Superior vena cava syndrome svcs is a medical condition which. Lonardi f, gioga g, agus g, coeli m, campostrini f. Open and endovascular treatment of superior vena cava. Chronic warfarin therapy up to 1 year has been described in some reports. Superior vena cava syndrome is a relatively rare presentation in which diminished venous return to the heart produces congestion of the neck, face and upper extremities. It transports blood from our arms, chest, neck, and head to our hearts.
Superior vena cava syndrome svcs is a relatively common sequela of. Patients can present with dyspnea, cough, dysphagia, and swelling of. We are writing in response to the brief report entitled superior vena cava syndrome due to nonsmallcell lung cancer by citron et al. Without treatment, death generally occurs within 24 to 72 hours. Several reports have shown thrombolytic therapy with intravenous streptokinase or urokinase to be effective in the treatment of this condition. To demonstrate the effectiveness of endovascular treatment evt with selfexpandable bare stents for malignant superior vena cava syndrome svcs and to analyze predictive factors of evt efficacy. Solid facial edema morbihans syndrome and various infiltrates included in the differential diagnosis were ruled out with a skin biopsy. Treatment of superior vena cava syndrome article pdf available in jama the journal of the american medical association 2519. Fullskin examination revealed multiple prominent varicosities on the chest and abdomen. Superior vena cava svc syndrome is a common complication of malignancy, with lung cancer being the predominant cause. Endovascular treatment of malignant superior vena cava.
Treatment of superior vena cava syndrome using angiojet. Obstruction of the venous flow through this vessel results in impaired venous drainage, with engorgement of the vessels from the head and upper body. Superior vena cava syndrome guidelines bmj best practice. General treatment considerations endovascular treatment. Palliative treatment of superior vena cava syndrome with nitinol stents. Superior vena cava syndrome an overview sciencedirect. Most treatment of superior vena cava syndrome focuses on addressing the underlying cause of the syndrome. Superior vena cava syndromea proposed classification system. Malignancyrelated superior vena cava syndrome uptodate. A 58yearold man presents with a 2week history of progressive dyspnea on exertion, neck swelling, decreased appetite, and fatigue. Cardiovascular and interventional radiological society of europe.
A transudative chylothorax associated with superior vena. Treatment of superior vena cava syndrome jama network. Superior vena cava syndrome svcs occurs when a persons superior vena cava is partially blocked or compressed. Superior vena cava syndrome svcs is often seen in the natural history of malignant thoracic diseases. It is evident in 4% of lung cancer patients at diagnosis, and it may also develop during the disease course. Svcs is characterized by unpleasant symptoms that usually lead to death. Caval syndrome is an advanced stage of heartworm disease and has a poor rate of survival. Superior vena cava syndrome svcs is obstruction of blood flow through the superior vena cava svc. Superior vena cava syndrome svcs includes various clinical signs and symptoms due to external compression. Caval syndrome in dogs symptoms, causes, diagnosis. One of the benign causes of svc obstruction is the longterm use of indwelling catheters and wires, increasing the chances of svc thrombosis. Heartworm removal from the vena cava, right atrium, and. In superior vena cava syndrome svcs, this vein has.
Superior vena cava svc syndrome results from any condition that. A reassessment of the clinical applications of the superior vena cava syndrome. Due to the substantial increase in the number of ivc filters placed in the united states and the very low filter. Catheters should be removed and local thrombolysis andor shortcourse anticoagulation should be considered in these patients. Lung cancer is the most common cause of superior vena cava syndrome svcs and requires timely recognition and management. The superior vena cava is one of the primary veins within our bodies. What is the pathophysiology of superior vena cava svc. As a consequence, various forms of therapy have been promptly. In this manuscript, we describe an extremely rare case of a transudative chylothorax secondary to superior vena cava syndrome svc as well as a description of five previously reported cases of transudative chylothoraces associated with svc syndrome published in the english literature between 1960 and 2018 via a medline search using the search terms. The majority of cases are caused by malignant tumors within the mediastinum, most commonly lung cancer and nonhodgkins lymphoma, directly compressing or invading the svc wall.
The resulting increased venous pressure in the upper body may cause edema of the head, neck, and upper. Superior vena cava syndrome svcs results from the impair. Treatment depends on the etiology of the obstructive process. The treatment of the benign svc syndrome is focused on achieving longterm durability and patency of the superior. It is a medical emergency and most often manifests in patients with a malignant disease process within the thorax. Superior vena cava syndrome svcs, is a group of symptoms caused by obstruction of the superior vena cava svc, a short, wide vessel carrying circulating blood into the heart. Doubleflash, largefraction radiation therapy as palliative treatment of malignant superior vena cava syndrome in the elderly.
However, superior vena cava syndrome can present acutely in the setting of vena cava thrombosis. Superior vena cava syndrome is caused by gradual compression of the superior vena cava svc. Symptoms and signs are more flowery, the blood returns to the heart via the upper abdominal veins and inferior vena cava. There are no detailed guidelines addressing the management of svc obstruction. A 42yearold male patient was seen at consultation presenting with a pulsatile, warm and slightly. Superior vena cava syndrome svcs, a clinical manifestation arising from compression of the thinwalled superior vena cava svc, was first described by william hunter in 1757 and can be caused by a variety malignancies hunter and johnston 1757. Superior vena cava syndrome svcs consists of a set of signs dilation of the veins of the neck, facial plethora, edema of the upper limbs and cyanosis and symptoms headache, dyspnea, cough, orthopnea, dysphagia, etc. The clinical diagnosis of svc syndrome is based largely on history and physical examination. Review of evolving etiologies, implications and treatment.
The superior vena cava was successfully bypassed in a patient with superior vena cava syndrome due to granulomatous mediastinitis. Quality assurance guidelines for superior vena cava stenting in malignant disease external link opens in a new window. We report a patient with svc syndrome secondary to documented ipsilateral peritracheal nodal involvement stage iiib disease who underwent neoadjuvant chemoradiotherapy. The most common cause of superior vena cava syndrome is cancer. Typically, a mediastinal mass produces external compression on the superior vena cava and reduces venous return. Definition superior vena cava svc syndrome is a clinical condition that occurs as a result of obstruction of the svc, leading to interrupted venous return from the head, thorax, and upper extremities to the right atrium. Six patients with caval pressure higher than 22 mm hg peripheral to the stenosis underwent stent.
Michael mcfadden the superior vena cava and its important intrathoracic venous tributaries are located in a tight compartment within the superior mediastinum, immediately adjacent and anterior to the trachea and right main bronchus. The superior vena cava is a major venous vessel that returns blood to the right atrium of the heart from the head, upper thorax, and upper extremities. Pdf treatment of superior vena cava syndrome researchgate. We report a case of superior vena cava thrombosis in a 53yearold woman receiving chemotherapy for breast carcinoma through. An intrathoracic malignancy is responsible for 60 to 85 percent of cases of superior vena cava syndrome svc syndrome, and svc obstruction is the presenting overview of the risk factors, pathology, and clinical manifestations of lung cancer view in chinese. This condition is mainly seen in pregnant females and the. Review of evolving etiologies, implications and treatment strategies. Thrombosis of the inferior vena cava ivc is an underrecognized entity that is associated with significant short and longterm morbidity and mortality. Primary tumors are most commonly the cause of this syndrome. Surgical treatment of benign superior vena cava syndrome. Since the majority of cases occur due to cancer, appropriate treatment is key. Superior vena cava syndrome symptoms, diagnosis and.
Svcs is a significant disorder affecting up to 10 % of small cell lung cancer sclc patients and 24 % of all lung cancer patients. Inferior vena cava syndrome is a pathological condition which is characterized by obstruction or blockage of the inferior vena cava vein which transports blood from the lower extremities to the heart. Introduction superior vena cava svc syndrome results from any condition that leads to obstruction of blood flow through the svc. Superior vena cava syndrome treatment algorithm bmj. The superior vena cava syndrome svcs comprises various symptoms due to occlusion of the svc, which can be easily obstructed by pathological conditions eg, lung cancer, due to the low internal venous pressure within rigid structures of the thorax trachea, right bronchus, aorta. It carries blood from the head, neck, upper chest, and arms to the heart. Endovascular treatment of superior vena cava syndrome. The drawing below shows where the superior vena cava is located in the body.
Superior vena cava syndrome itself is very treatable, and symptoms are usually greatly improved within the first month of treatment. The syndrome is rarely an oncologic emergency in the absence of tracheal compression and airway compromise. Superior vena cava syndrome with malignant causes nejm. Symptoms of the syndrome include shortness of breath. Advances in knowledge n use of endovascular expanded polytetrafluoroethylene eptfecovered stents appears to be a safe and effective method to treat patients with malignant superior vena cava syndrome. Treatment and prognosis depend on underlying etiology.
In absence of a congenital anomaly, the most common cause of ivc thrombosis is the presence of an unretrieved ivc filter. Primary or metastatic cancer in the upper lobe of the right lung can compress the superior vena cava. Lymph nodes that drain the entire right chest and lower portion of. Malignancies primarily lung cancer are the underlying cause of 8085% of cases, leaving 1520% caused by.
Signs and symptoms of svc syndrome were classified, graded, and scored in 11 cases. Catheterdirected thrombolysis in acute superior vena cava syndrome caused by central venous catheters. Superior vena cava syndrome is diagnosed by ultrasound, chest xray, ct scan, and in some cases biopsy. The obstruction of blood flow through the superior vena cava svc into the right atrium may present as a severe clinical syndrome. In the management of superior vena cava syndrome svcs, the goals are to relieve symptoms and to attempt cure of the primary malignant. Superior vena cava syndrome in thoracic malignancies. Treatment should also be individualized and should not be undertaken until a. Malignant superior vena caval svc syndrome due to nonsmall cell lung cancer is invariably fatal, with most therapy directed toward palliating the manifestations of the disease. Superior vena cava syndrome is caused by gradual compression of the superior vena cava where it enters the right atrium, leading to edema and retrograde flow. Endovascular treatment of superior vena cava syndrome with stent placement is a safe treatment option with very high efficiency and immediate clinical benefit for the patients.
Patients with malignant svc syndrome are typically seriously ill, and further deterioration is likely due to the presence of unresectable. Covered stent placement for the treatment of malignant. Telescoping sheaths made of polymer andor steel material can be applied with countering force for fibrous overgrowth that resists traction. Causes of superior vena cava include lung cancer, lymphoma, other cancers in the chest, blood clots in the superior vena cava, or. Therefore, we recommend endovascular treatment as the first line therapeutic approach of the syndrome. This includes palliative radiotherapy, chemotherapy or corticosteroids for lymphomas and thymomas, endovascular stents, or rarely bypass surgery. Clarkepearson md, in clinical gynecologic oncology ninth edition, 2018. We are writing in response to the brief report entitled superior vena cava syndrome due to nonsmallcell lung cancer by citron. Selfexpandable metallic stent therapy for superior vena. Organ failure leading to death can occur before or after treatment. Thrombotic occlusion of the superior vena cava is an uncommon but serious complication of chronic indwelling venous catheters. Vascular thrombosis, especially venous, is common in this condition and also constitutes a basic diagnostic criterion.
Percutaneous treatment for pacemakerassociated superior. Superior vena cava syndrome approach bmj best practice. Multimodality treatment of malignant superior vena caval. Its affection of the superior vena cava is rather rare with only a few cases described in the literature. Malignant obstruction can be caused by direct invasion of tumor into the svc, or by external compression of the svc by an adjacent pathologic process involving the right lung, lymph nodes, and other mediastinal structures, leading to stagnation of flow and. Lymphoma or other tumors located in the mediastinum can also cause compression of the superior vena cava less often, the superior vena cava can become blocked with a blood clot from within. The superior vena cava syndrome american journal of medicine. It has been reported that cisplatin regimens, as used in this report, can lead to responses in approximately. Superior vena cava syndrome in a patient with polycytemia vera. To study clinical and physiologic aspects of zstent therapy for superior vena cava svc syndrome secondary to malignant disease. Superior vena cava svc syndrome is the result of stenosis or occlusion of the svc or bilateral brachiocephalic veins. It carries a 14% to 42% risk of death, even with heartworm removal.
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