in comparison with their usual anatomy. The nature and extent of those developmental anomalies - detected during intravenous procedures, such as cardiac implantable electronic device (CIED) lead insertion or central venous catheter placement - may hinder the procedure itself and/or adversely affect its outcome, both at the stage of cardiac lead advancement through an abnormally shaped vessel and lead positioning within the heart. This may lead to problems in achieving optimal sensing and pacing parameters and in ensuring that the patient cannot feel the pacing impulses. These events accompanied a de novo CIED implantation procedure in the patient with a double superior vena cava and left brachiocephalic vein agenesis, who ultimately required reoperation.

Background: Anomalies in systemic venous return most commonly involve a persistent left supe-rior vena cava draining into the left atrium. Anomalous drainage of the inferior vena cava (IVC) into the left atrium is a rare congenital vascular disorder. The diagnosis was confirmed as anoma-lous drainage of the right superior pulmonary vein and large atrial septal defect following echo-cardiography. Anomalous drainage of the inferior vena cave was confirmed with computed tomog-raphy (CT). We report a rare combination of drainage of the inferior vena cava associated with atrial septal defect (ASD) and partial anomalous pulmonary venous return., Case: A 14-year-old girl was referred to our hospital for the evaluation of palpitations, hypoxia, exertional dyspnea, and cyanosis. Transthoracic echocardiography (TTE) revealed a large sinus venosus ASD and anomalous right superior pulmonary venous return. A cardiac CT demonstrated IVC drainage to the left atrium and an anomalous right superior pulmonary vein draining into the right atrium., Conclusions: In older patients with cyanosis, further imaging methods together with TTE will be useful in detecting additional cardiac anomalies. Patients with inferior vena cava opening to the left atrium are different from caval type ASD`s and should be surgically repaired using a patch. Corrective surgery involves repositioning of the interatrial septum via a patch.

Competing Interests: Competing interests: None declared.