A rare cause of superior vena cava syndrome: extramedullary plasmocytoma

We report the case of a 79-year-old man for whom investigations of superior vena cava syndrome have revealed extramedullary plasmocytoma. Extramedullary plasmocytoma (EMP) is the less frequent form of plasma cell neoplasms. The mediastinal location of EMP is rare, and its compressive nature is exceptional. Another particular feature we observed is that the EMP was associated with an authentic kappa light chain multiple myeloma.     

Aneurysms of an aberrant right subclavian artery: report of two cases

Aneurysm of the aberrant right subclavian artery is rare. We report two patients who underwent successful repair using hypothermic circulatory arrest and retrograde cerebral perfusion. One patient presented with progressive dysphagia to solids and hoarseness of voice due to pressure exerted by the expanding aneurysm, and the second patient, with a dissected subclavian aneurysm, presented with transient ischaemic attacks and a previous cerebral infarct secondary to embolism. Repair in both patients was done through a median sternotomy with femoral arterial and right atrial venous cannulation. Under hypothermic circulatory arrest, the origin of the subclavian aneurysm was divided off the aorta and closed with a Dacron patch from outside the aorta in one patient, and from within the aorta in the other patient. Concomitant revascularisation of the right upper limb was achieved using a 12-mm Hemishield Dacron graft from the ascending aorta to the distal right subclavian artery. The graft to subclavian anastomosis was done under hypothermic circulatory arrest as distal control was not possible. The graft to the aortic anastomosis was done over a side-biting clamp during rewarming. Both patients were successfully treated without major morbidity.     

Unusual collateral vessel from right subclavian vein to left atrium,a rare complication of superior vena cava obstruction

The most commonly reported collateral systems in the setting of superior vena cava obstruction are azygos venous system, vertebral venous system, external and internal thoracic venous system based on McLntire and Sykes classification. A 49‐year‐old female with renal disease complained dyspnea on exertion. Transesophageal echocardiography showed significant mitral annular calcification, large multi‐lobulated mass at posterior aspect of RA, and complete obstruction of superior vena cava by thrombus formation. Computed tomography angiography showed a collateral vein to the left atrium (LA) roof. This case report is the first one which shows development of collateral vein from right subclavian to LA.     

Coronary artery bypass and superior vena cava syndrome

Superior vena cava syndrome is the obstruction of the superior vena cava or its main tributaries by benign or malignant lesions. The syndrome causes edema and engorgement of the vessels on the face, neck, and arms, nonproductive cough, and dyspnea. We discuss the case of a 48-year-old obese diabetic woman who was admitted with unstable angina. She had previously been diagnosed with superior vena cava syndrome. Urgent coronary artery bypass grafting was necessary Although thousands of coronary artery bypasses are performed every year, there are not many reports on patients with superior vena cava syndrome who successfully undergo cardiopulmonary bypass and coronary artery grafting with an internal mammary artery as the conduit. The results of the case and alternative recommended methods are discussed.     

Aortic pseudoaneurysm as a rare cause of superior vena cava syndrome--a case report

This case is about an aortic pseudoaneurysm after Dacron graft replacement that presented as a rapidly evolving superior vena cava syndrome.     

Absent right superior vena cava with persistent left superior vena cava: Implications and management

Seven cases of absent right superior vena cava with persistent left superior vena cava and normal situs were diagnosed at Children's Hospital of Pittsburgh. All patients had associated cardiac defects. In two cases the diagnosis was made at autopsy, the first in 1957 in a 26 day old infant with multiple congenital defects and the second in 1965 in a 22 day old infant who had pulmonary atresia with ventricular septal defect and patent ductus arteriosus. Since 1966 absent right superior vena cava has been diagnosed at cardiac catheterization in five children. Three of these children have had surgery, two for subaortic stenosis and one for an atrial septal defect. One has an insignificant atrial septal defect and the fifth has a ventricular septal defect. The electrocardiogram of four reveals a short P-R interval and a leftward frontal plane axis of the P wave, suggesting a low atrial focus. None has had any significant conduction problem. All five children are living and well; the oldest has survived 13 years postoperatively. Certain precautions are necessary should corrective cardiac surgery or transvenous pacemaker insertion be necessary.     

Challenging pacemaker implantation: Persistent left superior vena cava with absent right superior vena cava

A persistent left superior vena cava (PLSVC) in combination with an absent right superior vena cava (RSVC) is a rare congenital cardiovascular abnormality which is usually found by chance during pacemaker (PM) implantation. In this case we describe a PM implantation using right cephalic approach through PLSVC and coronary sinus (CS), with lead fixation in right atrium and a posterolateral branch of the CS.     

Bilateral superior vena cava with right superior vena cava draining into left atrium

Anomalies of systemic venous return are extremely heterogeneous congenital malformations with variable ranges from completely normal physiology to severe forms of right to left shunting requiring surgical treatment. Anomalous drainage of a right-sided superior vena cava (SVC) to the left atrium (LA) is one of the rarest variants of systemic venous return anomalies, characterized by right-to-left shunt physiology and cyanosis. Here we report a 2 years old girl presented with cyanosis which was observed shortly after birth by her parents but not further investigated. She is otherwise active girl and with normal growth and development. Her clinical examination was unremarkable apart from mild clubbing of the fingers and low oxygen saturation of 88–90% in room air. Her ECG and chest X-ray were unremarkable. Echocardiography showed bilateral SVC connected by a small innominate vein. The right SVC drains directly into the LA while the left SVC drains into the right atrium (RA) via a dilated coronary sinus. There is a small superior sinus venosus type atrial septum defect (ASD) with left to right shunt. Also, there is partial anomalous pulmonary venous return with right upper and right middle pulmonary veins draining directly into the right SVC, which is connected to LA. The right lower pulmonary vein and left pulmonary veins drain directly to LA. The rest of her echocardiography demonstrated normal heart structures and function. This patient was referred for surgical correction, including baffling of the right SVC to the RA and closure of the ASD. We describe this case to highlight the importance of recognizing this rare anomalous systemic venous connection as one of the very rare causes of cyanosis in the pediatric age group as well as at older age.     

Migration of an endovascular stent from superior vena cava to the right ventricular outflow tract in a patient with superior vena cava syndrome

Migration of endovascular stents is a rare problem but can be fatal. We report an unusual case of an endovascular stent in the right ventricular outflow tract, which migrated from superior vena cava in a patient with superior vena cava syndrome.     

Implantation of pacemaker for sick sinus syndrome in a patient with persistent left superior vena cava and absent right superior vena cava

Absent right superior vena cava with persistent left superior vena cava in normal situs is an exceedingly rare congenital anomaly. In such cases, pacemaker implantation (PMI) is very difficult or even impossible. We report the case of a patient with sick sinus syndrome in whom PMI was easily performed via the left superior vena cava by using a steerable stylet.     

Repair of left subclavian artery and aberrant right subclavian artery aneurysms

Left subclavian artery aneurysm with an aneurysm of the aberrant right subclavian artery is a rare condition with a reported incidence of 0.13% to 1%. We report the successful surgical correction of both conditions in a 34-year-old man.     

Bilateral absence of the superior vena cava presenting with superior vena cava syndrome: A case report

Bilateral absence of the superior vena cava (SVC) is a very rare congenital vascular anomaly that is mainly asymptomatic. In this report, we describe an adult male patient with bilateral absence of the SVC presenting with SVC syndrome. Blood from the upper body returned to the right atrium via the superficial thoracoepigastric veins, the great saphenous veins, the common femoral veins, and the inferior vena cava.