Prevalencia y espectro de las enfermedades que predisponen a la muerte súbita cardiaca en niños mexicanos: una muestra obtenida del Hospital Infantil de México Federico Gómez / Prevalence and spectrum of diseases that predispose to sudden cardiac death in Mexican children: a sample obtained from The Federico Gomez Children's Hospital of Mexico

Resumen Objetivo: Determinar la prevalencia y espectro de las enfermedades que predisponen la muerte súbita cardiaca en niños mexicanos e identificar los principales signos y síntomas tempranos que pueden permitir al personal de salud sospechar acerca de estas enfermedades y referir a los pacientes a un hospital de tercer nivel de manera temprana. Métodos: La incidencia, prevalencia y prevalencia de periodo, así como los primeros síntomas, los datos clínicos y el seguimiento, se describen en todos los niños con enfermedades que predisponen a la muerte súbita cardiaca en el Hospital Infantil de México. Resultados: Cincuenta y nueve pacientes de 8 ± 5 años, 40 con miocardiopatías y 19 con enfermedades arritmogénicas hereditarias. La prevalencia del periodo fue de 9.5/1,000 pacientes/año. Los primeros síntomas más comunes fueron disnea, palpitaciones y síncope. En 9 casos se encontró un patrón de herencia mendeliana. Tres pacientes fallecieron de muerte súbita cardiaca durante el periodo de estudio. Conclusión: Las enfermedades que predisponen a la muerte súbita cardiaca en los niños no son muy conocidas por la comunidad médica y general. Todo niño con disnea, palpitaciones y/o síncope debe referirse para la búsqueda intensiva de estas enfermedades. Una evaluación cardiológica completa en todos los miembros de la familia está indicada.

Abstract Objective: To determine the prevalence and spectrum of diseases that predispose to sudden cardiac death in Mexican children, and to identify the main early signs and symptoms that can enable the health personnel to suspect these diseases and to refer the patients to a tertiary hospital in a timely manner. Methods: Incidence, prevalence, and period prevalence, as well as early symptoms, clinical data, and follow-up were recorded on all children found with diseases that predispose to sudden cardiac death in The Children's Hospital of Mexico. Results: The study included 59 patients, with a mean age of 8 ± 5 years old, with 40 cardiomyopathies, and 19 with inherited arrhythmogenic diseases. The period prevalence was 9.5/1,000 patients/year. The most common early symptoms were dyspnoea, palpitations, and syncope. A Mendelian inheritance pattern was found in 9 cases. Three patients died of sudden cardiac death during the period of the study. Conclusion: Diseases that predispose to sudden cardiac death in children are not very well known by the general medical community. Every child with dyspnoea, palpitations and/or syncope, should be referred for the intensive search of these diseases. A complete cardiological evaluation in all members of the family is indicated.

Síncope único o muy poco frecuente en 104 casos / Single or very isolated syncope in 104 cases

Resumen Introducción: Es frecuente de encontrar personas enviadas para realizar la prueba de Tilt Test (TT), que sufrieron un solo síncope aislado o que ocurre muy ocasionalmente durante su vida. En este trabajo nos preguntamos en qué se diferencian estas personas de las que nunca tienen síncopes. Métodos: Realizamos el examen de TT en 104 pacientes que sufrieron sólo uno o como máximo 5 síncopes por cualquier causa durante su vida. Intentamos explicar cómo actúan los diferentes factores predisponentes para provocar el síncope. Resultados: Encontramos diferencias en factores predisponentes del síncope, entre nuestros pacientes y los controles en factores como: la herencia, la hipermovilidad articular, el encharcamiento venoso durante el TT, la ingesta de alimentos, el uso de fármacos, el estrés y la emoción. Conclusiones: En el síncope único o muy ocasional, deben conjugarse en la persona dos factores. Lo explicamos: Estos pacientes tienen una predisposición orgánica (herencia -hipermovilidad articular- falla en los baroreflejos, algunas enfermedades neurológicas, etc.) a presentar síncopes. Pero los síncopes no ocurrirán a menos que se agregue simultáneamente un factor ambiental, que actúa como un desencadenante (estadía larga de pie, estrés, dolor y emoción, deshidratación, drogas, comida abundante, etc.), es por eso que en estos pacientes los síncopes son tan raros.

Introduction: It is frequent meeting people sent to perform a tilt test suffering from a single or such isolated syncope that occur very occasionally during the patient's life. We ask ourselves how these people differ from those who never have syncopes. Methods: We performed tilt test in 104 patients who suffer a single or maximum 5 syncopes from any cause during their lifetimes. We try to explain how different predisposing factors act to provoke syncope. Results: We found differences between cases and controls in inheritance of syncopes, joint hypermobility, venous pooling during tilt test, food intake, use of drugs, stress and emotion as a trigger for syncope. Conclusions: Patients with single or occasional syncope have an organic predisposition (inheritance - joint hypermobility - failure in the baroreflexes, some neurological diseases, etc.) to present syncopes, but syncopes do not occur unless it is added simultaneously an environmental factor, which acts as a trigger (prolonged standing up - stress, pain and emotion - dehydration - drugs - abundant food, etc.) that is why these episodes are so rare.

Síndrome da morte súbita do lactente / Sudden infant death syndrome

Entre as diversas causas de morte na infância, a Síndrome da Morte Súbita do Lactente (SMSL) assume posição de destaque. Estudos conduzidos nas últimas décadas foram capazes de elucidar os principais fatores epidemiológicos envolvidos, permitindo uma abordagem eficaz para reduzir significativamente a mortalidade por esta condição.

Among the many causes of death in childhood, Sudden Infant Death Syndrome (SIDS) assumes a prominent position. Researches conducted in recent decades were able to elucidate the main epidemiological factors involved, enabling effective approach to significantly reduce mortality from this condition.

Echocardiographic findings in a contemporary Afro-Caribbean population referred for evaluation of unexplained syncope / Hallazgos ecocardiográficos en una población afrocaribeña contemporánea referida para evaluación del síncope inexplicado

Echocardiographic findings were reviewed for 106 patients (mean age 41.3 ± 23.0 years, range 3 to 90 years, 61% female) referred for evaluation of unexplained syncope. Abnormal echocardiographic findings were seen in 36/106 (34%) patients, of which 12/106 (11%) may have an abnormality that contributed to symptoms. Abnormal echocardiographic findings (64 vs 6%, p < 0.01) and those possibly causing syncope (22 vs 0%, p < 0.05) were significantly more likely in the oldest tercile of patients compared with the youngest. No patient less than 35 years old had a possibly diagnostic abnormality.

Se revisaron los hallazgos ecocardiográficos de 106 pacientes, edad promedio 41.3 ± 23.0 años, rango 3 a 90 años, 61% hembras, referidos para evaluación del síncope inexplicado. Se observaron hallazgos ecocardiográficos anormales en 36/106 (34%) pacientes, de los cuales 12/106 (11%), podían tener una anormalidad que contribuyera a los síntomas. Los hallazgos ecocardiográficos anormales (64 versus 6%, p < 0.01) y aquellos que posiblemente causan síncope (22 versus 0%, p < 0.05) fueron significativamente más probables en el tercil de pacientes de mayor edad en comparación con los más jóvenes. Ningún paciente menor de 35 años de edad tuvo alguna anormalidad diagnóstica.

Relevância diagnóstica da massagem do seio carotídeo durante teste de inclinação / Diagnostic relevance of the carotid sinus massage during a head up tilt table test (HUTT)

FUNDAMENTO: As síncopes por hipersensibilidade do seio carotídeo (HSC) podem ser pouco diagnosticadas por causa da baixa desconfiança diagnóstica ou do medo de complicações da própria massagem do seio carotídeo. OBJETIVO: Investigar o papel da hipersensibilidade do seio carotídeo (HSC) em desmaios não-convulsivos e quedas inexplicadas. MÉTODOS: Duzentos e cinqüenta e nove pacientes com idade média de 50±24 anos, encaminhados para investigação de desmaios não-convulsivos e quedas inexplicadas, foram submetidos ao Teste da Mesa Inclinada (TI) sem ("passivo") e com provocação farmacológica ("sensibilizado"). Exames clínicos e complementares não revelaram causa óbvia para as queixas. Cinqüenta e cinco voluntários com idade média de 57±21 anos sem história de epilepsia, desmaios e quedas serviram de controles. Todos os participantes foram submetidos ao TI precedido por estimulação digital dos seios carotídeos a zero e a 60º. RESULTADOS: A estimulação dos seios carotídeos foi positiva em 4 controles. O diagnóstico clínico de HSC foi endossado por resposta positiva à estimulação carotídea a 60º em 28 pacientes, em sua maioria homens idosos. A estimulação carotídea foi positiva a 0º em apenas três desses pacientes. Não houve diferença estatística no TI entre os pacientes com e sem HSC. CONCLUSÃO: A estimulação dos seios carotídeos a 60º deve ser incluída na avaliação rotineira de pacientes com desmaios não-convulsivos ou quedas inexplicadas, uma vez que os exames cardiovasculares de rotina, incluindo o TI, não foram úteis para estabelecer o diagnóstico nesses casos. Novos estudos em indivíduos normais são necessários para estabelecer o significado da resposta positiva à estimulação carotídea na ausência de história de desmaios ou quedas.

BACKGROUND: Syncope due to carotid sinus hypersensitivity (CSH) may be underdiagnosed due to a low level of diagnostic suspicion and fear of complications caused by massage of the carotid sinus. OBJECTIVE: To investigate the relevance of carotid sinus massage in the diagnosis of non-convulsive faints and unexplained falls. METHODS: Two-hundred and fifty-nine patients with a mean age of 50±24 years referred for the investigation of non-convulsive faints or unexplained falls were evaluated with a head up tilt table test (HUTT) without ("passive") and with pharmacological stimulation. Clinical and laboratory work-up did not reveal an obvious cause for the complaints. Fifty-five volunteers with a mean age of 57±21 years with no history of seizures, faints or falls were used as controls. All participants underwent a HUTT preceded by digital stimulation of each carotid sinus both at zero and 60º. RESULTS: Carotid sinus massage was positive in 4 controls. The relevance of CSH in the evaluation of syncope was supported by a positive response to carotid sinus massage at 60º in 28 patients, most of whom were elderly men. Carotid sinus massage was positive at 0º in only three of such patients. The results of the HUTT did not show statistical difference between patients with and without CSH. CONCLUSION: Carotid sinus massage at 60º under controlled conditions should be included in the assessment of patients with non-convulsive faints or unexplained falls. Routine cardiovascular exams, including the HUTT, are not useful for the diagnosis in such cases. Further studies in normal individuals are needed to establish the significance of a positive response to carotid sinus massage in the absence of a history of fainting or falling.

Clinical profile of pediatric somatoform disorders.

OBJECTIVES: To study the prevalence, pattern, clinical and socio-demographic characteristics of somatoform disorders in children. METHODS: From Aug 2004 to July 2005, children up to 18 years with unexplained physical symptoms were evaluated prospectively using DSM-IV criteria. Detailed evaluation followed for those meeting criteria. RESULTS: The prevalence of Somatoform disorders was 0.59% and 0.78% among out-door and in-door patients respectively. Among 124 children (40 boys and 84 girls) meeting criteria, conversion disorder was the commonest (57.3%), followed by undifferentiated somatoform disorder (25.2%). Girls were significantly more represented among conversion disorder patients compared to other groups of somatoform disorders (78.9 vs. 52.8 %, P=0.002). In conversion disorder, 2/3rd patients presented within 3 months, whereas in other somatoform disorders, 2/3rd patients presented within 3 months after symptoms. Fainting attacks (52.1%) and ataxia (43.7%) in conversion disorder and pain abdomen (52.8%) and headache (52.8%) in other somatoform disorders, were the commonest symptoms. Stressors were identified in 73.4% and acute precipitating stressors were present in 14.4% children. Boys had significantly higher rates of poor inter-personal relations and communication problems within the family (72.5% vs. 41.7%, P=0.001), while girls had significantly higher rates of conflicts with the parents and other family members (21.4% vs. 5%, P=0.02). CONCLUSION: Somatoform disorders, particularly conversion disorder is commoner in girls. Important stressors are poor inter-personal relations and communication problems within the family in boys, and conflicts with family members among girls.

Perdas transitórias de consciência e convulsões: epilepsia ou síncope? / Transient loss of consciousness, convulsions and falls in patients with normal or nonspecific electroencephalographic findings: syncope or epilepsy?

Introdução: pacientes com perdas transitórias da consciência e convulsões são quase sempre considerados epiléticos, mesmo que o eletroencefalograma seja normal. Um diagnóstico equivocado pode expor o paciente por muito tempo a drogas anticonvulsivas desnecessárias e expô-lo a situações de risco, quando a doença de base for uma cardiopatia grave passível de tratamento. Objetivo: avaliar a proporção de pacientes...

Síncope / Syncope

Síncope é um evento clínico bastante comum, de difícil diagnóstico, muitas vezes incapacitante e, em diversas situaçöes, de mau prognóstico. Muitas são as possíveis etiologias e o principal papel do médico é distinguir causas benignas daquelas de mau prognóstico. Na maioria das vezes, muitos testes dispendiosos têm sido realizados, sem que se atinja o sucesso esperado. O objetivo deste artigo será determinar os principais mecanismos fisiopatológicos da síncope e qual a melhor estratégia para que se determine a etiologia e, portanto, a terapêutica ideal.

Evaluación Diagnóstica del Paciente con Síncope

La evaluación diagnóstica del paciente con síncope recurrente es un reto para el clínico. A pesar de grandes avances diagnóstico, la etiologìa de los episodios sincopales recurrentes permanece sin aclarar en más del 50 por ciento de los casos; sinembargo en la última década de introducción de nuevos métodos no invasivos tales como la electrocardiografía de señal promediada y la utilización de pruebas de función autonómica, especialmente la prueba con mesa inclinada, han contribuído a clarificar el diagnóstico en más del 60 por ciento de los paicentes que consultan por síncope de etiología desconocida. Este artículo revisa las indicaciones de los diferentes exámenes diagnósticos disponibles para la evaluación del paciente con síncope recurrente y propone la utilización de un esquema racional para la evaluación diagnóstica de estos pacientes

Síncope: una visión global / Syncope: a global vision

En este artículo se revisa la epidemiología, clasificación, fisiopatología, enfoque diagnóstico y terapéuticos del cuadro conocido como síncope