Prevalence of joint hypermobility, postural orthostatic tachycardia syndrome (POTS), and orthostatic hypotension in school-children. / Prevalencia de la hipermovilidad articular, el síndrome de taquicardia postural (POTS) y la hipotensión ortostática en escolares.

INTRODUCTION: Children with joint hypermobility, postural orthostatic tachycardia syndrome, and orthostatic hypotension report autonomic symptoms such as dizziness, nausea, headaches, and palpitations. It is unclear if there is a pathophysiological link between connective tissue disorders and autonomic symptoms. There is no published data on the prevalence of disorder at the community level. PRIMARY OBJECTIVE: To assess the prevalence of joint hypermobility, orthostatic hypotension, and postural or thostatic tachycardia syndrome in children. SECONDARY OBJECTIVE: To determine the relationship bet ween joint hypermobility, orthostatic hypotension, and postural orthostatic tachycardia syndrome. PATIENTS AND METHOD: Participants aged 10 to 18 years were selected from public schools in three Colombian cities. The surveys included historical questions on the incidence of dizziness, nausea, headache, tremor, blurred vision, vertigo, anxiety, near syncope and syncope, sweating, palpitations triggered by standing in the two months prior to the investigation. Each of these signs and symptoms was also assessed during the recumbency (10 minutes) and standing (2, 5 and 10 minutes) phases of the investigation. HR and BP measurements were obtained at the same intervals. Joint mobility was measured with a mechanical goniometer and assessed with the Beighton score. RESULTS: Prevalence of joint hyperlaxity: 87 of 306 (28.4%). Prevalence of orthostatic hypotension: 5 of 306 (1.6%). Prevalen ce of postural orthostatic tachycardia syndrome: 6 of 306 (2.0%). Of 87 children with joint hyperlaxi ty, only 1 child had joint hyperlaxity at the same time as postural hypotension (1.2%) (p = 0.6735), and 1 child had joint hyperlaxity and postural orthostatic tachycardia syndrome simultaneously (1.2%) (p = 0.5188). CONCLUSION: Children with joint hyperlaxity did not have a higher prevalence of postural orthostatic tachycardia syndrome and orthostatic hypotension. It seems unlikely that con nective tissue disorders are responsible for most cases of postural orthostatic tachycardia syndrome and orthostatic hypotension in the community. Of note, the pathophysiology of postural orthostatic tachycardia syndrome and orthostatic hypotension requires further investigation.

[Carotid sinus syndrome associated to cervical B-cell lymphoma]. / Síndrome del seno carotídeo asociado a linfoma B cervical.

We present the case of a 75-year-old male with history of 5 months of right submaxillary tumor, with no clear etiology, who consulted for episodes of syncope preceded by dyspnea and sudden onset of profuse sweating. During his stay at the hospital, electrocardiographic records showed more than 3-second-long pauses after head movements, with a sensation of imminent loss of consciousness associated. A temporary pacemaker was required. Biopsy showed a diffuse non-germinal large B-cell B lymphoma, which required chemotherapy. After the first chemotherapy session, a significant reduction of the tumoral mass was observed, with abolition of the symptoms associated to cervical movements. The definitive diagnosis was malignant carotid sinus syndrome associated with cervical lymphoma.

Se describe el caso de un varón de 75 años con tumoración submaxilar derecha de 5 meses de evolución sin diagnóstico etiológico, que consultó por episodios de síncope precedidos por disnea y sudoración profusa de instalación súbita. Durante su internación se detectaron, en el monitoreo electrocardiográfico, pausas mayores a 3 segundos luego de la lateralización cervical reproduciendo los síntomas mencionados y asociadas a sensación de pérdida inminente de la conciencia, por lo que se implantó marcapasos transitorio. Se realizó biopsia de la masa tumoral cervical, consistente con linfoma B difuso de células grandes no centrogerminal, y se inició quimioterapia. Luego de dicho tratamiento quimioterápico se observó reducción significativa de la masa tumoral con abolición de los síntomas asociados a las maniobras cervicales. El diagnóstico definitivo fue síndrome del seno carotídeo maligno asociado a linfoma cervical.

Síncope y paro cardiorrespiratorio, formas graves de presentación de la embolia pulmonar central: reporte de tres casos clínicos / Syncope and cardiorespiratory arrest, severe forms of presentation of central pulmonary embolism: report of three clinical cases / Síncope e parada cardiorrespiratória, formas graves de apresentação de embolia pulmonar central: relato de três casos clínicos

El tromboembolismo pulmonar tiene una presentación clínica variada. Es fundamental tener un alto índice de sospecha para arribar al diagnóstico oportuno. El síncope se asocia a casos graves y tiene importancia pronóstica. El tratamiento trombolítico es la piedra angular en el subgrupo de pacientes de alto riesgo. Se presentan tres casos clínicos de tromboembolia pulmonar de alto riesgo con el fin de discutir el escenario clínico de presentación y el tratamiento instaurado.

Pulmonary thromboembolism has a varied clinical presentation. It is essential to have a high index of suspicion to arrive at a timely diagnosis. Syncope is associated with severe cases and is of prognostic significance. Thrombolytic treatment is the cornerstone in the subgroup of high-risk patients. Three clinical cases of high-risk pulmonary thromboembolism are presented in order to discuss the clinical presentation scenario and the established treatment.

O tromboembolismo pulmonar tem a presentação clínica variada. É essencial ter um alto índice de suspeita para chegar a um diagnóstico oportuno. A síncope está associada a casos graves e tem significado prognóstico. O tratamento trombolítico é a pedra angular no subgrupo de pacientes de alto risco. São apresentados três casos clínicos de tromboembolismo pulmonar de alto risco para discutir o quadro clínico e o tratamento instituído.

Age-Related Changes in Inferior Vena Cava Dimensions among Children and Adolescents with Syncope.

OBJECTIVE: To test the hypothesis that increased venous compliance manifested as inferior vena cava (IVC) dilation is an important substrate for syncope in children. STUDY DESIGN: IVC diameters were measured in 191 children and adolescents with syncope and in 95 controls. Subjects were divided based on age <12 years (younger group) and ≥12 years (older group). IVC measurements at the right atrial junction (IVC-RA), 10 mm below the IVC-RA junction (IVC-RA10), and at the point of maximal diameter (IVCmax) were made. The linear relation to body surface area (BSA) was confirmed, as were dimensions indexed to BSA (iIVC). Relationships between iIVC and the time of day were evaluated. RESULTS: In the syncope group, the mean age was 12.9 ± 3.6 years, mean weight was 54.7 ± 23 kg, and mean BSA was 1.5 ± 0.4 m2. Among controls, all IVC dimensions varied linearly with BSA (P < .001). In the older group (140 patients with syncope and 60 controls), all iIVC dimensions were larger in the syncope cohort: iIVC-RA, 9 vs 7.7 mm/m2 (P < .0001); iIVC-RA10, 9.4 vs 8.1 mm/m2 (P < .0001); iIVCmax, 11.7 vs 10.6 mm/m2 (P = .002). In the younger group (51 patients with syncope and 35 controls), there were no differences in iIVC measurements between the syncope cohort and controls: iIVC-RA, 10.2 vs 11.3 mm/m2; iIVC-RA10, 11.7 vs 12.0 mm/m2; iIVCmax, 14.2 vs 14.7 mm/m2 (P > .05 for all). CONCLUSIONS: The IVC is enlarged in teenagers with syncope compared with controls, suggesting that venous capacitance and resultant pooling play roles in the pathogenesis of syncope. In contrast, younger children with syncope do not demonstrate IVC dilation, suggesting that their syncope arises from a different mechanism.

Foramen Magnum Meningioma Presenting with Cough Syncope (Case Report and Review of the Literature)

Foramen magnum meningiomas cause different symptoms based on the size and the location of the tumor. They often present with involvement of the long tracts and of the lower cranial nerves.Ataxia and occipitocervical headache are other common symptoms. In the present study, we report a case of foramen magnum meningioma presenting with cough syncope. A mass lesion located anterolateral to the foramenmagnumwas detected in a 38-year-oldmanduring amagnetic resonance imaging (MRI) exam; the lesion extended from the inferior clivus to the level of the C2 vertebra. The neural axis has pushed towards posterior and contralateral side by the mass. We think that syncope occurred due to the encasement of the vertebral arteries by the tumor in addition to the compression of the neural axis. The posterolateral approach without condylar resection provides a safe surgical plane for total excision of these tumors. In our case, the tumor was totally removed and the syncope episodes were resolved.

Múltiplas causas de síncope em pacientes com distrofia miotônica tipo I / Multiple causes of syncope in patients with myotonic dystrophy type I

A distrofia miotônica é a doença neuromuscular mais frequente na população adulta. Embora tenha caráter multissistêmico, apresenta especial predileção pelo sistema de condução cardíaco, manifestando-se tanto com bloqueios atrioventriculares como com taquiarritmias ventriculares e supraventriculares. O foco deste trabalho é apresentar, através do relato de um caso, a importância de uma investigação mais detalhada dos casos de síncope em pacientes portadores de distrofia miotônica, pois alterações inaparentes e potencialmente graves podem passar despercebidas

Myotonic dystrophy is the most frequent neuromuscular disease in the adult population. Although it is a multisystem disease, it usually has a special preference for the cardiac conduction system manifesting itself as atrioventricular conduction block and as ventricular and supraventricular tachyarrhythmias. The focus of this work is to use a case report to demonstrate the importance of a more detailed investigation of syncope in patients with myotonic dystrophy, since unapparent and potentially serious changes may go by unnoticed

Síndrome do QT longo congênito: o que sabemos até o momento? / Congenital long QT syndrome: what do we know so far?

Síndrome do QT longo congênito é uma síndrome arrítmica hereditária caracterizada por prolongamento do intervalo QT no eletrocardiograma de 12 derivações, torsades de pointes e maior chance de morte súbita cardíaca. A síndrome do QT longo congênito possui padrão autossômico dominante (síndrome de Romano-Ward), bem como padrão autossômico recessivo raro (síndrome de Jervell e Lange-Nielsen). Desde 1957, quando Jervell e Lange-Nielsen relataram os primeiros casos de síndrome do QT longo congênito familiarcom surdez congênita, a compreensão dos mecanismos genéticos e eletrofisiológicos dessa afecção melhorousignificativamente os métodos diagnósticos e os tratamentos. No entanto, tornou-se evidente que a síndrome do QT longo congênito nem sempre pode ser explicada pela mutação de um único gene. Esta revisão teve por objetivo resumir as características da síndrome do QT longo congênito (principalmente LQT1, LQT2 e LQT3) e descrever brevemente os mais recentes avanços no diagnóstico clínico e no tratamento da afecção...

Congenital long QT syndrome is an inherited arrhythmia syndrome characterized by a prolonged QT interval on the 12-lead electrocardiogram, torsades de pointes and a higher chance of sudden cardiac death. Congenital long QT syndrome includes an autosomal dominant pattern (Romano-Ward syndrome) as well as a rare autosomal recessive pattern (Jervell and Lange-Nielsen syndrome). Since 1957 when Jervell and Lange Nielsen reported the first familial long QT syndrome with congenital deafness, the understanding of genetic and electrophysiological mechanisms of long QT syndrome has significantly improved diagnostic methods and treatments. However, is clear that long QT syndrome cannot always be explained by a single gene mutation. This review is aimed at summarizing the characteristics of congenital long QT syndrome (mainly LQT1, LQT2 e LQT3) and briefly describe the most recent advances in long QT syndrome clinical diagnostics and treatment...

Preditores de morte súbita cardíaca em jovens universitários / Predictors of sudden cardiac death in young university students

Introdução: A morte súbita cardíaca é definida como morte de ocorrência inesperada, que ocorre ematé uma hora do início dos sintomas. Este estudo teve como objetivo identificar fatores de risco para morte súbita cardíaca em população de jovens universitários. Método: Estudo observacional com aplicação do questionárioSudden Cardiac Death – Screening of risk factors a 142 jovens universitários de diferentes cursos na cidade de Goiânia (GO), na faixa etária entre 18 anos e 35 anos e realização de eletrocardiograma. Os laudos dos exames foram elaborados por um único médico cardiologista e enviados por e-mail. Os jovens que tiveram resultado alterado receberam o exame e foram orientados a procurar um especialista para melhor investigação. Foi realizada análise descritiva das respostas e alterações eletrocardiográficas. Resultados: Encontrou-se média de idade de22,4 anos e predomínio do sexo feminino. História de síncope foi referida por 39,4% dos jovens, dos quais 49,1%relataram 2 episódios a 5 episódios nos últimos 5 anos. Os contextos mais associados à síncope foram jejum eestresse (ambos com 19,64%), 39,4% afirmaram ter crises de taquiarritmia e 38% relataram precordialgia com frequência. História de doenças cardíacas familiares foi referida por 33,8% dos participantes, dos quais 54,1% relataram ocorrência de morte súbita em familiares com idade < 50 anos. Alterações eletrocardiográficas foram observadas em 27,4%, sendo as mais frequentes a repolarização precoce e o distúrbio de condução do ramo direito (ambos com 23%). Conclusão: Identificou-se a presença de fatores preditores de morte súbita cardíaca na população estudada. No entanto, não foi possível definir ou estratificar o risco do evento pelos meios propostos.

Background: Sudden cardiac death is defined as the unexpected occurrence of death occurring within one hour of symptom onset. This trial aimed to identify risk factors for sudden cardiac death in a population of university students. Method: Observational study including the application of the Sudden Cardiac Death - Screening of risk factors questionnaire to 142 university students between 18 to 35 years of age taking different courses in the city of Goiânia (GO) and an electrocardiogram. The reports were made by the same cardiologist and emailed. Those with abnormal test results were instructed to visit a specialist for further investigation. A descriptive analysis of there sponses and electrocardiographic changes were performed. Results: Mean age was 22.4 years with a prevalence of females. History of syncope was reported by 39.4% of the participants, of which 49.1% reported 2-5 episodes in the last five years. The events associated with syncope were fasting and stress (both with 19.64%), 39.4% reported having tachyarrhythmia and 38% reported frequent chest pain. Family history of heart disease was reported by 33.8% of the respondents, of whom 54.1% had sudden death before 50 years of age. Electrocardiographic changes were identified in 27.4% and the most frequent were early repolarization and right bundle branch conduction disturbance (both with 23%). Conclusion: Predictors of sudden cardiac death were identified in this population. However, it was not possible to define or stratify the risk of the event using the proposed methods.

Comorbid Conditions Do Not Differ in Children and Young Adults with Functional Disorders with or without Postural Tachycardia Syndrome.

OBJECTIVE: To determine if several multisystem comorbid conditions occur more frequently in subjects with tilt-table defined postural tachycardia syndrome (POTS) compared with those without. STUDY DESIGN: Retrospective chart review of 67 subjects aged 6-24 years, referred to a tertiary care neurogastroenterology and autonomic disorders clinic for a constellation of functional gastrointestinal, chronic pain, and autonomic complaints. All patients underwent formal autonomic testing, Beighton scores assessment for joint hypermobility (0-9), and fibromyalgia tender points (0-18) (43 subjects). RESULTS: Twenty-five subjects (37%) met tilt table criteria for POTS. The median age of 16 years (range, 12-24 years) in the POTS group differed from 15 years (range, 6-21 years) in the no-POTS group (P = .03). Comorbidities including chronic fatigue, sleep disturbances, dizziness, syncope, migraines, functional gastrointestinal disorders, chronic nausea, fibromyalgia, and joint hypermobility did not differ between groups. All subjects with fibromyalgia by tender point-examination had a Beighton score ≥ 4 (P = .002). CONCLUSIONS: Comorbid conditions are equally prevalent in children and young adults with and without tilt-table defined POTS, suggesting that POTS itself is not a cause of the other comorbidities. Instead, POTS likely reflects another comorbid condition in children with functional disorders. Dizziness and syncope, classically associated with POTS, are not predictive of a diagnosis of POTS by tilt table, a test that is still required for formal diagnosis. These results suggest a paradigm shift in the concept of POTS as the physiological basis of many functional symptoms.

Displasia arritmogênica do ventrículo direito: relato de caso e revisão dos critérios diagnósticos e atuais das indicações de cardiodesfibrilador implantável / Arrhythmogenic right ventricular dysplasia: case report and review of diagnostic criteria and current indications for implantable cardioverter defibrillator(ICD)

A displasia arritmogênica do ventrículo direito é uma forma de cardiomiopatia geneticamentedeterminada, que afeta primariamente o ventrículo direito. Caracteriza-se pela substituição dos miócitos portecido fibrogorduroso. É considerada uma doença cardíaca potencialmente letal e tem sido associada a arritmias,insuficiência cardíaca e morte súbita. Relata-se o caso clínico de um paciente jovem, em que a síncope foi aprimeira manifestação da doença.

Arrhythmogenic right ventricular dysplasia is a genetically determined form of cardiomyopathy thatprimarily affects the right ventricle. It is characterized by a gradual replacement of the myocytes by adipose andfibrous tissue. It is considered a potentially lethal heart disease and has been associated with arrhythmias, heartfailure, and sudden death. In this paper, we report a clinical case of a young man that presented syncope as firstmanifestation of the disease.

Bidirectional ventricular tachycardia?

A 65-year-old woman was admitted to the hospital because of a syncopal episode with documented transient complete atrioventricular block. A DDD pacemaker was implanted. Post implantation, the patient was diagnosed with bidirectional ventricular tachycardia. Analysis of the arrhythmia and differential diagnosis is performed.

Síncope: diagnóstico e tratamento / Syncope: diagnosis and treatment

Síncope é definida como perda transitória da consciência secundária à hipoperfusao cerebral difusa e caracteriza-se por início súbito, curta duração e recuperação espontânea. Apresenta alta incidência, especialmente na população idosa. Pode ser classificada de acordo com o mecanismo para a perda de consciência em: síncope reflexa neuromediada (por exemplo: síncope vasovagal; síncope do seio carotídeo, dentre outras); ortostática (depleção de volume; falência autonômica e hipotensão induzida por droga) e cardíaca (incluindo as arritmias cardíacas e doença arterial estrutural). Com o objetivo de avaliar o risco e identificar a causa subjacente à perda da consciência, todo paciente deverá ser submetido a um avaliação inicial que incluí história clínica detalhada, exame físico (com medida de PA em posição supina e ortostática) e eletrocardiograma de 12 derivações. Uma vez afastada a gravidade e identificada a causa subjacente, o principal objetivo é evitar a recorrência e aumentar a sobrevida.

Syncope is defined as a transient loss of the secondary consciousness to diffuse cerebral hypoperfusion and is characterized by having a sudden onset, short-lived and spontaneous recovery. It features high incidence especially in the elderly. It can be classified according to the mechanism for loss of consciousness: neuromediated reflex syncope (e.g. vasovagal syncope, carotid sinus syncope, among others); orthostatic (volume, depletion, autonomic failure induced by drugs) and cardiac (including cardiac arrhythmias and structural heart disease). In order to evaluate the risk and identify the underlying cause of loss of consciousness, every patient should undergo an initial assessment which includes detailed clinical history, physical examination (with PA in supine and orthostatic positions) and a 12 lead electrocardiogram. Once away the gravity and the underlying cause identified, the main objective will be to prevent the recurrence and increase survival.

Taquicardia ventricular de dificil controle em caso atípico de miocardite com acometimento de ventrículo direito / Hard-to-control ventricular tachycardia in an unusual case of myocarditis with involvement of the right ventricle

A apresentação clínica da miocardite varia desde casos assintomáticos a episódios de morte súbita. Nossintomáticos, a insuficiência cardíaca é a manifestação mais frequente. Arritmias, bloqueios cardíacos e clínica sugestiva de síndrome coronariana podem ocorrer. A diversidade da apresentação clínica, somada à dependência da biopsia miocárdica, torna o diagnóstico um desafio. Novos métodos, como a ressonância miocárdica (RM), estão adquirindo grande importância,auxiliando na investigação dos casos suspeitos. Os autores relatam um caso de taquiarritmia ventricular de difícil controle e com foco sugestivo em ponta de ventrículo direito. Miocardite foi a hipótese diagnóstica, confirmada pela RM.

Tratamento não-farmacológico da síncope neuromediada: [revisão] / Non-pharmacological treatment in neurally mediated syncope: [review]

A síncope neuromediada resulta de fenômeno transitório de hipofluxo cerebral. Normalmente está relacionada a uma condição benigna e passível de tratamento, farmacológico ou não. Diversas formas de tratamento não farmacológico têm sido propostas com a finalidade de obter maior controle sobre a frequência e a intensidade dos episódios de perda de consciência. O treinamento físico, o treinamento postural passivo e as contramanobras apresentam-se como alternativa terapêutica, visando promover maior adesão ao tratamento, por parte dos pacientes que relutam em fazer uso de medicação. O objetivo desta revisão é discorrer sobre as opções não-farmacológicas que envolvem exercício físico, para tratamento dessa entidade clínica, discutindo a fisiopatologia envolvida e as repercussões dos diversos tratamentos.

Efeito da sazonalidade tropical sobre os resultados do teste da mesa de inclinação / Seasonal fluctuation of tilt-test outcomes in the tropics

Fundamentos: Ambientes com temperatura elevada são considerados facilitadores para o desenvolvimento de síndrome neurocardiogênica. No entanto, existem poucas informações a respeito dos efeitos da sazonalidade nos resultados do teste da mesa de inclinação. Objetivo: Avaliar a influência da sazonalidade na taxa de positividade do teste de inclinação em uma cidade de clima tropival. Métodos: Foram analisados 501 testes consecutivos realizados em duas instituições na cidade do Rio de Janeiro, de junho de 2003 a agosto 2008, em uma população cuja idade variou de 6 anos a 90 anos, sendo 39 por cento homens. A resposta ao teste foi considerada positiva de acordo com os critérios do VASIS. O resultado do exame foi comparado no período de acordo com a taxa de positividade média mensal e sazonal. Após ter sido assumida a normalidade das distribuições e verificada a homocedasticidade através do teste de Bartlett, os achados foram comparados, utilizando-se os testes one-way ANOVA, para medidas não repetidas. Os resultados...

Background: High-temperature environments are rated as triggers for vaso-vagal orthostatic intolerance (VOI). However, there is little information on seasonal fluctuations of head-up tilt table test (HUT) outcomes.Objective: To evaluate seasonal fluctuation in HUT outcomes in a tropical city. Methods: We assessed 501 consecutive HUTs conducted at two institutions in the city of Rio de Janeiro, from June 2003 to August 2008 (6 y.o to 90.y.o, 39% males). The test response was defined as positive by the VASIS criteria. The VOI positive (+) outcome rate was compared during the period with the average monthly andseasonal rates. Having tested normality assumption and homocedasticity through the Bartlett test, the findingswere compared through one-way ANOVA tests for nonrepeatedmeasurements. The outcomes are presented as mean±DP. Results: The VOI was positive in 30.3%. Normality(Std Skewness: 1.5±0.4; Std Kurtosis: -0.4±0.8) and homocedasticity assumptions were accepted in all groups. No significant differences were observed in the monthly or seasonal average rates of VOI+ outcome in all comparisons (respectively, p=NS and p=NS). Conclusions: In the city of Rio de Janeiro, positive outcomes for HUT present uniform distribution throughout the year for months and seasons.