Right ventricular failure due to primary right ventricle osteosarcoma

A 68-year-old woman with symptoms of dyspnea and peripheral edema was referred to our hospital. Chest computed tomography (CT) scans revealed a huge mass occupying the pulmonary trunk and invading the right main pulmonary artery, with metastatic nodules in the left main and left lower pulmonary artery. She was given a diagnosis of pulmonary thromboembolism and was anticoagulated to no effect, which suggested a neoplasm. Palliative resection of the tumor was carried out even though she was in serious condition with right ventricular failure, liver congestion, renal dysfunction, and coagulopathy disorder. The histopathology, postoperative systemic CT scan, and bone scintigram provided a definitive diagnosis of a primary right ventricle osteosarcoma. Primary cardiac osteosarcoma has a poor prognosis, and this patient was at an advanced stage with pulmonary metastases. Surgical intervention should offer these patients significant palliation to relieve the clinical symptoms due to obstruction.     

Right ventricular thrombosis early after bidirectional Glenn shunt.

Thrombosis in the right ventricle occurred early after bidirectional superior cavopulmonary shunt in 2 patients with pulmonary atresia with intact ventricular septum and major right ventricular coronary artery communication, and perioperative brain infarction occurred in 1 patient. Clinicians should be aware of the hazards of this potentially lethal complication, and transfusion of platelets and fresh plasma should be minimized. Although the hemodynamic state is good, echocardiography should be performed frequently and strict anticoagulation should be started as early as possible.     

Plasma heparin cofactor II, protein C and antithrombin in elective surgery

The coagulation inhibitors heparin cofactor II (HC II), protein C (PC) and antithrombin (AT) were measured in 14 patients undergoing uncomplicated abdominal, orthopedic or vascular surgery. Marked transient decrease of all the inhibitors was found after all surgical procedures, most prominently in vascular surgery. The greatest mean reduction was in HC II. The fall in PC and AT showed a pattern similar to that of albumin. In contrast to some earlier suggestions our results indicated that, except for HC II, the reduction probably was not due to consumption. Redistribution, hemodilution and, probably, reduced synthesis were the most important contributing factors. Serial measurements of the three coagulation inhibitors may be of prognostic value, but the present study's observed mean decrease of c. 25% in abdominal and 50-62% in vascular surgery must be borne in mind. Still lower inhibitor levels suggest consumption and a complicated postoperative course.     

Right ventricular thrombosis in two patients with pulmonary atresia with intact ventricular septum

Thrombosis was detected in the right ventricle 7 days after a bidirectional Glenn operation in a 1-year-old boy with pulmonary atresia with intact ventricular septum (PAIVS) and immediately before pulmonary valvuloplasty in a 21-day-old boy with PAIVS. The risk of thrombosis and the blood clotting cascade should be assessed by blood tests, and surveillance echocardiography should be frequently applied in order to diagnose thrombosis early in such patients.     

Hypocomplementemic type II membranoproliferative glomerulonephritis in a male patient with familial lecithin-cholesterol acyltransferase deficiency due to two different allelic mutations.

Patients with familial lecithin-cholesterol acyltransferase (LCAT) deficiency very often show progressive glomerulosclerosis with evolution to end-stage disease. High levels of an abnormal lipoprotein (lipoprotein X) cause glomerular capillary endothelial damage. The ultrastructural study of renal biopsy specimens shows characteristic glomerular deposits of membrane-like, cross-striated structures and vacuole structures. The gene encoding for LCAT has been mapped to chromosome 16q22.1, and several mutations of this gene cause LCAT deficiency which is inherited as an autosomal recessive trait and which is characterized by corneal opacities, normochromic normocytic anemia, and renal dysfunction. Herein we report clinical features and renal histological findings concerning a 24-year-old male patient with classical familial LCAT deficiency due to two different allelic mutations: a nonsense mutation inherited from the father and a missense mutation inherited from the mother. Moreover, the patient showed glomerular histological lesions and an immunofluorescent glomerular pattern typical of hypocomplementemic membranoproliferative type II glomerulonephritis (dense-deposit disease). The nature of electron-dense material that characterizes dense-deposit disease is still unknown, but there are suggestions that some chemical modifications might occur in the renal basement membranes. Therefore, this clinical case might induce to consider possible relations between disorders of the lipoprotein metabolism and renal dense-deposit disease.     

Local heparin is superior to systemic heparin in preventing arterial thrombosis

Thrombosis poses a significant problem in microvascular surgery, despite antithrombotic therapy. The purpose of the present study was to investigate whether a topical application of unfractionated heparin is equally efficient as a systemic bolus in avoiding thrombosis. A rat femoral artery model was used. Three different doses of systemic heparin (50, 100, and 200 U/kg) and one dose of locally administered heparin (100 U/ml) were evaluated and compared to a control group receiving isotonic saline. A thrombogenic injury, simulating poor microsurgical technique, was applied to the artery. The thrombus area was visualized by transillumination, and recorded for 60 min on video, for subsequent measurement. In addition, the level of activated partial thromboplastin time (APTT) and of anti-activated clotting factor X (aXa) was determined. Local heparin significantly reduced thrombus size as compared to isotonic saline and systemic heparin (50 and 100 U/kg). High-dose systemic heparin (200 U/kg) was equally potent, but local heparin had significantly less influence on the hemostatic parameters.     

Cerebral sinus thrombosis in patients with severe iron deficiency anaemia due to myoma uteri

Summary Two middle-aged women with haemorrhagic infarction due to thrombosis of the superior sagittal and transverse sinus, respectively, are herein reported. Severe iron deficiency anaemia secondary to myoma uteri seen in these patients was suggested to play a significant role in the development of the sinus thrombosis. In middle-aged women, myoma uteri deserves special attention as a possible aetiological contributor to cerebral sinus thrombosis.     

Successful penile reconstruction following prior arteriovenous loop thrombosis due to undiagnosed protein-S deficiency and exogenous testosterone

Flap failure from microvascular thrombotic occlusion is a rare but significant cause for unsuccessful reconstructive surgery. We encountered thrombosis of arteriovenous loop in a patient undergoing phallus reconstruction. Further investigations revealed underlying previously asymptomatic hypercoagulable state due to protein-S deficiency in addition to long-term exogenous testosterone administration. Role of thrombophilia testing, thrombogenic potential of testosterone and the need for therapeutic perioperative anti-coagulation in such situations are described here.KEY WORDS: Microvascular thrombosis, testosterone, thrombophilia     

Right ventricular myocardial infarction and late cardiac tamponade due to right coronary artery aneurysm--a case report

Coronary artery aneurysm is a relatively rare disease, which may cause angina, myocardial infarction, or sudden unexpected death due to thrombosis, embolization or rupture. This report describes a case of a 46 year old male who suffered an inferior myocardial infarction with right ventricular involvement, third degree atrioventricular block, cardiogenic shock and late cardiac tamponade, all caused by a right coronary artery aneurysm. He was successfully treated with emergency coronary artery bypass grafting. A review of the literature is also given to emphasize the importance of prompt recognition and correct management of the coronary artery aneurysm.