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Síndrome de QT prolongado congénito y embarazo: reporte de dos casos

Keywords: congenital long qt, pregnancy, implantable cardioverter defibrillator.

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Abstract:

congenital long qt syndrome is a clinical entity characterized by impairment of myocardial repolarization given by significant prolongation of the corrected qt interval with an increased risk of syncope, polymorphic ventricular tachycardia and sudden death. this is produced by an alteration in the function of ion channels responsible for the action potential of cardiac cells as a consequence of multiple mutations, the most common of which are in the sodium and potassium channels. the relationship with pregnancy and especially the presence of events in the postpartum period is clearly determined by the presence of ventricular arrhythmias or episodes of sudden death, that should lead to a thorough evaluation of prolonged qtc and its triggers or concomitant diseases. we present the clinical records of two patients who had sudden death during the postpartum and were diagnosed as congenital long qt syndrome.

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