Revista portuguesa de cardiologia : orgão oficial da Sociedade Portuguesa de Cardiologia = Portuguese journal of cardiology : an official journal of the Portuguese Society of Cardiology
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Congenital long QT syndrome (LQTS) is associated to an increased risk of ventricular arrhythmia, syncope and sudden cardiac death (SD). Four disease genes have been identified and different mutations described in each gene. This locus heterogenicity appears to have important functional and prognostic implications. ⋯ Other therapeutic options include left cervicothoracic sympathectomy, pacemakers, and the implantable cardioverter defibrillator. Risk stratification and efficacy of the subsequent treatment has significantly changed the clinical outcome of patients with LQTS. Recent molecular biology studies and data analysis from the International LQTS Registry may contribute to the definition of the best strategy for the future.
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In recent years there has been growing evidence that high levels of plasmatic homocysteine constitute an independent risk factor for early cardiovascular disease. In this article we review the main theories of atherosclerosis which take into account the proteins, namely homocysteine, homocysteine metabolism, the cause that may be responsible for high levels of homocysteinemia, the pathophysiologic mechanisms of vascular lesion induced by hyperhomocysteinemia, the clinical evidence that homocysteinemia constitutes a vascular risk factor and finally, the evidence that it is possible to control homocysteinemia with supplementation of co-factors of homocysteine metabolism, namely vitamin B6, B12 or folic acid.
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Case Reports
[A pseudoaneurysm of the mitral-aortic intervalvular fibrosis after aortic valve replacement].
A 58-year-old male underwent a transesophageal echocardiogram for suspected aortic valve infective endocarditis, four years after surgery. The examination, performed with a multiplane probe, showed a pulsatile (systolic expansion) echo-free cavity in the intervalvular mitral-aortic region, communicating with the left ventricle outflow tract, with no signs of rupture to the left atrium or the aorta. These findings meet the criteria for the diagnosis of pseudoaneurysm of the mitral-aortic intervalvular fibrosa.