Efficient approach to superior vena cava baffle stenosis following the Mustard procedure: Expanding the role of a mechanical rotating dilator sheath for lead extraction.

INTRODUCTION: In adult congenital patients with transposition of the great arteries originally treated with the Mustard (atrial switch) procedure, the most common reason for re-intervention is baffle stenosis. This may be exacerbated by permanent transvenous pacemaker lead placement across the baffle. CASE REPORT: A 47-year-old female status post Mustard procedure performed at 15 months old presented with a high-grade stenosis of the superior vena cava (SVC) baffle from the SVC to the left atrium, with a nonfunctional permanent pacemaker lead passing through the baffle. A mechanical rotating dilator sheath was used for attempted lead extraction, relieving the baffle stenosis almost completely as a secondary effect, before the placement of a 10 × 27 mm Visipro balloon-expandable stent in the SVC baffle. CONCLUSIONS: Use of the mechanical rotating dilator sheath is an evolving treatment strategy in adult congenital heart disease to minimize the risk of bleeding, trauma to surrounding structures, and death. Its ability to fully alleviate baffle stenosis even when full lead extraction is not feasible or is associated with significant procedural risk, further demonstrates its expanded role in this patient population. A multidisciplinary approach and great diligence must be employed to avoid potential complications.

Successful venoplasty of superior vena cava stenosis in a patient with a total artificial heart after orthotopic heart transplantation due to primary graft failure.

BACKGROUND: With the limited number of available suitable donor hearts resulting in plateaued numbers of heart transplantations, short- and long-term mechanical circulatory support devices, including the implantation of total artificial hearts (TAHs) are modalities that are increasingly being used as treatment options for patients with end-stage heart failure. The superior vena cava syndrome has been described in this context in various disease processes. We report successful venoplasty for superior vena cava syndrome in a patient with a TAH. CASE PRESENTATION: A 65-year-old man with a history of nonischemic cardiomyopathy had received a left ventricular assist device, and then 2 years later, underwent orthotopic heart transplantation using the bicaval anastomosis technique. The postprocedural course was complicated by primary graft failure, resulting in the need for implantation of a TAH. About 5 months after TAH implantation, he started to develop complications such as volume retention, swelling of the upper extremities, and was diagnosed to have a superior vena cava syndrome. The patient underwent a successful venoplasty of his superior vena cava by interventional radiology with resolution of upper body edema, normalization of renal, and liver function. CONCLUSION: Potential fatal complications caused by catheter or wire entrapment in the right-sided mechanical valve of a TAH have been reported. We describe a safe method for the treatment of superior vena cava syndrome in patients with TAH.

Congenital systemic venous drainage obstruction: A case report.

BACKGROUND: Vena cava anomalies are rare congenital defects due to incorrect development during fetal life, ranging from minor asymptomatic anatomic variations to complex life-threatening abnormalities. Echocardiography plays a fundamental role in the diagnosis, with advanced imaging techniques allowing detailed anatomic delineation. Invasive cardiology techniques are a promising therapeutic approach, but surgery is probably the best option when diffuse compromise of the systemic veins is present. CASE REPORT: An 8-month-old infant presenting episodes of labial cyanosis and a failure to thrive was diagnosed with severe superior vena cava (SVC) stenosis at the right atrium ostium, with decompression via azygos vein and mild inferior vena cava (IVC) stenosis. The patient was referred for surgery, with IVC enlargement and removal of a fibromuscular tissue band on the anterior aspect of the SVC ostium. At a 6-month follow-up, the patient is asymptomatic, with excellent surgical outcome. To the authors' knowledge, only two cases of congenital SVC stenosis have been previously described.

Management of superior vena cava syndrome in critically ill cancer patients.

PURPOSE: The purpose of this study is to describe the management and outcome of critically ill cancer patients with Superior Vena Cava Syndrome (SVCS). METHODS: All cancer patients admitted to the medical intensive care unit (ICU) of the Saint-Louis University Hospital for a SVCS between January 2004 and December 2016 were included. RESULTS: Of the 50 patients included in the study, obstruction of the superior vena cava was partial in two-thirds of the cases and complete in one-third. Pleural effusion was reported in two-thirds of the patients, pulmonary atelectasis in 16 (32%), and pulmonary embolism in five (10%). Computed tomography of the chest showed upper airway compression in 18 (36%) cases, while echocardiography revealed 22 (44%) pericardial effusions. The causes of SVCS were diagnosed one (0-3) day after ICU admission, using interventional radiology procedures in 70% of the cases. Thirty (60%) patients had hematological malignancies, and 20 (40%) had solid tumors. Fifteen (30%) patients required invasive mechanical ventilation, seven (14%) received vasopressors, and renal replacement therapy was implemented in three (6%). ICU, in-hospital, and 6-month mortality rates were 20, 26, and 48%, respectively. The cause of SVCS was the only factor independently associated with day 180 mortality by multivariate analysis. Patients with hematological malignancies had a lower mortality than those with solid tumors (27 versus 80%) (odds ratio 0.12, 95% confidence interval (0.02-0.60), p < 0.01). CONCLUSION: Airway obstruction and pleural and pericardial effusions contributed to the unstable condition of cancer patients with SVCS. The vital prognosis of SVCS was mainly related to the underlying diagnosis.

Tetralogy of Fallot with left superior vena cava and coronary sinus atrial septal defect: a rare association.

This report describes a rare case of Tetralogy of Fallot with associated left superior vena cava and coronary sinus atrial septal defect. The initial diagnosis was made by echocardiography. The patient underwent complete repair at 2 months of age. Her postoperative course was complicated by low cardiac output requiring ECMO. She was subsequently weaned off of ECMO and discharged home. She continues to do well on serial follow-up.

Surgical management of an epithelioid hemangioendothelioma of the superior vena cava protruding into the right atrium.

Epithelioid hemangioendothelioma (EH) is a rare malignant tumor of vascular origin that often involves soft tissues and visceral organs, and less commonly, large veins. We report a case of EH of the superior vena cava protruding into the right atrium and its surgical management.

Epithelioid hemangioendothelioma of the right atrium invaded the superior vena cava: case report and review of literature.

Epithelioid hemangioendothelioma (EHE) is a rare hemangioma that can occur anywhere in the body. It occurs most commonly in the liver and lungs, rarely from the heart, and the etiology or risk factors are unclear. So far, timely detection and radical resection is a more acceptable treatment. Reviewing the literature, few cases of cardiac EHE have been reported. We present a rare case of EHE of the right atrium invaded the superior vena cava.

Bridging Stent Placement in Vena Cava Syndrome With Tumor Thrombotic Extension in the Right Atrium.

Venous stent placement of symptomatic occlusion of the superior and inferior vena cava is considered the treatment of choice in malignant disease because this technique can restore patency and achieve a relief of symptoms. However, tumor thrombus extension into the right atrium harbors the potential risk of stent migration and perforation. One strategy to avoid this potential life-threatening complication could be the placement of a bridging stent from the superior vena cava-to-inferior vena cava. This case reports describes the superior vena cava-to-inferior vena cava bridging stent technique in 2 patients with malignant occlusion of the superior and inferior vena cava. Special considerations such as technical details of the devices and potential complications are discussed.

Surgical treatment of asymptomatic epithelioid hemangioendothelioma originating from the superior vena cava: A case report.

RATIONALE: Epithelioid hemangioendothelioma is a rare endothelial tumor with a low-grade malignancy. This tumor can be treated with complete resection. PATIENT CONCERNS: A 20-year-old Korean man visited our hospital due to an abnormal finding on standing chest PA X-ray. He did not have any past medical history. DIAGNOSIS: Chest computed tomography shows a well-defined, oval-shaped tumor invading the brachiocephalic vein and superior vena cava. A malignant tumor of vascular origin was diagnosed by a percutaneous needle biopsy. INTERVENTIONS: We performed en-bloc resection including the great vessels for complete resection of the tumor. Histologic evaluation confirmed the lesion to be a hemangioendothelioma and the surgical margins were free from tumor invasion. OUTCOMES: Fourteen days later, the patient was discharged without any complication. Thirty months after surgery, recurrences, or metastasis were not detected. LESSONS: Epithelioid hemangioendothelioma is a rare malignant endothelial tumor in the central vein. Surgery is the treatment of choice and shows good results. We introduce and appropriate surgical method to ensure successful treatment for rare disease.

Radical repair of cor triatriatum with partially unroofed coronary sinus and persistent left superior vena cava 35 years after operation for tetralogy of Fallot.

We report a rare case of simultaneous repair for Cor triatriatum (CT), partially unroofed coronary sinus (CS) with persistent left superior vena cava (PLSVC), moderately severe tricuspid regurgitation, and atrial flutter 35 years after radical operation for tetralogy of Fallot (TOF). A 40-year-old woman underwent patch closure of atrial septal defect as to drain blood from left SVC to right atrium (RA), excision of diaphragm in left atrium (LA), tricuspid annuloplasty, and cryoablation. Postoperative course was uneventful with successful anatomical correction.

Alleviation of severe compressive symptoms in a patient with advanced lung carcinoma using tracheal and superior vena cava stents.

Lung cancer is now the leading cause of death from cancer worldwide. Although surgery remains the treatment of choice, the majority of patients will be unresectable at presentation with a poor survival outcome. In those patients who also have tracheobronchial involvement; the aim of intervention is to restore airway patency, thus improving quality of life in a minimally invasive way. Superior vena cava (SVC) infiltration by lung carcinoma is present in 3%-5% of patients with lung cancer. In patients with malignant SVC syndrome, the average life expectancy is 3 to 10 months. In these cases the disease progresses rapidly and obstruction develops before there is time for collaterals to develop. We present a case of a 75-year-old man who had alleviation of severe compressive symptoms secondary to advanced lung carcinoma using tracheal and SVC stents placed as a single procedure.

Thymoma metastatic to liver and pancreas: case report and review of the literature.

A 71-year-old man presented with a thymic mass involving the superior vena cava. A mediastinoscopical biopsy initially suggested a diagnosis of type A thymoma. After neoadjuvant chemotherapy, the patient underwent en-bloc thymectomy and vascular resection for a pathology-confirmed type B3 thymoma involving the superior vena cava, the left brachiocephalic vein and the distal part of the right brachiocephalic vein. Adjuvant radiotherapy was administered. Two years after the primary surgery, abdominal computed tomography (CT) and whole body fluorodeoxyglucose (18-FDG) positron emission tomography (PET) scans showed a single hepatic lesion that was treated with wedge liver resection. Pathological examination confirmed metastatic type B3 thymoma. Almost 4 years later, abdominal CT and 18-FDG PET revealed a 2.9-cm solid mass involving the body of the pancreas. Distal pancreatectomy with lymph node dissection was performed. Pathological examination showed a pancreatic metastasis from a type B3 thymoma, without lymph node involvement. The patient is alive and free of disease 6 months after the pancreatectomy (68 months after the initial thymectomy surgery). Intra-abdominal recurrence and pancreatic metastases are very uncommon manifestations of thymoma, but this event should be kept in mind when an abdominal mass is seen during follow-up.

Thyroid cancer with massive invasion into the neck and mediastinal great veins.

Papillary thyroid carcinoma with massive invasion into the great veins of the neck and mediastinum has rarely been reported and is thought to have a poor prognosis. Here we report successful management of a case of papillary thyroid carcinoma with extensive invasion into the left internal jugular vein, left brachiocephalic vein, and superior vena cava, followed by reconstruction of the superior vena cava using an artificial graft. The operation was conducted to prevent sudden death due to complete obstruction of venous flow, improve the patient's quality of life, and prolong survival. The patient has survived for more than two years after surgery, with good general condition.

Surgical Planning by 3D Printing for Primary Cardiac Schwannoma Resection.

We report herein a case of benign cardiac schwannoma in the interatrial septum. A 42-year-old woman was transferred from a clinic because of cardiomegaly as determined by chest X-ray. A transthoracic echocardiography and chest computed tomography examination revealed a huge mass in the pericardium compressing the right atrium, superior vena cava (SVC), left atrium, and superior pulmonary vein. To confirm that the tumor originated from either heart or mediastinum, cine magnetic resonance imaging was performed, but the result was not conclusive. To facilitate surgical planning, we used 3D printing. Using a printed heart model, we decided that tumor resection under cardiopulmonary bypass (CPB) through sternotomy would be technically feasible. At surgery, a huge tumor in the interatrial septum was confirmed. By incision on the atrial roof between the aorta and SVC, tumor enucleation was performed successfully under CPB. Pathology revealed benign schwannoma. The patient was discharged without complication. 3D printing of the heart and tumor was found to be helpful when deciding optimal surgical approach.

Robot-assisted radical adrenalectomy with clamping of the vena cava for excision of a metastatic adrenal vein thrombus: a case report.

BACKGROUND: Renal or adrenal neoplastic vein thrombi are relative contra-indications for laparoscopic treatment. To the best of our knowledge, we present the first robot-assisted radical adrenalectomy (RARA) with the presence of a thrombus in the adrenal vein. METHODS: A 54 year-old male with a history of laparoscopic left radical nephrectomy for clear cell carcinoma was referred to our department with a diagnosed right adrenal tumour extending into the adrenal vein. A RARA was planned through a trans-peritoneal approach, and an en bloc resection of the adrenal and its vein with clamping of the vena cava was performed. RESULTS: Console time was 94 min and the estimated blood loss was 44 ml. The pathology report confirmed clear cell carcinoma with negative surgical margins. Convalescence was uneventful. CONCLUSION: RARA with thrombectomy and vascular reconstruction can be safe, effective and feasible in experienced hands, using robotic bulldogs.

Oncologic emergencies I. Metabolic and space-occupying consequences of cancer and cancer treatment.

Proper management of the consequences of cancer and cancer treatment is necessary to give affected children the increased chances of survival that recent therapeutic advances offer them. This article discusses the pathophysiology, diagnosis, and management of those metabolic and space-occupying consequences that are likely to face the primary care physician.

Superior vena cava thrombosis with peripartum dilated cardiomyopathy.

A 30 years multiparous female with history of emergency caesarean section 10 days back was referred to us with cough, severe breathlessness at rest, orthopnea with pain in neck and arms. Clinical examination revealed signs of heart failure. Echocardiography showed ejection fraction of 15%, with no right ventricular strain. A diagnosis of peripartum cardiomyopathy was made. Doppler ultrasound of neck veins showed bilateral internal jugular vein thrombosis. Subsequent multislice CT examination showed thrombosis of superior vena cava and both internal jugular veins (with collateral formation) and pulmonary embolism. There were no mediastinal abnormalities on the CT scan. Her thrombophilia screen and CT scan brain was normal. She was managed in collaboration with cardiologist. Following treatment with subcutaneous enoxaparin therapy and warfarin her symptoms of upper limb pain improved. She responded very well to medical therapy for heart failure with marked improvement of NYHA functional class.

Radical surgery in an unusual case of thymoma with intraluminal growth into the superior vena cava and right atrium.

We report a very rare case of malignant invasive thymoma with intraluminal growth through the thymic veins into the superior vena cava (SVC), with intracardiac right atrium extension. A 44-year old female with SVC syndrome underwent a radical thymectomy with pericardiectomy and complete removal of the endovascular and endocardiac neoplastic thrombus by a longitudinal incision starting from the atrium and extending along the SVC. The left anonymous vein was sacrificed, and the SVC and atrium were repaired with a continuous 5-0 Prolene suture. The hospital stay was uneventful. Postoperatively, the patient received adjuvant chemoradiotherapy (three cycles of cisplatin, doxorubicin and cyclophosphamide and subsequent mediastinal irradiation with 50 Gy). Nine months after surgery, no recurrences were seen and the patient is still well. This thymoma presentation with intravascular growth without direct vascular wall infiltration, although very rare, is possible and the management may be challenging. In our case, a primary radical operation was considered mandatory due to the clinical symptoms and the risk of neoplastic embolization. The collection of other similar cases could better clarify the role of adjuvant therapy.