Takayasu arteritis of subclavian artery in a Caucasian.

Takayasu arteritis, an inflammatory and obliterative disease of medium and large arteries, is classified as a giant cell arteritis. It has a predilection for the aortic arch, its main branches, and coronary and pulmonary arteries. The early symptoms of Takayasu arteritis may be mainly systemic and may resemble polymyalgia rheumatica. The etiology is unknown but autoimmunity has been suggested to play a role. Diagnosis is based on symptoms, physical findings, and imaging, because tissue diagnosis is rarely feasible. Unlike atherosclerotic vascular disease, Takayasu arteritis affects primarily, but not exclusively, young women. Contrary to earlier reports, it is not limited to the women of Japanese origin but is present worldwide. The current report is of a Caucasian woman who presented with nonspecific complaints of upper back pain, weakness, malaise, and fatigue. Her physical examination revealed absent left radial pulse and a blood pressure differential, later confirmed by radiological imaging studies to be due to left subclavian artery stenosis consistent with Takayasu arteritis. The presentation and management of the patient is described, and Takayasu arteritis is succinctly reviewed.

Thoracic cystic lymphangioma (cystic hygroma): a chest pain syndrome--a case report.

Vascular malformations of the mediastinum including mediastinal lymphangioma are exceedingly rare in adults. A resected case of mediastinal cystic lymphangioma (cystic hygroma) that presented with acute onset of retrosternal chest pain is reported in a 45-year-old man. Cystic hygromas are benign cysts frequently discovered incidentally on chest radiograph. Although computed tomography scan provides helpful information about the size, density, and site of the cysts, it cannot establish a precise diagnosis concerning its nature. Complete removal of the cyst is the treatment of choice and allows precise diagnosis on histologic examination. However, surgical excision is sometimes difficult, owing to the size and extension of the cysts, infiltrating mediastinal planes, enveloping great vessels, and displacing mediastinal organs without invasion. The difficulty of completely eradicating certain cysts explains cases of insidious progression with compressive recurrence. The subject is succinctly reviewed.

Cardiac tamponade and superior vena cava syndrome in lung cancer--a case report.

A combination of pericardial effusion with cardiac tamponade and superior vena caval syndrome is an unusual first presentation of carcinoma of lung, although cardiac involvement is often a late finding in widespread malignancy. Clinical identification can be difficult antemortem. Accurate diagnosis and prompt intervention are necessary to prevent adverse outcomes. Decisions regarding treatment must take into account the clinical presentation and echocardiographic findings. Echocardiography-guided pericardiocentesis with catheter drainage and/or pericardial window is the primary treatment strategy of choice for most large or hemodynamically significant effusions. New cardiac symptoms or classic findings of cardiac tamponade should prompt aggressive investigation. We present a case of adenocarcinoma of the lung that initially presented as pericardial effusion with tamponade and superior vena cava syndrome. The patient had all the clinical features of tamponade such as pulsus paradoxus, tachycardia, elevated jugular venous pressure, hypotension, and electrical alternans on surface electrocardiography. The findings were confirmed on echocardiography and computed tomography of chest, both of which allowed for rapid confirmation of the presence of an effusion and compression of the superior vena cava. The existing literature on the subject is succinctly reviewed.

Positional ventricular tachycardia from a fractured mediport catheter with right ventricular migration--a case report.

The totally implantable catheter system has gained popularity as venous access when prolonged treatment is needed. Despite its frequent use, intravascular fracture and embolization of catheter fragments from implantable venous port-catheter systems present a rare but potentially life-threatening complication. Any implanted catheters should therefore be removed after completion of the treatment or the system's integrity should be monitored on a regular basis. This report illustrates such a case, which presented with ventricular tachycardia triggered by changes in body position from a fractured Mediport catheter with cardiac migration. A 34-year-old woman had a venous port catheter (Mediport) implanted into the right subclavian vein for neoadjuvant radio-chemotherapy for Hodgkin's lymphoma. Owing to the patient's difficult venous access the catheter was left in situ after treatment. Three years after insertion of the Mediport she presented with shortness of breath and palpitations when lying in the left lateral position. Physical examination revealed no abnormalities. An electrocardiogram was within normal rhythm. An outpatient Holter monitor revealed multiple episodes of nonsustained and sustained ventricular tachycardia triggered by lying in the left lateral position. A chest radiograph showed a normal location of the port-system, but the distal fragment of the catheter had embolized into the right ventricle. The embolized fragment was extracted with a gooseneck snare technique and the reservoir of the system was removed under local anesthesia without any complications. The patient was free of symptoms at 7 seven months follow-up.

Right atrial extension of primary venous leiomyosarcoma: pulmonary embolism and Budd-Chiari syndrome at presentation-a case report.

Venous leiomyosarcomas are rare and arise predominantly in the inferior vena cava (IVC). The clinical findings are nonspecific and may precede the diagnosis by several years. IVC leiomyosarcoma is predominantly seen in women. Intracavitary extension of vascular tumors tends to result from embolization or propagation along great veins, and this is a serious risk factor for pulmonary embolism and sudden death when it reaches the right heart. Modern imaging modalities using computed tomography, magnetic resonance imaging, individually or in combination with cavography, ultrasound, and echocardiography, allow an early and accurate preoperative diagnosis, resulting in a higher rate of surgical resection and improved survival. The authors present a 72-year-old woman who presented with pulmonary embolism and Budd-Chiari syndrome. Pathological examination revealed a leiomyosarcoma. The tumor, involving the IVC, was diagnosed with imaging techniques that showed intracardiac extension of a primary venous leiomyosarcoma. The literature discussing leiomyosarcoma of the IVC is briefly reviewed.

Cardiac tamponade in patients with human immunodeficiency virus disease.

The characteristics of cardiac tamponade in patients with human immunodeficiency virus (HIV) disease were examined by evaluating the cases, case series, and related articles, including autopsy series, identified through a comprehensive literature search. One-hundred eighty-five cases of cardiac tamponade have been reported in patients with HIV disease. Sex data were available in 176 patients, of whom 154 (87%) were males. The mean age was 34.7 +/- 10.4 years (range, 11 months to 61 years). Mean CD4 cell count was 98 +/- 95 cells/mm3 (range, 3 to 430 cells/mm3). The most common etiology of pericardial tamponade was mycobacterial infection (78 patients), including Mycobacterium tuberculosis, Mycobacterium avium-intracellulare, and Mycobacterium kansasii. A bacterial cause was found in 20 patients (11%). Staphylococcus aureus was the predominant bacteria, followed by streptococci, Pseudomonas aeruginosa, Listeria monocytogenes, Klebsiella pneumoniae, and Rhodococcus equi. Lymphoma was found in 15 (8%) patients and Kaposi sarcoma in 13 (7%) patients. Numerous unusual organisms, including Cryptococcus neoformans, Nocardia asteroides, Aspergillus species, cytomegalovirus, and herpes simplex were also associated with cardiac tamponade in HIV patients. Occasionally, HIV itself was involved in the pathogenesis. In 48 patients (26%), no cause was found or reported. The most common clinical presentation was dyspnea, followed by fever, cough, chest pain, and cardiac arrest. The predominant pericardial fluid color composition was serosanguineous. The majority of patients died during hospitalization or in the immediate follow-up period. Vigilance for cardiac tamponade in patients with HIV disease, especially in those with opportunistic infections and/or malignancies, and cardiac symptoms, may result in early and proper management of cardiac tamponade in these patients.

Cardiac papillary fibroelastoma originating from pulmonary vein--a case report.

Cardiac papillary fibroelastoma is a primary cardiac neoplasm that typically affects the cardiac valves, mainly the aortic and mitral valves, and very rarely the endocardium of cardiac chambers. Cardiac papillary fibroelastoma is rarely diagnosed during life, as the majority are incidental findings at autopsy, but with the advent of echocardiography, it is being increasingly recognized. Although the tumor is usually small and histologically benign, it may have a malignant propensity for life-threatening complications, such as a cerebrovascular accident, myocardial ischemia or infarction, or sudden death. The patient reported here presented with an embolic stroke from a thrombus on the surface of a left atrial papillary fibroelastoma. The papillary fibroelastoma was originating from the lower portion of the left inferior pulmonary vein and was protruding into the left atrial cavity. Papillary fibroelastoma originating from the pulmonary veins has not been reported before. The tumor was successfully removed by intraoperative transesophageal echocardiography-guided cardiac surgery. Grossly, the surface of the tumor was smooth and translucent. The gelatinous membrane on the surface tore easily, and soft papillary tumor with multiple fronds was visible. Histology confirmed the mass was a papillary fibroelastoma. Postoperative recovery was uneventful. Follow-up transthoracic echocardiogram revealed no residual or recurrence of tumor. The patient was in excellent health at 2-year follow-up. The case is described and the clinical characteristics of cardiac papillary fibroelastoma are reviewed.