The Portuguese Registry of Hypertrophic Cardiomyopathy: Overall results

INTRODUCTION: We report the results of the Portuguese Registry of Hypertrophic Cardiomyopathy, an initiative that reflects the current spectrum of cardiology centers throughout the territory of Portugal. METHODS: A direct invitation to participate was sent to cardiology departments. Baseline and outcome data were collected. RESULTS: A total of 29 centers participated and 1042 patients were recruited. Four centers recruited 49% of the patients, of whom 59% were male, and mean age at diagnosis was 53±16 years. Hypertrophic cardiomyopathy (HCM) was identified as familial in 33%. The major reason for diagnosis was symptoms (53%). HCM was obstructive in 35% of cases and genetic testing was performed in 51%. Invasive septal reduction therapy was offered to 8% (23% of obstructive patients). Most patients (84%) had an estimated five-year risk of sudden death of <6%. Thirteen percent received an implantable cardioverter-defibrillator. After a median follow-up of 3.3 years (interquartile range [P25-P75] 1.3-6.5 years), 31% were asymptomatic. All-cause mortality was 1.19%/year and cardiovascular mortality 0.65%/year. The incidence of heart failure-related death was 0.25%/year, of sudden cardiac death 0.22%/year and of stroke-related death 0.04%/year. Heart failure-related death plus heart transplantation occurred in 0.27%/year and sudden cardiac death plus equivalents occurred in 0.53%/year. CONCLUSIONS: Contemporary HCM in Portugal is characterized by relatively advanced age at diagnosis, and a high proportion of invasive treatment of obstructive forms. Long-term mortality is low; heart failure is the most common cause of death followed by sudden cardiac death. However, the burden of morbidity remains considerable, emphasizing the need for disease-specific treatments that impact the natural history of the disease.info:eu-repo/semantics/publishedVersio

Impact of nitrogen seeding on carbon erosion in the JET divertor

Nitrogen has been introduced in H-mode plasmas in JET in order to study its radiation cooling capability and impact on the erosion of divertor plasma-facing components made of carbon-fiber composites (CFC). Experiments in the ionizing plasma regime with low nitrogen injection show a reduction of the total carbon erosion in the divertor measured with the aid of optical spectroscopy on C(+). Though chemical sputtering by nitrogen takes place, identified by the appearance of CN B-X band emission, the additional carbon source is overcompensated by a reduction of regular sputtering by deuterium bombardment. Moderate plasma cooling associated with reduction of the sputtering yield and dilution of the CFC surface by nitrogen can be attributed to the favorable reduction of the carbon source

Strike-point splitting induced by external magnetic perturbations: Observations on JET and MAST and associated modelling

International audienceHeat flux deposition profiles, calculated from infrared imaging, are presented for JET and MAST cases in the presence of external magnetic perturbations. In L-mode, a clear splitting of the strike-point is observed and well reproduced by modelling based on field line tracing with no plasma magnetic response included (vacuum field approximation). In H-mode, in contrast, the splitting is not clearly observed in spite of being expected from vacuum field modelling. As illustrated by modelling results, screening effects could explain the absence of a clear splitting in H-mode. (C) 2010 Elsevier B.V. All rights reserved.Assoc EURATOM IPP CR, Inst Plasma Phys AS CR Vvi, Prague, Czech Republic.Max Planck Inst Plasma Phys, D-85748 Garching, Germany.Culham Sci Ctr, EURATOM CCFE Fus Assoc, Abingdon OX14 3DB, Oxon, England.Assoc Euratom Enea Fus, Consorzio RFX, I-35127 Padua, Italy.Asociac EURATOM CIEMAT, Lab Nacl Fus, Madrid, Spain.FOM Inst Plasma Phys Rijnhuizen, NL-3439 MN Nieuwegein, Netherlands.Ecole Polytech, CNRS, UMR 7648, LPTP, F-91128 Palaiseau, France.Forschungszentrum Julich, Assoc EURATOM FZJ, D-52425 Julich, Germany.TEC, EURATOM Belgian State Assoc, LPP ERM KMS, Brussels, Belgium