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Congenital long QT syndrome (LQTS) represents a group of heart diseases of genetic origin characterized by prolongation of the QT interval and an abnormal T wave on the electrocardiogram (ECG). They can have a dominant or recessive expression, the latter associated with sensorineural deafness. In both cases, its clinical presentation is associated with recurrent syncope and sudden death as a consequence of ventricular tachycardia, specifically Torsades de Pointes. Currently they are classified according to the specific genetic defect, being able to compromise around 16 genes and almost 2000 mutations. It should be suspected in individuals with related symptoms, electrocardiographic findings, and family history. Management is based on the reduction or elimination of symptoms, and concomitantly the prevention of sudden death (SD), in those children with congenital deafness, the management requires the application of the otolaryngologist specialist's own measures. The cardiovascular management implies the modification of lifestyles, mainly the prohibition of competitive sports, including swimming, avoiding exposure to loud sounds or triggers. The medications used include beta-blockers, and more rarely flecainide, ranozaline, and verapamil; invasive management consists of the implantation of a cardioverter defibrillator or even left sympathetic denervation, each with its own risks and benefits. In any of the cases, we must avoid the circumstances that increase the QT interval, as well as carry out the appropriate analysis of the benefits and risks of each possible invasive measure. El síndrome QT largo (SQTL) congénito representa un grupo de enfermedades cardiacas de origen genético, caracterizado por la prolongación del intervalo QT y una onda T anormal en el electrocardiograma (ECG). Pueden tener una expresión dominante o recesiva, esta última asociada con sordera neurosensorial. En ambos casos su presentación clínica está asociada con síncopes recurrentes y muerte súbita como consecuencia de una taquicardia ventricular, específicamente a torsades de pointes. Actualmente se clasifican en función del defecto genético específico, pudiendo comprometer alrededor de 16 genes y casi 2000 mutaciones. Debe ser sospechada en individuos con la clínica relacionada, hallazgos electrocardiográficos y antecedentes familiares. El manejo está basado en la disminución o eliminación de los síntomas y, concomitantemente, la prevención de la muerte súbita (MS), en aquellos niños con sordera congénita el manejo requiere la aplicación de medidas propias del otorrinolaringólogo. Desde el punto de vista cardiovascular, el manejo implica la modificación de estilos de vida, principalmente la prohibición de deportes competitivos, entre ellos la natación, evitar la exposición a sonidos intensos o factores desencadenantes. La medicación usada abarca a los betabloqueadores y, más raramente, flecainida, ranozalina y verapamilo; el manejo invasivo consiste el implante de un cardiodesfibrilador o, incluso, la denervación simpática izquierda, cada una de ellas con sus propios riesgos y beneficios. En cualquiera de los casos debemos evitar las circunstancias que incrementen el intervalo QT, así como realizar el adecuado análisis de los beneficios y riesgos de cada posible medida invasiva.

Symptoms of hypopituitarism are usually chronic and nonspecific, but rarely the disease can have acute and life threatening manifestations. We report a 53 years old female with a pituitary adenoma that was admitted to our hospital because of syncope. The electrocardiogram showed sinus bradycardia with a prolonged QT interval. Frequent runs of non-sustained polymorphic ventricular tachycardia were noted on telemetry. The patient had a history of severe acute headaches in the previous days and laboratory tests revealed severe secondary hypothyroidism, adrenal insufficiency and a decrease in pituitary hormones. A magnetic resonance imaging of the head showed changes in the size and contrast enhancement of the adenoma. A diagnosis of hypopituitarism secondary to pituitary apoplexy was made and treatment with hydrocortisone and, subsequently, levothyroxine was started. Hormonal disorders such as hypothyroidism, adrenal insufficiency or hypopituitarism should be considered as unusual causes for reversible cardiomyopathy, long QT syndrome and ventricular arrhythmias.

Symptoms of hypopituitarism are usually chronic and nonspecific, but rarely the disease can have acute and life threatening manifestations. We report a 53 years old female with a pituitary adenoma that was admitted to our hospital because of syncope. The electrocardiogram showed sinus bradycardia with a prolonged QT interval. Frequent runs of non-sustained polymorphic ventricular tachycardia were noted on telemetry. The patient had a history of severe acute headaches in the previous days and laboratory tests revealed severe secondary hypothyroidism, adrenal insufficiency and a decrease in pituitary hormones. A magnetic resonance imaging of the head showed changes in the size and contrast enhancement of the adenoma. A diagnosis of hypopituitarism secondary to pituitary apoplexy was made and treatment with hydrocortisone and, subsequently, levothyroxine was started. Hormonal disorders such as hypothyroidism, adrenal insufficiency or hypopituitarism should be considered as unusual causes for reversible cardiomyopathy, long QT syndrome and ventricular arrhythmias.

Presentamos el caso de un varón tratado por convulsiones con diazepam en varias ocasiones. Finalmente se demuestra un síndrome de QT largo adquirido que le predisponía a torsades de pointes manifestándose con convulsiones y, por tanto, tratado erróneamente. Se repasa el síndrome del QT largo, describiendo las diferentes causas tanto congénitas como adquiridas, diagnóstico electrocardiográfico, la medición corregida del intervalo QT según la frecuencia cardiaca y tratamiento. The present is the case of a male treated on several occasions for seizures with diazepam. Eventually, an acquired long QT syndrome was demonstrated which predisposed him to torsades de pointes, manifesting as seizures and henceforth treated erroneously. The long QT syndrome is reviewed, describing the different causes of long QT interval, both congenital and acquired, electrocardiographic diagnosis, according to heart rate and treatment.

Introducción: el intervalo QTc prolongado ha sido identificado como factor de riesgo en arritmias ventriculares e incluso muerte súbita. Objetivo: establecer la prevalencia de QTc prolongado en pacientes internados. Metodología: estudio observacional, descriptivo, prospectivo que incluyó 123 pacientes internados en el Servicio de Clínica Médica y salas de urgencias del Hospital Nacional en 2014. Se consideró como QTc prolongado un valor > 0,44 seg en varones y >0,46 seg en mujeres en 4 derivaciones: aVL, DII, V5 y V6. Resultados: la prevalencia de QTc prolongado fue 26%, con predominio del sexo masculino (71%). La principal comorbilidad asociada fue la diabetes mellitus. Los electrolitos K+, Ca+2, Mg+ estaban bajos en la mayoría de estos afectados. El 16% de los pacientes con QTc prolongado terminaron en óbito. Conclusiones: se halló alta prevalencia de QTc prolongado (26%). Se requieren estudios más complejos para determinar la asociación de esta patología con las comorbilidades, los cuadros neurológicos, el uso de medicamentos y las alteraciones de los electrolitos detectadas. Introduction: The prolonged QTc interval has been identified as a risk factor for ventricular arrhythmias and even sudden death. Objective: To establish the prevalence of prolonged QTc in hospitalized patients. Methodology: Prospective descriptive observational study that included 123 patients hospitalized in the Service of Medical Clinic and urgency rooms of the National Hospital in 2014. A value > 0.44 seg in men and >0.46 seg in women for 4 derivations: aVL, DII, V5 and V6Se were considered as prolonged QTc. Results: The prevalence of QTc was 26% with a predominance of men (71%). The main associated comorbidity was diabetes mellitus. K+, Ca+2 and Mg+ electrolytes were low in most patients. A 16% of the patients with prolonged QTc died. Conclusions: A high prevalence (26%) of prolonged Qtc was found. More complex studies are required to determine the association of this pathology with the comorbidities, neurological symptoms, use of medicines and alteration of electrolytes detected.

Napoleón Bonaparte murió a la edad de 52 años mientras purgaba exilio en la isla de Santa Helena, en el Atlántico Sur, una colonia británica donde fue desterrado luego de su derrota en Waterloo en 1815. Desde 1961 han abundado las teorías de que Napoleón murió envenenado con arsénico, particularmente porque el análisis de sus cabellos mostró elevados niveles del elemento tóxico. Sin embargo, de acuerdo a una nueva investigación sobre la causa de muerte del emperador francés, su muerte tuvo origen en una causa más prosaica de lo que muchos pensarían, sucumbió a un cáncer del estómago más que por envenenamiento arsenical. La autopsia describe un tumor gástrico de 10 cm de extensión. Otras fuentes históricas muestran que el obeso líder francés había perdido cerca de nueve kilogramos de peso en los últimos meses de su vida, otro signo de cáncer gástrico. La cavidad gástrica estaba llena de un líquido en borra de café, un claro signo de importante sangrado en el tracto digestivo. Este sangrado masivo, fue la causa inmediata de su muerte. Napoleon Bonaparte died at age 52 while in exile on the South Atlantic island of Saint Helena, a british colony, where he was banished after his defeat at the battle of Waterloo in 1815. Theories that Napoleon was poisoned with arsenic have abounded since 1961, when an analysis of his hair showed elevated levels of the toxic element. According to new research into what killed the french emperor his death’ cause was more prosaic than some people would like to think, succumbing to stomach cancer rather than arsenic poisoning, The autopsy describes a tumor in his stomach that was 4 inches (10 centimeters) long. Other historical sources indicate that the rotund French leader had lost about 20 pounds (nine kilograms) in the last few months of his life, another sign of stomach cancer. His stomach also contained a dark material similar to coffee grounds, a telltale sign of extensive bleeding in the digestive tract. The massive bleeding was likely the immediate cause of death.

La mayoría de los casos de intoxicación aguda por arsénico se producen por la ingestión accidental o voluntaria de insecticidas o pesticidas, y sus manifestaciones clínicas reflejan afectación de todos los sistemas corporales. El arsénico puede prolongar el intervalo QT y causar torsades de Pointes, un tipo de arritmia con riesgo vital característica de la prolongación del intervalo QT. En una revisión de la literatura sólo hemos encontrado cinco casos de torsades de Pointes debidas a la intoxicación aguda por arsénico. Recientemente, se han publicado cuatro casos adicionales en pacientes con leucemia promielocítica aguda refractaria o recurrente tratados con trióxido de arsénico. En los nueve casos, las torsades de Pointes aparecieron tardíamente tras la intoxicación. Se describe un caso de intoxicación aguda por arsénico que tuvo torsades de Pointes precoces, hipopotasemia y altos niveles séricos de troponina I. Most cases of acute arsenic poisoning occur through accidental or voluntary ingestion of pesticides or insecticides, and all body systems are affected. Arsenic can prolong the QT interval and lead to torsades of Pointes, a crucial type of arrhythmia characteristic of such QT interval prolongation. In our revision of the literature, there have been found only 5 cases of torsades of Pointes due to acute arsenic poisoning. Recently, there have been published four additional cases in patients with refractory or recurrent acute promyelocytic leukemia being treated with arsenic trioxide. In all nine cases, torsades of pointes appeared slowly after poisoning. Herein is described a case of acute arsenic poisoning which led to an early onset of torsades of Pointes, hypopotasemia and high levels of serum troponin I.