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      Muerte súbita por causa eléctrica en sujetos sin enfermedad cardíaca estructural demostrable: Experiencia cubana Translated title: Sudden death due to electrical causes in individuals without demonstrable structural cardiac disease: Experience in Cuba

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          Abstract

          La muerte súbita cardíaca de causa eléctrica en sujetos con corazón "sano", constituye un problema clínico y de salud pública, aún no resuelto. Los objetivos del trabajo fueron: caracterizar pacientes reanimados de un evento de muerte súbita de causa eléctrica y conocer su evolución intervenida en tres años; y estudiar los signos eléctricos premonitorios de muerte súbita. Se estudiaron 42 sujetos, 30 hombres y 12 mujeres, edad promedio 37.7 años, con corazón "sano", por métodos clínicos y paraclínicos. Se consideraron 9 subpoblaciones, en mayor número los síndromes de Brugada y de QT largo y la fibrilación ventricular idiopática. Las arritmias responsables del evento fueron, en primer lugar, la fibrilación ventricular y la torsión de puntas. Existieron signos premonitorios en el 92.8% de los pacientes. Fueron frecuentes las recidivas de las arritmias malignas (71.4%) aunque en la estimulación eléctrica programada, sólo se logró reproducirlas en 4 de 18 pacientes. La fibrilación auricular predominó como arritmia coexistente (19%). En resumen, son frecuentes los signos premonitorios (en especial la fibrilación auricular) y las recidivas de las arritmias malignas aunque su inducibilidad en el laboratorio es pobre. La estratificación de riesgo es muy difícil, por el bajo valor predictivo de los métodos diagnósticos.

          Translated abstract

          Sudden cardiac death due to electrical causes in individuals with no evidence of structural heart disease is an important clinical and public health problem, and it is not yet solved. The objectives of this study were: to characterize patients reanimated from a sudden death event of electrical cause; to know the mediated evolution during a period of three years and to study premonitory electrical signs. 42 individuals were studied, 30 were male and 12 female, mean age 37.7 years, healthy heart, by clinic and paraclinic methods. Nine subpopulations were studied, being Brugada syndrome, long QT syndrome and idiopathic ventricular fibrillation the most frequent. Ventricular fibrillation and twisting of the points were the arrhythmias responsible for most death events. There were premonitory signs in 92.8% and clinical recurrences of life-threatening events in 71.4% but they were induced during programmed electrical stimulation only in 4 of 18 patients. Atrial fibrillation was the most frequent coexistent arrhythmia (19%). In summary, there are frequent premonitory signs (particularly atrial fibrillation), and also malignant arrhythmic recurrences but a poor inducibility at the electrophysiology laboratory. It is very difficult to stratify the risk because of the low predictive value of diagnostic methods.

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          Most cited references28

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          Proposed diagnostic criteria for the Brugada syndrome: consensus report.

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            Sudden cardiac death with apparently normal heart.

            Mechanisms of sudden cardiac death (SCD) in subjects with apparently normal hearts are poorly understood. In survivors, clinical investigations may not establish normal cardiac structure with certainty. Large autopsy series may provide a unique opportunity to confirm structural normalcy of the heart before reviewing a patient's clinical history. We identified and reexamined structurally normal hearts from a 13-year series of archived hearts of patients who had sudden cardiac death. Subsequently, for each patient with a structurally normal heart, a detailed review of the circumstances of death as well as clinical history was performed. Of 270 archived SCD hearts identified, 190 were male and 80 female (mean age 42 years); 256 (95%) had evidence of structural abnormalities and 14 (5%) were structurally normal. In the group with structurally normal hearts (mean age 35 years), SCD was the first manifestation of disease in 7 (50%) of the 14 cases. In 6 cases, substances were identified in serum at postmortem examination without evidence of drug overdose; 2 of these chemicals have known associations with SCD. On analysis of ECGs, preexcitation was found in 2 cases. Comorbid conditions identified were seizure disorder and obesity (2 cases each). In 6 cases, there were no identifiable conditions associated with SCD. In 50% of cases of SCD with structurally normal hearts, sudden death was the first manifestation of disease. An approach combining archived heart examinations with detailed review of the clinical history was effective in elucidating potential SCD mechanisms in 57% of cases.
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              Cellular basis for QT dispersion.

              The cellular basis for the dispersion of the QT interval recorded at the body surface is incompletely understood. Contributing to QT dispersion are heterogeneities of repolarization time in the three-dimensional structure of the ventricular myocardium, which are secondary to regional differences in action potential duration (APD) and activation time. While differences in APD occur along the apicobasal and anteroposterior axes in both epicardium and endocardium of many species, transitions are usually gradual. Recent studies have also demonstrated important APD gradients along the transmural axis. Because transmural heterogeneities in repolarization time are more abrupt than those recorded along the surfaces of the heart, they may represent a more onerous substrate for the development of arrhythmias, and their quantitation may provide a valuable tool for evaluation of arrhythmia risk. Our data, derived from the arterially perfused canine left ventricular wedge preparation, suggest that transmural gradients of voltage during repolarization contribute importantly to the inscription of the T wave. The start of the T wave is caused by a more rapid decline of the plateau, or phase 2 of the epicardial action potential, creating a voltage gradient across the wall. The gradient increases as the epicardial action potential continues to repolarize, reaching a maximum with full repolarization of epicardium; this juncture marks the peak of the T wave. The next region to repolarize is endocardium, giving rise to the initial descending limb of the upright T wave. The last region to repolarize is the M region, contributing to the final segment of the T wave. Full repolarization of the M region marks the end of the T wave. The time interval between the peak and the end of the T wave therefore represents the transmural dispersion of repolarization. Conditions known to augment QTc dispersion, including acquired long QT syndrome (class IA or III antiarrhythmics) lead to augmentation of transmural dispersion of repolarization in the wedge, due to a preferential effect of the drugs to prolong the M cell action potential. Antiarrhythmic agents known to diminish QTc dispersion, such as amiodarone, also diminish transmural dispersion of repolarization in the wedge by causing a preferential prolongation of APD in epicardium and endocardium. While exaggerated transmural heterogeneity clearly can provide the substrate for reentry, a precipitating event in the form of a premature beat that penetrates the vulnerable window is usually required to initiate the reentrant arrhythmia. In long QT syndrome, the trigger is thought to be an early afterdepolarization (EAD)-induced triggered beat. The likelihood of developing EADs and triggered activity is increased when repolarizing forces are diminished, making for a slower and more gradual repolarization of phases 2 and 3 of the action potential, which translates into broad, low amplitude and sometimes bifurcated T waves in the electrocardiogram. Our findings suggest that regional differences in the duration of the M cell action potential may be the basis for QT dispersion measured at the body surface under normal and long QT conditions. The data indicate that the interval delimited by the peak and the end of the T wave represents an accurate measure of regional dispersion of repolarization across the ventricular wall and as such may be a valuable index for assessment of arrhythmic risk. The presence of low amplitude, broad and/or bifurcated T waves, particularly under conditions of long QT syndrome, is indicative of diminished repolarizing forces and may represent an independent variable of arrhythmic risk, forecasting the development of EAD-induced triggered beats that can precipitate torsade de pointes. Although the QT interval, QT dispersion, the T wave peak-to-end interval, and the width and amplitude of the T wave often change in parallel, they contain different information and should not be expected to be e
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                Author and article information

                Contributors
                Role: ND
                Role: ND
                Role: ND
                Role: ND
                Role: ND
                Role: ND
                Journal
                acm
                Archivos de cardiología de México
                Arch. Cardiol. Méx.
                Elsevier (México )
                1405-9940
                December 2004
                : 74
                : 4
                : 283-289
                Affiliations
                [1 ] Instituto de Cardiología y Cirugía Cardiovascular Cuba
                Article
                S1405-99402004000400005
                792e0eb3-4456-4235-9a0d-15f2591bd042

                http://creativecommons.org/licenses/by/4.0/

                History
                Categories
                Cardiac & Cardiovascular Systems

                Cardiovascular Medicine
                Sudden cardiac death,Normal heart,Malignant arrhythmias,Muerte súbita cardíaca,Corazón sano,Arritmias malignas

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