Exercise-Induced Intraventricular Obstruction in a Child with Near Syncope and Chest Pain During Exercise

We report the case of a 10-year-old girl with two episodes of light-headedness and chest pain during exercise. She had an unremarkable clinical record, physical examination, ECG, and echocardiogram. Noninvasive ischemia tests were positive, but coronary angiography was normal. Exercise stress echocardiogram revealed an exercise-induced intra-left-ventricular obstruction with a peak gradient of 78 mmHg and replicated her symptoms. After starting beta-blocker therapy her clinical status improved and no residual obstruction was detected. The authors review this unsuspected clinical condition, seldom reported in the adult population and, to our knowledge, never before in a child.

Syncope and Intermittent Brugada ECG Pattern

A síndrome de Brugada é uma síndrome rara, com uma prevalência aproximada na Europa de 1-5/10 000 habitantes, mas cuja apresentação clínica inicial pode ser morte súbita. Embora com um padrão eletrocardiográfico típico, este é por vezes intermitente. Os autores apresentam o caso clínico de um doente de sexo masculino, de 32 anos, sem fatores de risco pessoais conhecidos e história familiar de morte súbita, que recorre ao Serviço de Urgência por síncope sem pródromos. O primeiro eletrocardiograma (ECG) em ritmo sinusal documenta a presença de uma elevação isolada e inespecífica do segmento ST em V2. Da restante investigação diagnóstica realizada, salienta-se a repetição do ECG, que revelou a presença de padrão de Brugada tipo 1. Este mesmo padrão é exacerbado posteriormente numa situação de infeção respiratória. O doente foi submetido a estudo eletrofisiológico, seguido de implantação de cardiodesfibrilhador (CDI), tendo tido um episódio de fibrilhação ventricular convertido com choque via CDI 2 meses após a implantação.

Catecholaminergic polymorphic ventricular tachycardia. An important diagnosis in children with syncope and normal heart

Syncope in children is primarily related to vagal hyperreactivity, but ventricular tachycardia (VT) way rarely be seen. Catecholaminergic polymorphic VT is a rare entity that can occur in children without heart disease and with a normal QT interval, which may cause syncope and sudden cardiac death. In this report, we describe the clinical features, treatment, and clinical follow-up of three children with syncope associated with physical effort or emotion and cathecolaminergic polymorphic VT. Symptoms were controlled with beta-blockers, but one patient died suddenly in the fourth year of follow-up. Despite the rare occurrence, catecholaminergic polymorphic VT is an important cause of syncope and sudden death in children with no identified heart disease and normal QT interval.

The impact of syncope during clinical presentation of sustained ventricular tachycardia on total and cardiac mortality in patients with chronic Chagasic heart disease

OBJECTIVE: To assess the impact of syncope during sustained ventricular tachycardia on total and cardiac mortality in patients with chronic chagasic heart disease. METHODS: We assessed 78 patients with sustained ventricular tachycardia and chronic Chagas' heart disease. The mean age was 53±10 years, 45 were males, and the mean ejection fraction was 49.6±13%. The patients were divided into 2 groups according to the presence (GI=45) or absence (GII=33) of syncope during sustained ventricular tachycardia. RESULTS: After a mean follow-up of 49 months, total mortality was 35% (28 deaths), 22 deaths having a cardiac cause (78.6%). No difference was observed in total (33.3% x 39.4%) and cardiac (26.7% x 30.3%) mortality, or in nonfatal sustained ventricular tachycardia between GI and GII patients (57.6% x 54.4%, respectively). However, the presence of syncope during recurrences was significantly greater in those patients who had had the symptom from the beginning (65.4% x 18.1%, p<0.01). CONCLUSION: Syncope during the presentation of sustained ventricular tachycardia is not associated with an increase in total or cardiac mortality in patients with chronic Chagas' heart disease. However, syncope during the recurrence ventricular tachycardia is greater in patients experiencing syncope in the first episode, of sustained ventricular tachycardia.

Diagnostic value of the tilt-table test for the assessment of syncope in children and adolescents

OBJECTIVE - To assess the diagnostic value, the characteristics, and feasibility of tilt-table testing in children and adolescents. METHODS - From August 1991 to June 1997, we retrospectively assessed 94 patients under the age of 18 years who had a history of recurring syncope and presyncope of unknown origin and who were referred for tilt-table testing. These patients were divided into 2 groups: group I (children) - 36 patients with ages ranging from 3 to 12 (mean of 9.19±2.31) years; group II (adolescents) - 58 patients with ages ranging from 13 to 18 (mean of 16.05±1.40) years. We compared the positivity rate, the type of hemodynamic response, and the time period required for the test to become positive in the 2 groups. RESULTS - The positivity rates were 41.6 % and 50% for groups I and II, respectively. The pattern of positive hemodynamic response that predominated in both groups was the mixed response. The mean time period required for the test to become positive was shorter in group I (11.0±7.23 min) than in group II (18.44±7.83 min). No patient experienced technical difficulty or complications. CONCLUSION - No difference was observed in regard to feasibility, positivity rate, and pattern of positive response for the tilt-table test in children and adolescents. Pediatric patients had earlier positive responses.

Raising the Level of Moral Thinking among Managers Will Not Help. Kohlberg's Conventionality and Systemic Ethical Flaws in Business

Business ethicists often assume that unethical behavior arises when individuals deviate from the norms and responsibilities that are institutionalized to frame economic activities. People's greed motivates them to violate the rules of the game. In Kohlberg's terms, it is assumed that such actors make decisions in a preconventional way and act opportunistically. In this article, we propose an alternative interpretation of deviant behavior, arguing that such behavior does not result from a lack of conventional moral guidance but rather from the fact that characteristics attributed to preconventional morality by Kohlberg - the purely incentive and punishment driven opportunistic morality - have become the conventionalized morality. The prevailing norms that economic actors have internalized as their yardstick are those of the preconventional Homo economicus. Not the deviation from, but the compliance with the rules of the game explains many forms of harmful and illegal decisions made in corporations.

Patient with syncope and LQTS carrying a mutation in the PAS domain of the hERG1 channel.

We report the case of a woman with syncope and persistently prolonged QTc interval. Screening of congenital long QT syndrome (LQTS) genes revealed that she was a heterozygous carrier of a novel KCNH2 mutation, c.G238C. Electrophysiological and biochemical characterizations unveiled the pathogenicity of this new mutation, displaying a 2-fold reduction in protein expression and current density due to a maturation/trafficking-deficient mechanism. The patient's phenotype can be fully explained by this observation. This study illustrates the importance of performing genetic analyses and mutation characterization when there is a suspicion of congenital LQTS. Identifying mutations in the PAS domain or other domains of the hERG1 channel and understanding their effect may provide more focused and mutation-specific risk assessment in this population.

Training, information and awareness raising

Projecto DESIRE Cabo Verde

Pacemaker therapy in patients with neurally mediated syncope and documented asystole: Third International Study on Syncope of Uncertain Etiology (ISSUE-3): a randomized trial.

BACKGROUND: The efficacy of cardiac pacing for prevention of syncopal recurrences in patients with neurally mediated syncope is controversial. We wanted to determine whether pacing therapy reduces syncopal recurrences in patients with severe asystolic neurally mediated syncope. METHODS AND RESULTS: Double-blind, randomized placebo-controlled study conducted in 29 centers in the Third International Study on Syncope of Uncertain Etiology (ISSUE-3) trial. Patients were ≥40 years, had experienced ≥3 syncopal episodes in the previous 2 years. Initially, 511 patients, received an implantable loop recorder; 89 of these had documentation of syncope with ≥3 s asystole or ≥6 s asystole without syncope within 12 ± 10 months and met criteria for pacemaker implantation; 77 of 89 patients were randomly assigned to dual-chamber pacing with rate drop response or to sensing only. The data were analyzed on intention-to-treat principle. There was syncope recurrence during follow-up in 27 patients, 19 of whom had been assigned to pacemaker OFF and 8 to pacemaker ON. The 2-year estimated syncope recurrence rate was 57% (95% CI, 40-74) with pacemaker OFF and 25% (95% CI, 13-45) with pacemaker ON (log rank: P=0.039 at the threshold of statistical significance of 0.04). The risk of recurrence was reduced by 57% (95% CI, 4-81). Five patients had procedural complications: lead dislodgment in 4 requiring correction and subclavian vein thrombosis in 1 patient. CONCLUSIONS: Dual-chamber permanent pacing is effective in reducing recurrence of syncope in patients ≥40 years with severe asystolic neurally mediated syncope. The observed 32% absolute and 57% relative reduction in syncope recurrence support this invasive treatment for the relatively benign neurally mediated syncope. CLINICAL TRIAL REGISTRATION: URL: http://www.clinicaltrials.gov. Unique identifier: NCT00359203.

Ictal bradycardia and asystole: an uncommon cause of syncope.

We report on two patients with recurrent syncope secondary to ictal bradyarrhythmias, triggered by partial epileptic seizures with atypical, stereotyped auras. Ictal bradyarrhythmias are potentially lethal, and likely originate from the involvement of limbic autonomic regions. The appropriate treatment is double-headed, including an antiepileptic drug and the implantation of a pacemaker.

Risk and global change : developing scientific methods for advocacy and awareness raising

Les catastrophes sont souvent perçues comme des événements rapides et aléatoires. Si les déclencheurs peuvent être soudains, les catastrophes, elles, sont le résultat d'une accumulation des conséquences d'actions et de décisions inappropriées ainsi que du changement global. Pour modifier cette perception du risque, des outils de sensibilisation sont nécessaires. Des méthodes quantitatives ont été développées et ont permis d'identifier la distribution et les facteurs sous- jacents du risque.¦Le risque de catastrophes résulte de l'intersection entre aléas, exposition et vulnérabilité. La fréquence et l'intensité des aléas peuvent être influencées par le changement climatique ou le déclin des écosystèmes, la croissance démographique augmente l'exposition, alors que l'évolution du niveau de développement affecte la vulnérabilité. Chacune de ses composantes pouvant changer, le risque est dynamique et doit être réévalué périodiquement par les gouvernements, les assurances ou les agences de développement. Au niveau global, ces analyses sont souvent effectuées à l'aide de base de données sur les pertes enregistrées. Nos résultats montrent que celles-ci sont susceptibles d'être biaisées notamment par l'amélioration de l'accès à l'information. Elles ne sont pas exhaustives et ne donnent pas d'information sur l'exposition, l'intensité ou la vulnérabilité. Une nouvelle approche, indépendante des pertes reportées, est donc nécessaire.¦Les recherches présentées ici ont été mandatées par les Nations Unies et par des agences oeuvrant dans le développement et l'environnement (PNUD, l'UNISDR, la GTZ, le PNUE ou l'UICN). Ces organismes avaient besoin d'une évaluation quantitative sur les facteurs sous-jacents du risque, afin de sensibiliser les décideurs et pour la priorisation des projets de réduction des risques de désastres.¦La méthode est basée sur les systèmes d'information géographique, la télédétection, les bases de données et l'analyse statistique. Une importante quantité de données (1,7 Tb) et plusieurs milliers d'heures de calculs ont été nécessaires. Un modèle de risque global a été élaboré pour révéler la distribution des aléas, de l'exposition et des risques, ainsi que pour l'identification des facteurs de risque sous- jacent de plusieurs aléas (inondations, cyclones tropicaux, séismes et glissements de terrain). Deux indexes de risque multiples ont été générés pour comparer les pays. Les résultats incluent une évaluation du rôle de l'intensité de l'aléa, de l'exposition, de la pauvreté, de la gouvernance dans la configuration et les tendances du risque. Il apparaît que les facteurs de vulnérabilité changent en fonction du type d'aléa, et contrairement à l'exposition, leur poids décroît quand l'intensité augmente.¦Au niveau local, la méthode a été testée pour mettre en évidence l'influence du changement climatique et du déclin des écosystèmes sur l'aléa. Dans le nord du Pakistan, la déforestation induit une augmentation de la susceptibilité des glissements de terrain. Les recherches menées au Pérou (à base d'imagerie satellitaire et de collecte de données au sol) révèlent un retrait glaciaire rapide et donnent une évaluation du volume de glace restante ainsi que des scénarios sur l'évolution possible.¦Ces résultats ont été présentés à des publics différents, notamment en face de 160 gouvernements. Les résultats et les données générées sont accessibles en ligne (http://preview.grid.unep.ch). La méthode est flexible et facilement transposable à des échelles et problématiques différentes, offrant de bonnes perspectives pour l'adaptation à d'autres domaines de recherche.¦La caractérisation du risque au niveau global et l'identification du rôle des écosystèmes dans le risque de catastrophe est en plein développement. Ces recherches ont révélés de nombreux défis, certains ont été résolus, d'autres sont restés des limitations. Cependant, il apparaît clairement que le niveau de développement configure line grande partie des risques de catastrophes. La dynamique du risque est gouvernée principalement par le changement global.¦Disasters are often perceived as fast and random events. If the triggers may be sudden, disasters are the result of an accumulation of actions, consequences from inappropriate decisions and from global change. To modify this perception of risk, advocacy tools are needed. Quantitative methods have been developed to identify the distribution and the underlying factors of risk.¦Disaster risk is resulting from the intersection of hazards, exposure and vulnerability. The frequency and intensity of hazards can be influenced by climate change or by the decline of ecosystems. Population growth increases the exposure, while changes in the level of development affect the vulnerability. Given that each of its components may change, the risk is dynamic and should be reviewed periodically by governments, insurance companies or development agencies. At the global level, these analyses are often performed using databases on reported losses. Our results show that these are likely to be biased in particular by improvements in access to information. International losses databases are not exhaustive and do not give information on exposure, the intensity or vulnerability. A new approach, independent of reported losses, is necessary.¦The researches presented here have been mandated by the United Nations and agencies working in the development and the environment (UNDP, UNISDR, GTZ, UNEP and IUCN). These organizations needed a quantitative assessment of the underlying factors of risk, to raise awareness amongst policymakers and to prioritize disaster risk reduction projects.¦The method is based on geographic information systems, remote sensing, databases and statistical analysis. It required a large amount of data (1.7 Tb of data on both the physical environment and socio-economic parameters) and several thousand hours of processing were necessary. A comprehensive risk model was developed to reveal the distribution of hazards, exposure and risk, and to identify underlying risk factors. These were performed for several hazards (e.g. floods, tropical cyclones, earthquakes and landslides). Two different multiple risk indexes were generated to compare countries. The results include an evaluation of the role of the intensity of the hazard, exposure, poverty, governance in the pattern and trends of risk. It appears that the vulnerability factors change depending on the type of hazard, and contrary to the exposure, their weight decreases as the intensity increases.¦Locally, the method was tested to highlight the influence of climate change and the ecosystems decline on the hazard. In northern Pakistan, deforestation exacerbates the susceptibility of landslides. Researches in Peru (based on satellite imagery and ground data collection) revealed a rapid glacier retreat and give an assessment of the remaining ice volume as well as scenarios of possible evolution.¦These results were presented to different audiences, including in front of 160 governments. The results and data generated are made available online through an open source SDI (http://preview.grid.unep.ch). The method is flexible and easily transferable to different scales and issues, with good prospects for adaptation to other research areas. The risk characterization at a global level and identifying the role of ecosystems in disaster risk is booming. These researches have revealed many challenges, some were resolved, while others remained limitations. However, it is clear that the level of development, and more over, unsustainable development, configures a large part of disaster risk and that the dynamics of risk is primarily governed by global change.

Loanword Phonology, Lexical Exceptions, Morphologically Driven Underapplication, and the Nature of Positionally Biased Constraints

In this paper we provide a formal account for underapplication of vowel reduction to schwa in Majorcan Catalan loanwords and learned words. On the basis of the comparison of these data with those concerning productive derivation and verbal inflection, which show analogous patterns, in this paper we also explore the existing and not yet acknowledged correlation between those processes that exhibit a particular behaviour in the loanword phonology with respect to the native phonology of the language, those processes that show lexical exceptions and those processes that underapply due to morphological reasons. In light of the analysis of the very same data and taking into account the aforementioned correlation, we show how there might exist a natural diachronic relation between two kinds of Optimality Theory constraints which are commonly used but, in principle, mutually exclusive: positional faithfulness and contextual markedness constraints. Overall, phonological productivity is proven to be crucial in three respects: first, as a context of the grammar, given that «underapplication» is systematically found in what we call the productive phonology of the dialect (including loanwords, learned words, productive derivation and verbal inflection); second, as a trigger or blocker of processes, in that the productivity or the lack of productivity of a specific process or constraint in the language is what explains whether it is challenged or not in any of the depicted situations, and, third, as a guiding principle which can explain the transition from the historical to the synchronic phonology of a linguistic variety.