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Introduction: Sinus of Valsalva aneurysm (SOVA), a rare cardiac malformation, is usually congenital and rarely acquired and most commonly occurring in the right coronary sinus. The clinical presentation of patients with SOVA varies. It is usually asymptomatic when it has not ruptured, and when it compresses neighboring structures or ruptures, it can lead to heart failure or shock, at which point urgent surgical intervention is usually required. Rupture of the sinus of Valsalva aneurysm (RSOVA) during pregnancy is really hard to come by, especially if the clinical presentations resemble that of an acute myocardial infarction. This report describes a pregnant woman with severe chest pain and hypotension with aVR and V1 ST-segment elevation due to RSOVA., Patient Concerns: Effects of RSOVA on the fetus, disease survival, and prognosis., Diagnosis: RSOVA., Interventions: Open SOVA repair., Outcomes: The patient's blood pressure returned to normal range and clinical symptoms disappeared after the surgery. After 3 months of follow-up, the patient was hemodynamically stable without chest discomfort, and an echocardiogram showed a normal aortic sinus., Conclusion: Progressive aneurysm dilatation or rupture has a poor prognosis. A thorough history and physical examination are fundamental, with echocardiography being the initial diagnostic tool of choice, and other ancillary tests (e.g., computed tomography) being used to complement and confirm the diagnosis. Surgery remains the current treatment of choice for patients with RSOVA, while the continuation of pregnancy in pregnant patients with RSOVA remains a case-by-case measure., Competing Interests: The authors have no funding and conflicts of interest to disclose., (Copyright © 2024 the Author(s). Published by Wolters Kluwer Health, Inc.)

A 15-year-old male patient presented with a 3-year history of recurrent dizziness and headaches and was initially diagnosed with patent foramen ovale. A transcatheter closure procedure was planned and conducted under general anesthesia, utilizing ultrasound guidance through the femoral vein. Preadmission echocardiography confirmed the presence of a patent foramen ovale. However, further investigation with transesophageal echocardiography (TEE) performed under general anesthesia, revealed that the observed atrial septal anomaly was not a patent foramen ovale. Instead, real-time TEE identified it as the left atrial opening of the coronary vein. Subsequent detailed TEE tracking confirmed a rare case of coronary sinus ostium atresia with left atrial reflux of the coronary vein, leading to a significant revision of the initial diagnosis and planned treatment., (© 2024 Wiley Periodicals LLC.)

Purpose To investigate the association between the anomalous aortic origin of the right coronary artery (R-AAOCA) from the left coronary sinus with interarterial course (IAC) found at coronary CT angiography and sudden cardiac death using a large data set from five university hospitals. Materials and Methods From a total of 89 314 CCTA scans (January 2009 to December 2016) that were retrospectively collected, 316 patients with R-AAOCA from the left sinus with IAC were retrospectively collected. After excluding patients with less than 2 years of follow-up, patients who had already undergone cardiovascular surgery or intervention, and patients with arrhythmia or heart failure before undergoing coronary CT angiography, 224 patients were analyzed. Follow-up was terminated upon the occurrence of major adverse cardiovascular events (MACE). Logistic regression was used to identify clinical and radiologic information as independent predictors of MACE. Results The period prevalence of R-AAOCA from the left sinus with IAC was 0.354%. The mean age was 62.03 years, with a male-to-female ratio of 182:134. During follow-up, 19 of 224 patients (8.5%) experienced MACE, but none had sudden cardiac death. Of these cases, only seven (3.13%) were suspected of being due to R-AAOCA from the left sinus with IAC and all of them had unstable angina. Coronary artery disease was significantly associated with MACE ( P < .001), while no significant correlation was observed with radiologic features. Conclusion Sudden cardiac death was not associated with R-AAOCA from the left sinus with IAC found at coronary CT angiography. The occurrence of MACE was low, with coronary artery disease being the sole significant predictor of a patient's prognosis. Keywords: Anomalous Aortic Origin of the Right Coronary Artery, Left Coronary Sinus with Interarterial Course, Coronary CT Angiography, Sudden Cardiac Death Supplemental material is available for this article. © RSNA, 2024.

Guidewire loss is a rare complication of central venous catheterization. A 65-year-old male was hospitalized in a high-dependency unit for exacerbation of chronic obstructive pulmonary disease, pneumonia, erythrocytosis, and clinical signs of heart failure. Upon admission, after an unsuccessful right jugular approach, a left jugular central venous catheter was placed. The next day, chest radiography revealed the catheter located in the left parasternal region, with suspected retention of the guidewire, visually confirmed by the presence of its proximal end inside the catheter. The left parasternal location of the catheter and the typical projection of the guidewire in the coronary sinus, later confirmed by echocardiography, raised suspicion of a persistent left superior vena cava (PLSVC). Agitated saline injected into the left antecubital vein confirmed bubble entry from the coronary sinus into the right atrium. After clamping the guidewire, the catheter was carefully retrieved along with the guidewire without any complications. This is the first reported case of guidewire retention in PLSVC and coronary sinus. It underscores the potential causes of guidewire loss and advocates preventive measures to avoid this potentially fatal complication., Competing Interests: The authors declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article., (© 2024 Palić et al.)

Unroofed sinus is categorized into four subtypes. Types I and II represent complete unroofing with or without an LSVC, respectively [1]. Types III and IV are partial unroofing involving the mid-CS (type III) or near the LA appendage and left superior pulmonary vein (type IV) [1]. CT has advantages over echocardiography in detection of this anomaly (illustrated in this case) as well as in precise delineation of defect and associated findings (presence or absence of LSVC). Short axis reconstructions at the level of CS are helpful in diagnosis. Considerations for repair include location of CS defect, presence of LSVC and other abnormalities as well as comorbidity risks [2]., (© 2024. The Author(s), under exclusive licence to Springer Nature B.V.)

Coronary sinus (CS) anomalies, although infrequent, are increasingly diagnosed with advances in interventional procedures and imaging techniques. Most cases are asymptomatic and incidentally diagnosed. We present a case of an elderly male without comorbidities who presented with acute angina. Coronary catheterisation revealed a double-vessel disease, but incidentally, sequential angiograms captured contrast filling in the levophase of CS, revealing a giant CS. Primary percutaneous angioplasty of the right coronary artery was performed successfully. Echocardiography confirmed the aneurysm, and a CT scan showed an aneurysmally dilated CS and other coronary veins alongside a normal-sized persistent left superior vena cava draining to the right atrium through CS. CS aneurysms may lead to complications such as thrombosis, embolic events, arrhythmias and heart failure, stressing the importance of vigilant monitoring and timely intervention. This case underscores the significance of recognising CS anomalies in cardiac procedures, even when asymptomatic, for proper management., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2024. No commercial re-use. See rights and permissions. Published by BMJ.)

The development of the inflow tract is undoubtedly one of the most complex remodeling events in the formation of the four-chambered heart. It involves the creation of two separate atrial chambers, the formation of an atrial/atrioventricular (AV) septal complex, the incorporation of the caval veins and coronary sinus into the right atrium, and the remodeling events that result in pulmonary venous return draining into the left atrium. In these processes, the atrioventricular mesenchymal complex, consisting of the major atrioventricular (AV) cushions, the mesenchymal cap on the primary atrial septum (pAS), and the dorsal mesenchymal protrusion (DMP), plays a crucial role., (© 2024. The Author(s), under exclusive license to Springer Nature Switzerland AG.)

Unroofed coronary sinus syndrome is a rare congenital cardiac anomaly, involving some anatomical variations. Approximately 60% of patients with unroofed coronary sinus syndrome have a concomitant atrial septal defect, which is termed unroofed coronary sinus atrial septal defect (CSASD). The precise detection of these abnormalities has been usually difficult with conventional echocardiography, mostly due to its small and complex structures. Herein, we report a case with unroofed coronary sinus atrial septal defect, in which preoperative contrast-enhanced computed tomography (CT) was useful in the operative decision making. We successfully repaired the defective roof of the coronary sinus with a bovine patch, while eliminating the inter-atrial shunt. The patient's postoperative course was uneventful with no residual shunt.

Introduction: In this study, we present a case of directing persistent left superior vena cava drainage into the unroofed coronary sinus to the right atrium in a patient with tetralogy of Fallot and coronary sinus orifice atresia without innominate vein., Case Report: A 16-month-old boy diagnosed with tetralogy of Fallot was admitted. Following intracardiac repair, the left superior vena cava was divided from the left atrial junction, passed through under the ascending aorta and anastomosed to the right superior vena cava., Conclusion: To our knowledge, this is the first patient reported with persistent left superior vena cava, unroofed coronary sinus, coronary sinus orifice atresia, and tetralogy of Fallot. The extracardiac direct anastomosis technique was used successfully to direct persistent left superior vena cava to the right atrium without any post-procedure complications.

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.

Competing Interests: Funding Support and Author Disclosures The authors have reported that they have no relationships relevant to the contents of this paper to disclose.

Background: Right coronary artery (RCA) fistulized to the coronary sinus is rare condition in adult cardiac anomalies, and the management and operative indication are controversial., Case Presentation: We describe the case of a 45-year female patient who presented with exertional dyspnea, accompanied by intermitted lower limbs and facial edema. She was diagnosed with severe tricuspid regurgitation second to a severely dilated RCA fistulized to the coronary sinus. After multidisciplinary discussion, she underwent surgery through routine medium sternotomy, the right atrium was opened under cardiopulmonary bypass. The coronary arteriovenous fistula from the distal portion of RC to a severely enlarged coronary sinus was found. Trans-coronary sinus closure of the fistula was performed with continuous stitching and a tricuspid ring annuloplasty was done. The patient recovered uneventful post operation., Conclusion: According to current literatures, surgical treatment was adopted for this case, instead of endovascular intervention. The optimal approach for these cases should consider the heart's anatomical characteristics. But we need to be aware of the occurrence of myocardial infarction and tricuspid regurgitation in the early and late stage after operation., (© 2022. The Author(s).)

Coronary artery fistula draining into the coronary sinus is a rare vascular malformation, and its diagnosis and clinical manifestations usually occur late. We describe the case of a 72-year-old female patient with dyspnea on exertion (New York Heart Association Class III) associated with palpitations. The transthoracic echocardiogram showed significant tricuspid insufficiency. Cardiac catheterization showed aneurysm of the circumflex coronary artery and fistula of this artery draining into the coronary sinus. The patient underwent fistula ligation and tricuspid valve repair, with excellent surgical results.

Persistent left superior vena cava (PLSVC) is the most common venous anomaly with an incidence of 0.3-0.5% in the general population. Here, we report a rare case of PLSVC with anomalous atrium in a cadaver during the student's dissection session at the University of Tsukuba. In this case, the coronary sinus had merged with the right atrium to form an enlarged sac-like structure and received systemic venous flow including inflow from the PLSVC. The roof of the coronary sinus with the right atrium was thicker than that of the control cases. We further found that the distance between the sinoatrial node and the opening of the coronary sinus was slightly more than half of that in control cases. This variant appears interesting and is worth reporting for developmental and clinical consideration., (© 2021. Japanese Association of Anatomists.)

Background: Anomalous aortic origin of the left coronary artery (AAOCLA) with an extended transseptal course behind the right ventricular outflow tract (RVOT) is a rare variant that poses challenges not addressed by current surgical techniques. We utilized a novel transconal approach in 7 consecutive patients., Methods: A retrospective review was made of a prospectively collected database for consecutive patients undergoing transconal unroofing of transseptal AAOLCA. Surgical repair entails transection of the RVOT, unroofing the septal course of the AAOLCA, followed by RVOT extension with a rectangular strip of autologous pericardium. Preoperative characteristics, operative details, and postoperative course were abstracted., Results: All 7 patients identified were symptomatic. Median age was 48 years (range, 12 to 62). The AAOLCA with transseptal course was confirmed by computed tomography angiography. Three patients had provocative testing demonstrating anterolateral ischemia. Four patients underwent cardiac catheterization with intravascular ultrasound and indexed fractional flow reserve, confirming flow-limiting lesion. Importantly, 3 patients had negative provocative noninvasive testing for ischemia. Median postoperative hospital length of stay was 6 days (range, 4 to 12). No mortality or major complications occurred during a median follow-up of 0.75 years. Postoperative evaluation demonstrated anatomically patent unroofed AAOLCA with improved indexed fractional flow reserve compared with preoperative (0.59 ± 0.16 vs 0.90 ± 0.03, P = .05)., Conclusions: Complete unroofing of AAOLCA with transseptal course repaired with posterior extension of RVOT is an effective technique with excellent early outcome. Multimodality provocative testing is critical to evaluate these lesions as individual studies may be misleading. Intravascular ultrasound with indexed fractional flow reserve is clinically useful to confirm the hemodynamic significance of specific lesions., (Copyright © 2021 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: Coronary sinus (CS) ostial atresia/abnormalities prevent access to the CS from the right atrium (RA) for left ventricular (LV) lead implantation. Some patients with CS ostial abnormalities also have a small persistent left superior vena cava (sPLSVC)., Objective: The purpose of this study was to describe CS ostial abnormalities and sPLSVC as an opportunity for LV lead implantation and unrecognized source of stroke., Methods: Twenty patients with CS ostial abnormalities and sPLSVC were identified. Clinical information, imaging methods, LV lead implantation techniques, and complications were summarized., Results: Forty percent had at least 1 previously unsuccessful LV lead placement. In 70%, sPLSVC was identified by catheter manipulation and contrast injection in the left brachiocephalic vein, and in 30% by levophase CS venography. In 30%, sPLSVC was associated with drainage from the CS into the left atrium (LA). When associated with CS ostial abnormalities, the sPLSVC diameter averaged 5.6 ± 3 mm. sPLSVC was used for successful LV lead implantation in 90% of cases. In 80%, the LV lead was implanted down sPLSVC, and in 20%, sPLSVC was used to access the CS from the RA. Presumably because of unrecognized drainage from the CS to the LA, 1 patient had a stroke during implantation via sPLSVC., Conclusion: When CS ostial abnormalities prevent access to the CS from the RA, sPLSVC can be used to successfully implant LV leads. In some, the CS partially drains into the LA and stroke can occur spontaneously or during lead intervention. It is important to distinguish sPLSVC associated with CS ostial abnormalities from isolated PLSVC., (Copyright © 2021 Heart Rhythm Society. Published by Elsevier Inc. All rights reserved.)

Background: An isolated coronary sinus (CS) atrial septal defect (ASD) is defined as a CS unroofed in the terminal portion without a persistent left superior vena cava or other anomalies. This defect is rare and part of the wide spectrum of unroofed CS syndrome (URCS). Recently, several reports have described this finding. The database of New Tokyo Hospital was searched to determine the incidence of this defect. Additionally, to raise awareness of this condition, the findings from five patients with CS ASD who underwent surgical repair at New Tokyo Hospital are discussed., Case Presentation: The patients were three women and two men with an age range of 63-77 years. All patients underwent transthoracic echocardiography and computed tomography, and one underwent magnetic resonance imaging. In two patients, the defect was found unexpectedly intraoperatively; left-to-right shunting was apparent in the other three patients preoperatively. The pulmonary-to-systemic blood flow ratio ranged from 1.42 to 3.1 following cardiac catheterization, and oxygen saturation step-up was seen on the right side of the heart. Valvular regurgitation was seen in 4/5 patients with different combinations and degrees of mitral, tricuspid, and aortic valve involvement. Right atrial and ventricular dilation were seen in 4/5 patients; three patients had left atrial dilation. Three patients experienced atrial fibrillation, and one of these also experienced paroxysmal ventricular contractions. All patients underwent surgical repair, and some underwent multiple procedures. One patient who had previously undergone kidney transplantation died approximately 1 year postoperatively; the remaining four patients are currently experiencing good activities of daily living without symptoms., Conclusions: CS ASD (Kirklin and Barratt-Boyes type IV URCS) comprised 1.3% of adult congenital heart surgeries and 0.07% of adult open-heart surgeries at New Tokyo Hospital from 1999 to 2019. At New Tokyo Hospital, cardiac surgery is performed mainly for patients with acquired cardiac disease, and CS ASD is rare. Early diagnosis is important, as well as early surgical repair in symptomatic patients, especially those with blood access shunts, which may overload the heart. The case of a poor prognosis in this series is noteworthy, as similar cases have not been reported previously.

Background: Ductus venosus (DV) abnormalities may be associated with intracardiac or extracardiac deformities, chromosomal anomalies, and/or congestive heart failure. Aberrant DV connecting with the coronary sinus (CS) is rare and the prenatal diagnosis presents challenges for most examiners., Case Presentation: A 35-year-old pregnant woman, gravida 2, para 1, was referred to our center at 27 gestational weeks for a full evaluation of fetal cardiac anomalies. Transverse scans indicated normal cardiac anatomy except for a dilated CS; we then scanned sagittal planes to clarify the reasons for the CS dilatation. High-definition flow imaging (HDFI) together with radiant flow (R-flow) imaging was used to delineate the aberrant DV returning to the CS, enabling the diagnosis. Three-dimensional (3D) technology was also used to obtain color-rendered images showing the spatial relationships of the vessels involved, thus confirming the two-dimensional (2D) diagnosis. Chromosomal analysis revealed a normal karyotype. The neonate appeared healthy and the echocardiogram showed a normal cardiac anatomy except for a dilated CS with the DV closed and imperceptible., Conclusions: The aberrant course of the DV returning to the CS was clearly demonstrable by traditional 2D echocardiography using HDFI and the R-flow technique. We deem it helpful to trace the inflow of the dilated CS to make the differential diagnosis. The 3D modality might also provide additional spatial information on the associated vessels and thereby assist in prenatal diagnosis.

Background: The hypoplastic coronary sinus (CS) is a rare anomaly of the cardiac venous system, wherein some of the tributaries fail to join the CS. These tributaries usually drain into atrial chambers through dilated thebesian channels. We report the first case where the tributaries are draining into the right superior vena cava (SVC)., Case Summary: A case of ischemic cardiomyopathy with severe LV systolic dysfunction with NYHA class III symptoms was taken for CRTD implantation. CS venogram after direct cannulation from left subclavian access revealed a hypoplastic CS. The part of CS beyond the attachment of the oblique vein of the left atrium to CS (distal to the posterolateral vein) formed a common channel and was draining into the right-sided SVC. The posterolateral vein was of sufficient caliber so that an left ventricle (LV) lead could be implanted, and the CRTD procedure could be completed., Discussion: Hypoplastic CS though has no pathological significance in the normal population but for CRT it can become a significant limitation. Tributaries of CS draining into right SVC are the rarest of the finding, the channel draining most likely is a remnant of the splanchnic plexus around the embryonic foregut that usually has a temporary communication with cardinal veins during intrauterine growth. This communication somehow has persisted and resulted in a channel between coronary vein and the SVC, which may be referred to as coronary veno-cardinal vein., (© 2020 Wiley Periodicals LLC.)

Anomalous aortic origin of a left coronary artery (L-AAOCA) with an intraseptal course is a rare anomaly and can be associated with myocardial ischemia and sudden cardiac death. No surgical or medical intervention is known to improve patient outcomes. A 7-year-old boy with intraseptal L-AAOCA presented with nonexertional chest pain, syncope, and had reversible myocardial ischemia on provocative testing. The patient was started on β-blockade, following which his symptoms improved and resolved over a period of six years. A follow-up dobutamine stress magnetic resonance imaging no longer showed reversible ischemia, and cardiac catheterization with fractional flow reserve did not show coronary flow compromise.

The importance of venous structure in the heart is usually overshadowed by that of the arterial system. Coronary sinus (CS) is a part of cardiac venous apparatus and connects left atria to the right atria. Other than having role in physiological contractions of both atria, it contributes substantially to cardiac electrical conduction system. Due to unique placement and connections of the CS, it has become growing interest in clinical cardiology. It is used in cardiac resynchronization therapy with biventricular pacing, left-sided catheter ablation of arrhythmias, and administration of retrograde cardioplegia in cardiac surgery. In some individuals, CS is presented with anatomical variants. CS diverticulum is a congenital outpouching that provides muscular connection between atria and ventricle. This connection provides a suitable substrate for occurrence of arrhythmias, which even results in life-threatening events such as sudden cardiac death. Early diagnosis leads to treatment with ablation techniques, which ultimately eliminates origins of arrhythmias., (© 2020 Wiley Periodicals LLC.)

A coronary sinus (CS) interatrial connection is a rare congenital anomaly which can be in various types from atrial septal defect type unroofing CS to the total absence of the CS. The pathology usually accompanies the left superior caval vein (LSCV) draining to CS and in case of its absence directly to the left atrium (LA). We present a 53-year-old woman after surgical correction of a secundum atrial septal defect in 1974, with a CS interatrial connection and paroxysmal atrial fibrillation (PAF). She gave a history of PAF and was admitted to our clinic due to progressive exertional intolerance, peripheral edema, and mild hypoxia (SatO 2 92%) with subsequent cyanosis. Transthoracic echocardiography showed a left-to-right shunt in the posteroinferior part of the atrial septum. Computed tomography revealed a persistent LSCV draining directly into the LA, the absence of the CS, and cardiac veins draining into the LA. The right atrium (RA) and the LA were connected via a tunnel with a visible contrast passage from the left to the right side-the persisting mouth of the coronary sinus. The patient was qualified for surgical correction. A glutaraldehyde-treated autologous pericardial patch was used to construct the tunnel connecting the LSVC and the RA. The second part of the patch was used to close the atrial communication at the inferior vena cava level. The patient had an uncomplicated postoperative course and is now classified in New York Heart Association Class II., (© 2020 Wiley Periodicals LLC.)

Systemic venous anomalies can occur in isolation or in association with other congenital heart disease. A left superior vena cava draining to a roofed coronary sinus represents the most common venous variant and is usually inconsequential. Embryologic or acquired disruptions in systemic veins can be problematic due to shunting and/or venous obstruction. In this case series, we report four systemic venous anomalies in patients with atrial and ventricular situs solitus. Each case demonstrates how clinically significant right to left shunts can occur from a systemic vein to the left atrium. Diagnosis in each of these patients was delayed due to suboptimal diagnostic testing. Accurate diagnosis can be made by thorough anatomic evaluation of the presence, patency, and drainage of all systemic veins and supported with proper planning and interpretation of bubble studies.

Background: Our aim is to identify the pathognomonic anatomical markers and the best terminology to describe the cardiac malformations associated with absent or multiple spleens, which are known as asplenia or polysplenia syndromes or isomerism., Materials and Methods: We have reviewed 65 hearts with isomerism of atrial appendages of the Anatomical Collections of Congenital Heart Disease, Institute of Pathological Anatomy of the University of Padua consisting of 1800 specimens. All the hearts were classified according to sequential segmental classification., Results: The incidence of isomerism was 3.6%. Of the total, 45 hearts with isomerism of right atrial appendages showed bilateral trilobed lungs, short bronchi, and absent spleen. The atrioventricular junction was univentricular in 49% of cases with a common atrioventricular valve in 91%. Pulmonary atresia and double outlet right ventricle were present in 40% and 47% of cases, respectively. Total anomalous pulmonary venous drainage and absent coronary sinus were always present. In 20 hearts with isomerism of left atrial appendages, bilateral bilobed lungs with long bilateral bronchi and multiple spleens were always found. The biventricular atrioventricular connection was present in 65% with a common valve in 30% of the hearts. The ventriculoarterial connection was concordant in 45% of cases, and aortic atresia and pulmonary atresia were both noted in 15% of each. An anomalous symmetric pulmonary venous drainage was observed in 65% of the hearts and interruption of inferior vena cava was found in 75% of cases., Conclusions: We believe that the appropriate terminology is based on the symmetrical morphology of the atrial appendages. The absence of the coronary sinus and the total anomalous pulmonary venous drainage are the markers of isomerism of the right atrial appendages. Symmetric pulmonary venous drainage and interruption of inferior vena cava are the markers of isomerism of left atrial appendages. In recent years, thanks to the improvement of clinical diagnosis and of surgical techniques these patients have the possibility to survive to adult age., (Copyright © 2020 Elsevier Inc. All rights reserved.)

We report a case of a persistent left superior vena cava draining to the right atrium via the coronary sinus in conjunction with partial anomalous pulmonary venous return of the left pulmonary veins to the coronary sinus. Although a persistent left superior vena cava is typically of little clinical consequence, in this case, it complicated surgical repair of the congenital heart disease. Successful repair of this unusual combination of systemic and pulmonary venous anomalies required a combination of two well-described surgical techniques.

Umbilical vein anomalies are a rare congenital defect, which have been associated with absent ductus venosus, with few cases also involving a congenital diaphragmatic hernia. We describe a case of postnatal development of an anterior diaphragmatic hernia of Morgagni in a four-year-old patient diagnosed prenatally with mesocardia, absent ductus venosus with a large umbilical vein, a large secundum atrial septal defect, and patent ductus arteriosus.

Undiagnosed coronary sinus obstruction can be a source of paediatric exertional chest pain and death. We present a case series of three interesting children who developed only exertional angina from various degrees of coronary sinus stenosis or atresia. We discuss the diagnosis and management of each case and the potential pathophysiology involved with their symptoms.

Persistent left superior vena cava draining into the left atrium in the absence of coronary sinus and atrial septal defect (Raghib complex) is an uncommon anomaly of systemic venous drainage. We present a unique case of simultaneous presentation of cor triatriatum and persistent left superior vena cava draining into the left atrium in an adult female with partial AV canal and common atrium. Complex intra-atrial baffling including a procedure to redirect flow from a proximal atrial chamber was successful.

The superior vena cava is formed during the fetal period by the development of anastomoses between the right and left anterior cardinal veins, and the regression of the central part of the left anterior cardinal vein. The persistence of this part of the left anterior cardinal vein causes the formation of a left superior vena cava, which is a rare anomaly in cadaver dissection. We report the case of a persistent left superior vena cava with a normal right superior vena cava in a 95-year-old male cadaver, which was discovered during anatomical dissection for medical students at Kawasaki Medical School in 2016. The left superior vena cava was formed by the confluence of the left internal jugular and left subclavian veins and terminated in the right atrium via what would normally be the coronary sinus. The right and left superior venae cavae received intercostal veins via a right and left azygos vein, respectively. However, the right azygos vein was shorter than the normal azygos vein and received only the second to fifth intercostal veins, whereas the left azygos vein received the fifth to eleventh left intercostal veins and the sixth to eleventh right intercostal veins. We consider that the anomalies of the azygos venous system were the result of regression of right supracardinal vein and the persistence of the left supracardinal vein during development. An awareness of such variations of major thoracic veins is important for the interpretation of unusual CT images.

An eight-year-old boy with tricuspid atresia was found to have atretic coronary sinus ostium during cardiac catheterization. Single-stage extracardiac fenestrated Fontan operation was performed with surgical unroofing of the coronary sinus into the left atrium to avoid the risk of cardiac congestion.

Unroofed coronary sinus syndrome complicated by coronary sinus orifice atresia is a rare congenital anomaly. There are two alternate exits for coronary venous return: unroofed coronary sinus and persistent left superior caval vein. The coronary venous direction could be bidirectional depending on the pressure balance between the left atrium and the systemic vein. This anomaly has the risk of heart failure, paradoxical embolism, and cyanosis.