The impact of trisomy 21 on treatment modalities and outcome in adults with congenital heart disease in Switzerland.

Trisomy 21 (T21) is associated in 40-45% of cases with heart defects, most commonly shunt lesions. These defects, if not repaired, can lead to irreversible shunt-induced pulmonary hypertension (i.e. Eisenmenger syndrome [ES]). In ES patients, intracardiac repair is no longer possible, but selective pulmonary vasodilators may increase exercise capacity and improve prognosis. This study aimed to estimate the prevalence of cardiac defects and ES in adult T21 patients and to assess the impact of T21 on treatment modalities and outcome in ES patients. A questionnaire was sent to 6906 Swiss physicians inviting them to indicate the number of adults with T21 under their care (survey report). We also analyzed all adults with ES (with and without T21) included in the Swiss Adult Congenital HEart disease Registry (SACHER) and studied the impact of T21 on the use of selective pulmonary vasodilators and survival. In the survey, 348 physicians cared for 695 adult T21 patients. Overall, 24% of T21 survey patients were known to have a cardiac defect, one in four with a defect had developed ES and 13% of those with ES were on specific pulmonary vasodilators. In SACHER, ES was present in 2% of adults with congenital heart disease and selective pulmonary vasodilators were used in 68% of ES patients with T21. In SACHER, survival during follow-up was worse with higher nt-proBNP levels (hazard ratio [HR] = 1.15 per 1000 units, 95% confidence interval [CI] = 1.02-1.29) and lower left ventricular ejection fraction (HR = 1.07 per percent decrease, 95% CI = 1.01-1.13). Age at inclusion and T21 did not affect survival. The prevalence of cardiac defects in adults with T21 in Switzerland is half the prevalence in children. T21 is over-represented among adults with ES. Raised awareness of the therapeutic options for T21 patients with ES is warranted

Swiss Adult Congenital HEart disease Registry (SACHER) - rationale, design and first results.

In 2013, a prospective registry for adults with congenital heart disease (CHD) was established in Switzerland, providing detailed data on disease characteristics and outcomes: Swiss Adult Congenital HEart disease Registry (SACHER). Its aim is to improve the knowledge base of outcomes in adults with CHD. The registry design and baseline patient characteristics are reported. All patients with structural congenital heart defects or hereditary aortopathies, followed-up at dedicated adult CHD clinics, are asked to participate in SACHER. Data of participants are pseudonymised and collected in an electronic, web-based, database (secuTrial®). Collected data include detailed diagnosis, type of repair procedures, previous complications and adverse outcomes during follow-up. From May 2014 to December 2016, 2836 patients (54% male, mean age 34 ± 14 years), with a wide variety of congenital heart lesions, have been enrolled into SACHER. Most prevalent were valve lesions (25%), followed by shunt lesions (22%), cyanotic and other complex congenital heart disease (16%), diseases affecting the right heart, i.e., tetralogy of Fallot or Ebstein anomaly (15%), and diseases of the left ventricular outflow tract (13%); 337 patients (12%) had concomitant congenital syndromes. The majority had undergone previous repair procedures (71%), 47% of those had one or more reinterventions. SACHER collects multicentre data on adults with CHD. Its structure enables prospective data analysis to assess detailed, lesion-specific outcomes with the aim to finally improve long-term outcomes

Study of ^194 Ir via thermal neutron capture and (d,p) reactions

Levels of ^194 Ir were studied using thermal neutron capture reaction. A pair spectrometer was used to measure the high-energy gamma-ray spectrum from thermal-neutron capture in enriched ^193 Ir target over the energy range 4640 - 6100 keV. The low-energy gamma-radiation from the reaction was studied with crystal diffraction spectrometers, and conversion electrons were observed with magnetic spectrometers. The high-sensitivity measurements at the Grenoble reactor, evaluated for transition energies up to 500 keV, are compared with lower-sensitivity measurements at the Wuerenlingen and Salaspils reactors. The comparison helped to obtain reliable isotopic identification for a number of ^194 Ir lines. The multipolarity admixtures for 29 gamma-transitions were determined on the basis of conversion lines from different electron subshells. Prompt and delayed gamma-gamma coincidences were measured using semiconductor and scintillation detectors. The ^193 Ir(d,p) high-resolution spectra, observed with a magnetic spectrometer, are given. All these data contributed to establishing a detailed level scheme of ^194 Ir. Additional data and the interpretation of the results in terms of current models will be presented in a forthcoming paper

Proučavanje 194Ir uhvatom termičkih neutrona I (d, p) reakcijom

Levels of 194Ir were studied using thermal neutron capture reaction. A pair spectrometer was used to measure the high-energy γ-ray spectrum from thermal-neutron capture in enriched 193Ir target over the energy range 4640 - 6100 keV. The low-energy γ-radiation from the reaction was studied with crystal diffraction spectrometers, and conversion electrons were observed with magnetic spectrometers. The high-sensitivity measurements at the Grenoble reactor, evaluated for transition energies up to 500 keV, are compared with lower-sensitivity measurements at the Wuerenlingen and Salaspils reactors. The comparison helped to obtain reliable isotopic identification for a number of 194Ir lines. The multipolarity admixtures for 29 γ-transitions were determined on the basis of conversion lines from different electron subshells. Prompt and delayed γ-γ coincidences were measured using semiconductor and scintillation detectors. The 193Ir(d,p) high-resolution spectra, observed with a magnetic spectrometer, are given. All these data contributed to establishing a detailed level scheme of 194Ir. Additional data and the interpretation of the results in terms of current models will be presented in a forthcoming paper.Proučavala su se stanja u 194Ir reakcijama 193Ir(n, γ) i 193Ir(d, p). Mjerenja uhvata termičkih neutrona načinjena su uz reaktore u Grenoblu, Wuerenlingenu i Salapsisu. Za mjerenja γ-zračenja visoke energije upotrebljavao se spektrometar parova, a za niske energije difraktometar. Konverzijske elektrone se mjerilo magnetskim spektrometrom. Mjerenja reakcije (d, p) visokog razlučivanja izvedena su magnetskim spektrometrom. Usporedbe tih mjerenja omogućile su pouzdano izotopno prepoznavanje prijelaza u 194 Ir, a spektri konverzijskih elektrona i određivanje multipolnosti prijelaza. Dobiveni su podaci osnova sheme raspada 194Ir

Proučavanje 194Ir uhvatom termičkih neutrona I (d, p) reakcijom

Levels of 194Ir were studied using thermal neutron capture reaction. A pair spectrometer was used to measure the high-energy γ-ray spectrum from thermal-neutron capture in enriched 193Ir target over the energy range 4640 - 6100 keV. The low-energy γ-radiation from the reaction was studied with crystal diffraction spectrometers, and conversion electrons were observed with magnetic spectrometers. The high-sensitivity measurements at the Grenoble reactor, evaluated for transition energies up to 500 keV, are compared with lower-sensitivity measurements at the Wuerenlingen and Salaspils reactors. The comparison helped to obtain reliable isotopic identification for a number of 194Ir lines. The multipolarity admixtures for 29 γ-transitions were determined on the basis of conversion lines from different electron subshells. Prompt and delayed γ-γ coincidences were measured using semiconductor and scintillation detectors. The 193Ir(d,p) high-resolution spectra, observed with a magnetic spectrometer, are given. All these data contributed to establishing a detailed level scheme of 194Ir. Additional data and the interpretation of the results in terms of current models will be presented in a forthcoming paper.Proučavala su se stanja u 194Ir reakcijama 193Ir(n, γ) i 193Ir(d, p). Mjerenja uhvata termičkih neutrona načinjena su uz reaktore u Grenoblu, Wuerenlingenu i Salapsisu. Za mjerenja γ-zračenja visoke energije upotrebljavao se spektrometar parova, a za niske energije difraktometar. Konverzijske elektrone se mjerilo magnetskim spektrometrom. Mjerenja reakcije (d, p) visokog razlučivanja izvedena su magnetskim spektrometrom. Usporedbe tih mjerenja omogućile su pouzdano izotopno prepoznavanje prijelaza u 194 Ir, a spektri konverzijskih elektrona i određivanje multipolnosti prijelaza. Dobiveni su podaci osnova sheme raspada 194Ir

Study of ^194 Ir via thermal neutron capture and (d,p) reactions

Levels of ^194 Ir were studied using thermal neutron capture reaction. A pair spectrometer was used to measure the high-energy gamma-ray spectrum from thermal-neutron capture in enriched ^193 Ir target over the energy range 4640 - 6100 keV. The low-energy gamma-radiation from the reaction was studied with crystal diffraction spectrometers, and conversion electrons were observed with magnetic spectrometers. The high-sensitivity measurements at the Grenoble reactor, evaluated for transition energies up to 500 keV, are compared with lower-sensitivity measurements at the Wuerenlingen and Salaspils reactors. The comparison helped to obtain reliable isotopic identification for a number of ^194 Ir lines. The multipolarity admixtures for 29 gamma-transitions were determined on the basis of conversion lines from different electron subshells. Prompt and delayed gamma-gamma coincidences were measured using semiconductor and scintillation detectors. The ^193 Ir(d,p) high-resolution spectra, observed with a magnetic spectrometer, are given. All these data contributed to establishing a detailed level scheme of ^194 Ir. Additional data and the interpretation of the results in terms of current models will be presented in a forthcoming paper

International comparison of current transformer calibrations

NRC publication: Ye

The nature of magnetic fields and the flux quantum

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