Histology of sudden death in arrhythmogenic right ventricular cardiomyopathy/dysplasia.

Circulation (Impact Factor: 15.2). 08/2004; 110(3):e20; author reply e20. DOI: 10.1161/01.CIR.0000135590.37950.30
Source: PubMed
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    ABSTRACT: There is limited evidence that certain heritable thrombophilias may be associated with an increased risk of peripheral vascular disease. In particular, increased activated protein C resistance and FV Leiden have been described as being more prevalent in patients with peripheral arterial disease. There is no clear evidence at present, however, that other heritable thrombophilias are associated with an increased risk of arterial occlusive disease. Heritable thrombophilia, in particular FV Leiden, may be associated with an increased risk of vascular reconstruction failure but there is a lack of evidence to show that intervention with anticoagulants influences the outcome of graft surgery in these patients.
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    ABSTRACT: Arrhythmogenic right ventricular dysplasia, also called right ventricular cardiomyopathy, is a genetically determined heart muscle disease, characterised by life-threatening ventricular arrhythmias in apparently healthy young people. The primary myocardial pathology is that the myocardium of the right ventricular free wall is replaced by fibrous or fibrofatty tissue, with scattered residual myocardial cells. Right ventricular function is abnormal and in severe cases is associated with global right ventricular dilation and overt biventricular heart failure. Although still relatively rare, arrhythmogenic right ventricular cardiomyopathy is a well recognised cause of sudden unexpected peri-operative death. In this review, we describe the basic characteristics of this disease, emphasising the diagnosis and we offer some suggestions for the anaesthetic management of these patients in the peri-operative period.
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    ABSTRACT: Inherited thrombophilia can be defined as a genetically determined predisposition to the development of thromboembolic complications. Since the discovery of activated protein C resistance in 1993, several additional disorders have been described and, at present, it is possible to identify an inherited predisposition in about 60 to 70% of patients with such complications. These inherited prothrombotic risk factors include qualitative or quantitative defects of coagulation factor inhibitors, increased levels or function of coagulation factors, defects of the fibrinolytic system, altered platelet function, and hyperhomocysteinemia. In this review, the main inherited prothrombotic risk factors are analyzed from epidemiological, laboratory, clinical, and therapeutic points of view. Finally, we discuss the synergism between genetic and acquired prothrombotic risk factors in particular conditions such as childhood and pregnancy.
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