BACKGROUND: Mediastinal venous malformation (MVM) and fusiform superior vena cava aneurysm (F-SVCA) are both rare congenital vascular anomalies.
METHODS: A 46-year-old male presented with acute ischemic stroke of unknown etiology. Computed tomography (CT) angiography revealed the coexistence of MVM and F-SVCA. Diagnostic venography demonstrated a significant reduction in blood flow velocity within the F-SVCA, but failed to identify a direct connection to the left heart system or pulmonary vein. The patient expired due to extensive brain damage caused by a stroke.
CONCLUSIONS: This case may increase the necessity of meticulous radiological evaluation and preventive management for these anomalies, as mediastinal vascular anomalies can result in thromboembolic complications.

Superior vena cava (SVC) stenosis is rarely caused by iatrogenic trauma. Herein, the case of a 5-year-old boy who underwent radiofrequency ablation for paroxysmal supraventricular tachycardia but developed SVC stenosis and related syndromes is reported. Notably, the child exhibited an enlarged left atrial appendage that had partially breached the pericardium. Subsequent interventions involved successful removal of the stenosis, artificial vascular reconstruction, and comprehensive radiofrequency ablation of the entire right atrium, along with ligation of the left atrial appendage under direct vision. As a result, the child experienced relief from symptoms.

Greater interest in imaging the superior vena cava (SVC) in recent years has arisen because of increased focus on disorders of the right heart; the growing use of transvenous access lines, dialysis catheters, and device leads; and the emergence of right ventricular mechanical circulatory support systems via the transcatheter approach. As a low-pressure venous conduit in the right upper mediastinum, the SVC is prone to compression by various pathologic processes, to invasion by malignancies originating in nearby structures, and to complications arising from intraluminal device leads and indwelling catheters. Computed tomography and magnetic resonance venography are the modalities of choice for structural imaging of the SVC. Ultrasound allows a reasonable, yet less detailed anatomic assessment of this venous conduit. Spectral and color Doppler imaging by ultrasound are the most valuable noninvasive tools for the interrogation of SVC blood flow, a marker of the filling pattern of the right heart. Analysis of the velocity, duration, and direction of the Doppler waveforms and their phasic response to respiration makes it possible to distinguish normal from abnormal flow patterns and offers diagnostic insights into disorders that affect right heart function. The aims of this review are to demonstrate the added value SVC imaging provides during transthoracic and transesophageal echocardiographic studies, to outline its usefulness for the detection and evaluation of structural abnormalities, and to detail the role of spectral Doppler imaging in aiding the diagnosis of various disorders that affect the right heart.

BACKGROUND: Intravascular lipomas are rare occurrences, especially in major vessels. This tumour is composed of adipocytes in a fibrous capsule that has a slow growth rate and usually shows no symptoms. There were only eight reports in the literature regarding intravascular lipoma located in the superior vena cava.
METHODS: A 54-year-old man had episodes of supraventricular tachycardia and atrial flutter for over a year. Preoperative radiological findings showed a giant mass that arose from the superior vena cava to the right atrium and a biopsy catheter showed that there were no signs of malignancy. The patient then underwent surgery through median sternotomy and the mass was extirpated on the highest part of the stalk that could be reached. The patient was stable and remained to show no symptoms or evidence of residual mass or stalk in 2 years follow-up.
CONCLUSIONS: The surgical approach in excising lipoma in SVC should be considered wisely with the support of adequate preoperative diagnosis. Since lipoma is a very slow-growing tumour, extensive manipulation that could increase surgical technique difficulty or postoperative morbidity and mortality is not necessary.

Epithelioid Hemangioendothelioma (EHE) is a rare vascular neoplasm. Common locations of EHE are the bone, soft tissue, liver, and lung, but the mediastinal location is extremely rare. Few cases of mediastinal EHE, invading the Superior Vena Cava (SVC) have been reported.
We report a case of a 21-year-old man with EHE invading the SVC, which was incidentally detected on performing chest radiography. A contrast-enhanced chest Computed Tomography (CT) scan demonstrated a well-defined, oval mass located on the right side of the anterior mediastinum. The mass showed homogeneous enhancement with punctate calcifications, and it invaded the SVC at the confluence area of the right and left brachiocephalic veins.
Mediastinal EHE invading the SVC may present as a homogeneously enhancing mass with punctate calcifications. It should be added to the differential diagnosis of tumors of the mediastinum. Accurate preoperative diagnosis of EHE is critical for surgical planning; therefore, knowledge of the radiologic features of EHE is important.

Bilateral absence of the superior vena cava (SVC) is an uncommon congenital vascular anomaly, mainly asymptomatic, usually undetected, and often associated with other cardiac anomalies. Though usually harmless and totally innocent, this vascular anomaly might complicate cardiovascular surgery, the insertion of a central venous catheter and the transvenous placement of a pacemaker. This SVC anomaly is still not well known, underdiagnosed and its incidence is much higher than described. A better understanding of this anomaly and its detection could play a key role in avoiding its potential complications. We are sharing a case of a female adult, with no medical history, who presented herself to the department of visceral surgery with a collateral venous circulation of the upper thorax, that was at first, mistaken for a portal hypertension syndrome, findings were pushed to finally conclude a bilateral absence of the SVC.

Coronary artery fistulas (CAFs) represent a spectrum of abnormal connections between a coronary artery and another coronary artery, vein, or major blood vessel, known as coronary-vascular fistulas, or between a coronary artery and a cardiac chamber, known as coronary-cameral fistulas. While CAFs generally remain asymptomatic into the fifth decade of adult life, they can present with a diverse symptomatic profile, typically with angina from abnormal myocardial perfusion, or in the setting of larger fistulas, as right- or left-heart failure from pulmonary or left ventricular circulatory overload. CAFs rarely manifest as myocardial infarction in the absence of thrombosis within the fistula. When clinically suspected based on a continuous murmur on physical exam or an accidental finding on radiology, computed tomography angiography (CTA) and coronary angiography are the preferred diagnostic imaging modalities. Fistula anatomic and patient specific characteristics guide clinical decisions on transcatheter or surgical management strategies. We present the case of a right coronary artery-superior vena cava fistula manifesting as a non-ST elevation myocardial infarction. We also present a review of the imaging techniques available for evaluation of CAFs, and a summary of the major national and international cardiology society guidelines on the diagnosis and management of CAFs.

In the healthy patient, blood returns to the heart via classic venous pathways. Obstruction of any one of these pathways will result in blood flow finding new collateral pathways to return to the heart. Although significant anatomic variation exists and multiple collateral vessels are often present in the same patient, it is a general rule that the collateral pathways formed are a function of the site of venous blockage. Therefore, knowledge of typical collateral vessel systems can provide insight in localizing venous obstruction and characterizing its severity and chronicity. In addition, knowledge of collateral anatomy can be essential in interventional procedural and/or surgical planning, especially when placing catheters in patients with venous blockage. In this pictorial review, we provide a systematic approach to understanding collateral pathways in patients with venous obstruction in the upper body.

Persistent left superior vena cava (PLSVC) is encountered occasionally during angiographic procedures. It usually coexists with right superior vena cava and drains to the right atrium through the coronary sinus, but multiple variations are described. Although PLSVC is extensively reported in the literature, there are very few articles addressing right heart catheterization in patients with isolated PLSVC. We present a patient with absent right superior vena cava and PLSVC draining to a dilated coronary sinus diagnosed during right heart catheterization in the setting of pulmonary hypertension. We were able to safely complete the procedure through the right internal jugular vein. Transthoracic echocardiography and chest CT scan were consistent with this finding. Although clinically silent most of the time, undiagnosed PLSVC can lead to catastrophic consequences when the patient undergoes invasive procedures. If PLSVC is suspected, the anatomy of the thoracic venous system must be identified before invasive cardiac procedures.

BACKGROUND: Proximal or \'downhill\' esophageal varices are a rare cause of upper gastrointestinal hemorrhage. Unlike the much more common distal esophageal varices, which are most commonly a result of portal hypertension, downhill esophageal varices result from vascular obstruction of the superior vena cava (SVC). While SVC obstruction is most commonly secondary to malignant causes, our review of the literature suggests that benign causes of SVC obstruction are the most common cause actual bleeding from downhill varices. Given the alternative pathophysiology of downhill varices, they require a unique approach to management. Variceal band ligation may be used to temporize acute variceal bleeding, and should be applied on the proximal end of the varix. Relief of the underlying SVC obstruction is the cornerstone of definitive treatment of downhill varices.
METHODS: A young woman with a benign superior vena cava stenosis due to a tunneled internal jugular vein dialysis catheter presented with hematemesis and melena. Urgent upper endoscopy revealed multiple \'downhill\' esophageal varices with stigmata of recent hemorrhage. As there was no active bleeding, no endoscopic intervention was performed. CT angiography demonstrated stenosis of the SVC surrounding the distal tip of her indwelling hemodialysis catheter. The patient underwent balloon angioplasty of the stenotic SVC segment with resolution of her bleeding and clinical stabilization.
CONCLUSIONS: Downhill esophageal varices are a distinct entity from the more common distal esophageal varices. Endoscopic therapies have a role in temporizing active variceal bleeding, but relief of the underlying SVC obstruction is the cornerstone of treatment and should be pursued as rapidly as possible. It is unknown why benign, as opposed to malignant, causes of SVC obstruction result in bleeding from downhill varices at such a high rate, despite being a less common etiology of SVC obstruction.