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Persistent left superior vena cava (PLSVC) is a rare congenital anomaly whose prevalence is 0.3 % of general population. The majority of PLSVC drain into right atrium (RA) through the coronary sinus without clinical harm. However, in about 10 % of patients with PLSVC, it drains into left atrium (LA) causing right-to-left shunt. Here, we present a 60-year-old male patient with a PLSVC draining into LA, who developed dyspnea and desaturation depending on the body position after trans-catheter coil embolization of coronary to pulmonary artery fistulas. PLSVC draining into LA should be included in the differential diagnosis of positional desaturation.

This study aimed to review clinical features and surgical outcomes of coronary sinus orifice atresia (CSOA). From 2003 to 2015, 6 patients were diagnosed with CSOA. Median age at diagnosis was 2 years (range 0.2-73). CSOA was preoperatively diagnosed in 2 patients, intraoperatively in 3, and postoperatively in 1. Coronary venous blood flow drained into innominate vein through the left superior vena cava (LSVC) in 2, into the atrium through Thebesian veins in 2, and into both in 2. Three patients required surgical treatment. The first patient with a congenitally corrected transposition of great arteries had undergone ligation of the LSVC and a cutback of the proximal LSVC into the functional right atrium during the double-switch operation. The second patient with tricuspid atresia gradually developed hypoxia after the Fontan operation due to the increase in veno-venous shunt blood flow from the Fontan pathway to the right atrium through the LSVC and Thebesian veins, so that the LSVC was ligated and the coronary sinus was unroofed 6 years after Fontan operation. The other functional single ventricle patient had undergone ligation of the LSVC and a cutback of the proximal LSVC into the left atrium during the Fontan operation. There were no mortalities at the median follow-up of 9 years (1-11). CSOA should be suspected when the LSVC and usual-sized innominate vein were presented. Surgical treatment is required if the postoperative hemodynamics would affect coronary venous perfusion, like single ventricular palliation (244 words).

We report a case of unroofed coronary sinus (URCS) in a 42-year-old female. At an outpatient clinic, she was found to have an atrioventricular septal defect and mitral regurgitation with pulmonary hypertension, and she was transferred to our institute for surgical treatment. Both atrioventricular valves were located at the same level, and both the right atrium (RA) and right ventricle were enlarged on two-dimensional transthoracic echocardiography. Color Doppler imaging demonstrated severe mitral and tricuspid regurgitation and a left-to-right shunt from the left atrium (LA) to the RA. Although an ostium primum defect of the atrial septum was suspected, the exact position of the shunt flow was unclear. Two-dimensional transesophageal echocardiography (2D-TEE) could visualize a direct communication between the LA and coronary sinus. Three-dimensional transesophageal echocardiography (3D-TEE) clearly visualized the entire route from the coronary sinus into the LA and RA. The utility of 3D-TEE as a modality complementary to 2D-TEE in diagnosis of URCS was confirmed.

Atresia of the coronary sinus (ACS) is a rare congenital anomaly. When associated with persistent left superior vena cava (PLSVC), this defect could have no significant hemodynamic effect, and the patient might remain asymptomatic. However, vascular interventions might induce changes or complications that could show the anomaly. Appropriate management requires a good understanding of this condition. We present the first reported case of ACS and PLSVC occurring after thrombosis of the innominate vein (IV) after central venous catheter placement. The patient presented with atypical subacute chest pain and recurrent extrasystoles. Diagnosis and characterization of vascular anomalies was made by computed tomography phlebography, and the patient was successfully managed by endovascular recanalization of the IV.

Persistent left superior vena cava (LSVC) is a relatively frequent finding in congenital cardiac malformation. The scope of the study was to analyze the timing of diagnosis of persistent LSVC, the timing of diagnosis of associated anomalies of the coronary sinus, and the global impact on morbidity and mortality of persistent LSVC in children with congenital heart disease after cardiac surgery. Retrospective analysis of a cohort of children after cardiac surgery on bypass for congenital heart disease. Three hundred seventy-one patients were included in the study, and their median age was 2.75 years (IQR 0.65-6.63). Forty-seven children had persistent LSVC (12.7 %), and persistent LSVC was identified on echocardiography before surgery in 39 patients (83 %). In three patients (6.4 %) with persistent LSVC, significant inflow obstruction of the left ventricle developed after surgery leading to low output syndrome or secondary pulmonary hypertension. In eight patients (17 %), persistent LSVC was associated with a partially or completely unroofed coronary sinus and in two cases (4 %) with coronary sinus ostial atresia. Duration of mechanical ventilation was significantly shorter in the control group (1.2 vs. 3.0 days, p = 0.04), whereas length of stay in intensive care did not differ. Mortality was also significantly lower in the control group (2.5 vs. 10.6 %, p = 0.004). The results of study show that persistent LSVC in association with congenital cardiac malformation increases the risk of mortality in children with cardiac surgery on cardiopulmonary bypass. Recognition of a persistent LSVC and its associated anomalies is mandatory to avoid complications during or after cardiac surgery.

We report a rare case of a 5-year-old child who presented with chronic hypoxemia and a normal cardiac examination, and was found to have a persistent left superior vena cava draining directly into the left atrium. The coronary sinus was absent. This case introduces a contradiction to the generally accepted concept that this anomalous connection can only occur in the presence of a partly or completely unroofed coronary sinus. High index of suspicion is required to include this pathology in the differential diagnosis for a patient with persistent cyanosis with normal cardiac examination.

An 11-month-old boy with a functionally single ventricle, coronary sinus ostial atresia, and bilateral superior vena cava (SVC) underwent coronary sinus unroofing upon a right bidirectional cavopulmonary shunt. A persistent left SVC left open to guarantee dual coronary venous drainage became dilated with cephalocaudal blood flow reversal and desaturation. The left SVC was surgically ligated on postoperative day 35.

Ventricular tachycardia (VT) arising from the coronary sinus cusps and mimicking right-ventricular morphology is a rare entity. In this report, we report the successful cryoablation of left-coronary sinus cusp VT.

This report describes the unique case of a newborn with total anomalous pulmonary venous connection to an unroofed coronary sinus associated with Ullrich-Turner syndrome and aortic coarctation. Total anomalous pulmonary venous connection to the unroofed coronary sinus is an extremely uncommon cardiac abnormality. This congenital heart disease is difficult to diagnose and rarely ever reported. Few symptoms are to be expected when it is not associated with other congenital heart defects. This lesion results in a "naturally" corrected total anomalous pulmonary venous return and a coronary sinus atrial septal defect presenting as a left-to-right shunting lesion. Making the diagnosis of this cardiac defect, and if possible, surgical repair is of importance because cardiac dysfunction due to significant atrial left-to-right shunting is a known long-term complication in the older adult patient.

We describe a patient who underwent radiofrequency (RF) catheter ablation of cavo-tricuspid isthmus-dependent atrial flutter (AFL). Extensive ablation at the isthmus failed to terminate the AFL. A coronary sinus (CS) diverticulum arising from the proximal portion of the middle cardiac vein was found near the isthmus. An RF energy application at the bottom of the CS diverticulum resulted in completion of a bidirectional block line at the isthmus, as well as AFL termination.

A 2-month-old boy with a heart murmur and suspected coronary artery anomalies presented for cardiac computed tomography to delineate the origin and course of these anomalies. A single-origin coronary artery was discovered, which arose from the left coronary sinus and coursed between the two outflow vessels, giving this single origin a potentially malignant course. This vessel then went on to split into right and left coronary arteries with normal anatomic distribution. Without the ability for collateral flow, this vessel was located in an extremely precarious position prone to compression during times of stress or exercise. Very rarely has a single-origin coronary artery supplied both ventricles, and even more astonishing was the presence of its interarterial course. This patient will need long-term follow-up care and undoubtedly will be a candidate for surgery to correct this rare potentially malignant anomaly to lessen the boy's chances of sudden cardiac death.

The patient was a neonate with a large coronary artery fistula (CAF) between the left circumflex artery and coronary sinus in whom an intractable progressive heart failure with myocardial ischemia developed within 24 h after birth. He survived the surgical intervention and is doing well. This is the first report of coronary fistula presenting with cardiogenic shock on the first day of life. Additionally, this is the fourth case of the CAF between the left circumflex artery and coronary sinus in children, and the first case in a newborn.

Persistent left superior vena cava is known to be associated with cor triatriatum. The persistent left superior vena cava drains into a dilated coronary sinus and can cause left atrial outflow obstruction by impinging on the posterior wall of the left atrium. Residual obstruction can persist, even after surgical resection of the cor triatriatum membrane. We describe a novel surgical treatment in a patient with cor triatriatum and persistent left superior vena cava.

There are no research studies that comprehensively analyze, with computed tomography, the coronary sinus (CS) ostium with respect to its importance for some electrophysiological procedures paying special attention to the Thebesian valve (ThebV). Our aim was to evaluate the characteristic features of the CS anatomy, which can be useful for electrophysiologists using multislice computed tomography (MSCT). An additional aim was to create a tomographic classification of ThebV types. Included into the study were 150 patients (aged 59.7 ± 11.4; 105M) (43 with heart failure). Due to the suspicion of coronary artery disease, 64-slice MSCT (Toshiba, Aquilion 64) was performed in all patients. All measurements and the search for the ThebV were performed on multiplanar reconstructions in axial projection at 0.5-mm slice thickness. The average diameter of CS ostium was 14.2 ± 3.5 mm and the angle of entrance of the CS to the right atrium was 112° ± 11°. Seven variants of the ThebV were introduced and six of them were confirmed in this group. The following frequency of variants of ThebV was confirmed: E, 11.3%; D, 10.6%; A1, 8.7%; A2, 7.4%; C, 6.0%; B2, 2.0%. A statistically significant correlation between age and the size of CS ostium was found (r = 0.25; p < 0.05). It is possible to visualize and evaluate the CS including measurements and ThebV evaluation in MSCT. Six anatomical variants of the valve were found. MSCT can potentially provide valuable knowledge before the CS cannulation.

This report describes the preoperative diagnosis of a 6-month-old child with tricuspid atresia related to an unroofed coronary sinus. The anomaly usually is diagnosed after the Fontan operation due to persistent arterial desaturation. Preoperative recognition of this association is important. All cases of tricuspid atresia should be screened for associated persistent left superior vena cava draining into an unroofed coronary sinus.

Left ventricular (LV) function is impaired by increased afterload in neonates with severe coarctation of the aorta, which may result in endocardial fibroelastosis. Repair of the coarctation usually solves the problem, with LV function normalizing after a few weeks. This report describes a patient who underwent successful repair of critical coarctation with normalization of LV function despite signs of endocardial fibroelastosis but with persisting elevation of cardiac troponin T. Cardiac catheterization showed the rare coincidence of anomalous origin of left coronary artery from the right pulmonary artery (ALCAPA) and coronary sinus orifice atresia with left superior vena cava.

Congenital coronary artery anomalies are a well-recognized risk factor for sudden cardiac death in children as well as young adults, mostly during or immediately after intense exertion on the athletic field. Because these malformations are amenable to surgical treatment, timely identification is crucial. Unfortunately, antemortem diagnosis is notoriously difficult, partly due to the absence of abnormal test results in routine investigations. We present a 15-year-old boy who collapsed during exercise due to ventricular fibrillation. Coronary abnormalities were initially not identified, but they were clearly visualized by means of an echocardiogram and confirmed by multislice computed tomography. We would like to emphasize that echocardiography is capable of accurately identifying congenital coronary anomalies when attention is paid to the correct diagnostic hallmarks.