Giant coronary sinus aneurysm: an incidental discovery in a case of acute coronary syndrome.

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.

A Surgical Case of Partially Unroofed Coronary Sinus Atrial Septal Defect in an Elderly Patient Diagnosed by Preoperative Contrast-Enhanced Computed Tomography.

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.

Coronary Sinus Defect, Premature Restriction of Foramen Ovale and Cysto-Colic Peritoneal Band.

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.

Persistent left superior vena cava draining into the unroofed coronary sinus in a patient with tetralogy of Fallot and coronary sinus orifice atresia.

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.

Transcoronary sinus repair of a severely dilated and tortuous right coronary artery fistulized to coronary sinus complicated with tricuspid valve insufficiency.

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.

Left pulmonary veins draining into persistent left superior caval vein in presence of unroofed coronary sinus.

We report a case of 5-year-old girl with double outlet right ventricle with a rare combination of partial anomalous pulmonary venous drainage into a persistent left superior caval vein in the presence of an unroofed coronary sinus while highlighting its possible embryological origins and therapeutic implications.

Circumflex Coronary Artery Fistula Draining into Coronary Sinus.

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.

"Dual" drainage in cardiac partial anomalous pulmonary venous return.

Anomalous drainage of pulmonary veins into the coronary sinus is an uncommon variety of anomalous pulmonary venous return. Rarely, anomalously draining pulmonary veins may show "dual" drainage. We present the imaging findings of an infant who had dual drainage of a cardiac type of partial anomalous pulmonary venous return in the setting of unroofing of the coronary sinus which has not previously been described.

Coronary sinus atrial septal defects in adults over the past 20 years at new Tokyo hospital: case series.

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.

Prenatal diagnosis of an aberrant ductus venosus draining into the coronary sinus using two- and three-dimensional echocardiography: a case report.

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.

The combination of coronary sinus ostial atresia/abnormalities and a small persistent left superior vena cava-Opportunity for left ventricular lead implantation and unrecognized source of thromboembolic stroke.

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.

Anomalous atrium associated with persistent left superior vena cava.

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.

Beta-Blockade in Intraseptal Anomalous Coronary Artery With Reversible Myocardial Ischemia.

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 ß-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.

Early Outcomes of Transconal Repair of Transseptal Anomalous Left Coronary Artery From Right Sinus.

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.

A rare case of hypoplastic coronary sinus partially draining into right superior vena cava: A case report.

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.