The occurrence of thrombosis in patients with congenital bleeding disorders represents an exceptional event. Hemophilia A and hemophilia B patients have been showed to present both arterial and venous thrombosis (85 cases of arterial thrombosis and 34 cases of venous thrombosis). The great majority of arterial thrombosis are myocardial infarction or other acute coronary syndromes, whereas the majority of venous thrombosis are deep vein thrombosis and/or pulmonary embolisms. However there are discrepancies in the proportion of arterial and venous thrombosis seen in hemophilia A versus hemophilia B. The ratio of arterial versus venous thrombosis in hemophilia A is 3.72 whereas that for hemophilia B is 1.12. This indicates that arterial thrombosis is more frequent in hemophilia A as compared to hemophilia B and the opposite is true for venous thrombosis. The potential significance of this discrepancy is discussed.

Hemophilia A and hemophilia B do not protect from arterial occlusions (mainly acute coronary syndromes), whereas they assure some protection from venous thrombosis. The opposite seems true for FVII deficiency. The potential significance of this discrepancy is discussed.

The aim of this article was to investigate the prevalence of venous thrombosis in patients with von Willebrand disease. Personal records on 486 patients were reevaluated together with a time unlimited PubMed search. The venous thrombotic event had to be proven by objective means. Only cases of congenital von Willebrand disease were taken into consideration and all types of the diseases were included. No case of venous thrombosis was reported in our cohort of patients. On the contrary, 33 patients with proven venous thrombosis were gathered from the literature (17 cases of deep venous thrombosis with or without pulmonary embolism; isolated pulmonary embolism was seen in seven instances, superficial veins or portal system thrombosis was present in the remaining cases). Associated risk factors, mainly replacement therapy, were present in 26 cases. Therapeutic approach was usually based on heparin and Coumadin. Overall results were fair or good, as no fatalities occurred.

Vascular thrombosis, both arterial and venous, is a condition associated with significant morbidity and mortality. There are multiple risk factors for thrombosis, both congenital and acquired, and in the majority of cases, these risk factors are not modifiable. Over the past 2 decades, multiple drugs (both illegal and legal) have been associated with increased risk of thrombosis. However, due to limited scientific literature regarding the prothrombotic tendencies of these drugs, there is a concomitant limited understanding of the pathophysiology of drug-induced thrombosis. As drugs are one of the few modifiable risk factors for thrombosis, further study and dissemination of knowledge regarding drug-associated and drug-induced thrombosis are essential and have the potential to lead to decreased future incidence of thrombosis. The mechanisms at the basis of the thrombophilic activity of these drugs are variable and sometimes still ill recognized. Increased levels of clotting factors, reduction in coagulation natural inhibitors, decreased fibrinolysis, activated clotting factors, increased blood viscosity, endothelial damage, and increased platelet number and activation are the most frequent causes. Arterial steal or coronary arteries no flow has also been implicated. In some cases due to the intake of several drugs, more than one mechanism is present in a given patient. The purpose of the present review is to analyze all the drugs demonstrated to be potentially thrombotic. It is hoped that a prudent use or nonuse of these drugs might result in a reduction of thrombosis-associated diseases.

BackgroundTo investigate the number of patients with congenital thrombophilia who presented an intracardiac thrombosis.MethodsPersonal files were reevaluated together with a time-unlimited search of the literature.ResultsTwenty-five patients with intracardiac thrombosis and congenital thrombophilia have been gathered from the literature including the two personal cases. The distribution observed in thrombophilia patients is similar for left side or right side heart (9 vs 11 cases). The left ventricle and the right ventricle were involved in six or five instances, respectively. In one case, both ventricles were involved. On the contrary, the left atrium was involved in three cases whereas the right atrium was affected in six cases. In the remaining cases, more than one heart chamber was involved.ConclusionsIn “normal” subjects, left side thromboses are predominant once catheter-associated thrombi are excluded. The reason of this discrepancy lies in the greater prothrombotic effect exercised by congenital thrombophilia on venous thrombosis compared to arterial thrombosis. The relative high prevalence of cardiac thrombosis seen in patients with antithrombin and protein C deficiencies indicated that a cardiac evaluation should be carried out in all patients with these two defects.