11/55. Klippel-Trenaunay syndrome with a life-threatening thromboembolic event.Klippel-Trenaunay syndrome is a congenital disorder characterised by the triad of cutaneous vascular nevi, soft tissue or bony hypertrophy, and varicose veins or venous malformations involving one or more extremities. An incidence of venous thromboembolism of up to 22% has been reported in this disorder. Also reported but rare is the development of trophic changes. Herein, we report the case of a male with Klippel-Trenaunay syndrome, deep vein thrombosis, venous ulceration, and death due to recurrent pulmonary embolism.- - - - - - - - - - ranking = 1keywords = thromboembolism (Clic here for more details about this article) |
12/55. Combined protein c and protein s deficiency in a family with repetitive thromboembolism and segregated gene mutations.A young man with repetitive deep venous thrombosis of the legs and the inferior vena cava, and his family were eventually diagnosed by means of molecular genetic analysis as having both hereditary protein c and protein s deficiency. There have been a few reports of families with combined protein c and protein s deficiency and only one report of such a family characterized at the dna level. This was the first reported family in japan with combined deficiency of protein c and protein S accompanied by segregation of gene lesions.- - - - - - - - - - ranking = 4keywords = thromboembolism (Clic here for more details about this article) |
13/55. An unusual case of recurrent Loffler endomyocarditis of the aortic valve.Idiopathic hypereosinophilic syndrome is a rare systemic disease with an unexplained elevated eosinophil count. Loffler endomyocarditis is hypereosinophilic syndrome with endocardial fibrosis and restrictive cardiomyopathy. The atrioventricular valves are frequently involved, causing valvular regurgitation. Previously, there has been one case report of combined aortic and mitral valve involvement with Loffler endomyocarditis that was treated with bivalvular replacement. We describe a previously healthy 50-year-old man diagnosed with Loffler endomyocarditis complicated by peripheral thromboembolism and severe aortic regurgitation due to valve fibrosis and fibrotic vegetation on the aortic valve. He underwent embolectomy and aortic valve replacement in addition to treatment for his hypereosinophilia. He later presented with cardiomyopathy with severe aortic insufficiency due to the destruction of the aortic valve prosthesis by sterile fibrinous vegetation. To our knowledge, this is the second case in the literature in which Loffler endomyocarditis involves the aortic valve and the first patient in whom only the aortic valve is involved.- - - - - - - - - - ranking = 1keywords = thromboembolism (Clic here for more details about this article) |
14/55. Large left atrial thrombus formation despite warfarin therapy after device closure of a patent foramen ovale.Appropriate anticoagulation after transcatheter device placement is controversial. patients with no history of thromboembolism or neurologic event typically receive antiplatelet therapy for several months while the device endothelializes. For patients with a history of stroke, there are no established guidelines for postdevice anticoagulation. Most patients receive warfarin, antiplatelet therapy, or a combination. Thrombus formation after transcatheter device placement has been reported for most commercially available devices. We describe a patient who developed a left atrial thrombus after closure of a patent foramen ovale with a CardioSEAL device. The patient had a normal hypercoaguable laboratory evaluation prior to device placement. Thrombosis occurred despite warfarin therapy before and after device placement. The patient's international normalized ratio was checked every 2 weeks after device placement and ranged between 2.0 and 2.8. She had no clinical arrhythmia during this time period. The left atrial thrombus was detected on routine follow-up transthoracic echocardiogram performed 6 months after device deployment. A subsequent transesophageal echocardiogram demonstrated no residual shunt, appropriate positioning of the device, flat against the septum, and a 1 x 2 cm thrombus attached to the superior and posterior left atrial arm near the junction with the native septum. A fluoroscopic image demonstrated no arm fractures. The device and thrombus were subsequently removed at surgery without complication. This case is perplexing in that the patient received appropriate anticoagulation had a negative hypercoaguable work-up, no residual shunt, and a well-positioned device.- - - - - - - - - - ranking = 1keywords = thromboembolism (Clic here for more details about this article) |
15/55. Recurrent episodes of deep vein thrombosis in a young man.We present the case of a 32-year-old man suffering from recurrent episodes of deep vein thrombosis (DVT). He was heterozygous for the G1691A mutation in the factor v gene. His father was heterozygous for the same mutation and had a unique episode of DVT after a fracture of the tibia. Genetic predisposition significantly influences the prevalence of thrombotic events, however, additional unknown factors may be involved in the initiation and recurrence of venous thromboembolism.- - - - - - - - - - ranking = 1keywords = thromboembolism (Clic here for more details about this article) |
16/55. Endocardial flap of left atrial dissection following radiofrequency ablation.This report describes an unusual mobile strand found by transesophageal echocardiography prior to a patient's second radiofrequency ablation for paroxysmal atrial fibrillation. This structure arose from the limbus of the left upper pulmonary vein, a location where radiofrequency energy bursts were delivered during the first ablation procedure. After seven months of therapeutic anticoagulation, there was no evidence of change in the size of the mass or of thromboembolism and the patient underwent radiofrequency ablation without complication. We believe this structure most likely represents an endocardial flap of left atrial dissection temporally related to radiofrequency ablation.- - - - - - - - - - ranking = 1keywords = thromboembolism (Clic here for more details about this article) |
17/55. Recurrent thrombosis despite anticoagulation in a man with monocytosis. Case 8.Recurrent thromboembolism despite oral anticoagulation is primarily suspicious of overt or occult neoplasia. We report on a man (age: 67 years) who presented with severe thrombophilia which was only controlled when the patient was set on a combined anticoagulation with low molecular weight heparin in supratherapeutic dosage and phenprocoumon with a target INR of 2.0. Despite repeated evaluation over about two years, a malignant tumour could never be demonstrated. However, the patient suffered in addition to a protein s deficiency from an antiphosphospholipid syndrome and a chronic myelomonocytic leukaemia. We postulate that the accepted strong thrombogenicity of antiphosphospholipid syndrome was further increased by protein s deficiency and a possibly procoagulatory effect of the abnormal monocytes explaining the severe thrombophilia resistant to standard therapeutic anticoagulation with a vitamin k antagonist and usual therapeutic doses of low molecular weight heparin, respectively.- - - - - - - - - - ranking = 1keywords = thromboembolism (Clic here for more details about this article) |
18/55. Use of low molecular weight heparin for prophylaxis and treatment of thromboembolism in pregnancy.Low molecular weight heparin (LMWH) preserves the antithrombotic action but not the anticoagulant activity of heparin. LMWH is safe, does not cross the placenta and is administered as a single daily injection. We report our experience with 6 pregnant women given LMWH for treatment or prophylaxis of thromboembolism. The drug was successfully given to 5 women for periods of 6 weeks--6 months and no thromboembolic complications occurred during pregnancy or pueperium. There were no hemorrhagic complications and no excessive bleeding was observed during delivery. The sixth patient relapsed after 6 weeks of therapy. This patient also showed resistance to standard heparin administered intravenously at a very high dose. LMWH should be considered an alternative to standard heparin in pregnant women requiring antithrombotic prophylaxis and therapy.- - - - - - - - - - ranking = 5keywords = thromboembolism (Clic here for more details about this article) |
19/55. Thrombophilic dysfibrinogen tokyo V with the amino acid substitution of gammaAla327Thr: formation of fragile but fibrinolysis-resistant fibrin clots and its relevance to arterial thromboembolism.Thrombophilic dysfibrinogen tokyo V was identified in a 43-year-old man with recurrent thromboembolism. Based on analyses of the patient fibrinogen genes, the amino acid sequence of the aberrant fibrinogen peptide, and deglycosylation experiments, fibrinogen tokyo V was shown to have an amino acid substitution of gamma Ala327Thr and possibly extra glycosylation at gamma Asn325 because the mutation confers the N-linked glycosylation consensus sequence Asn-X-Thr. The mutation resulted in impaired function and hypofibrinogenemia (hypodysfibrinogen). polymerization of fibrin monomers derived from patient fibrinogen was severely impaired with a partial correction in the presence of calcium, resulting in very low clottability. Additionally, a large amount of soluble cross-linked fibrin was formed upon thrombin treatment in the presence of factor xiii and calcium. However, tokyo V-derived fibrin was resistant to degradation by tissue plasminogen activator (tPA)-catalyzed plasmin digestion. The structure of tokyo V fibrin appeared severely perturbed, since there are large pores inside the tangled fibrin networks and fiber ends at the boundaries. Taken together, these data suggest that tokyo V fibrin clots are fragile, so that fibrinolysis-resistant insoluble fibrin and soluble fibrin polymers may be released to the circulation, partly accounting for the recurrent embolic episodes in the patient.- - - - - - - - - - ranking = 5keywords = thromboembolism (Clic here for more details about this article) |
20/55. Recurrent life-threatening thromboembolism and catastrophic antiphospholipid syndrome in a patient despite sufficient oral anticoagulation.We report on a 32-year old female patient with primary antiphospholipid syndrome (PAPS) and several thromboembolic events despite stable doses of oral anticoagulation, good patient compliance and maintained INR values of >3. Over the preceding 3 years the patient had presented a wide spectrum of manifestations of APS, including recurrent venous and arterial thromboses, cardiac, gynecological (hellp syndrome), neurological involvements, livedo reticularis, a mild thrombocytopenia and the most feared manifestation of the catastrophic antiphospholipid syndrome (CAPS). life-threatening bilateral subdural bleeding occurred while she was anticoagulated. The clinical features appeared to be refractory to oral anticoagulation with phenprocoumon. They were life threatening on each occasion and she developed repetitive episodes of organ damage with cardiac insufficiency (NYHA III), pulmonary hypertension and other residual defects. Even during heparinization recurrent thromboembolism supervened as well as livedo reticularis of the extremities. Lupus anticoagulants (LAC), anticardiolipin (aCL) antibodies and anti-beta(2)-glycoprotein-1 (beta(2)GPI) titers were all markedly elevated. This case report shows that recurrent episodes of thrombosis can occur despite seemingly adequate anticoagulation in patients with CAPS.- - - - - - - - - - ranking = 5keywords = thromboembolism (Clic here for more details about this article) |
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