Clinical characteristics of wild-type transthyretin cardiac amyloidosis: disproving myths.

Wild-type transthyretin amyloidosis (ATTRwt) is mostly considered a disease predominantly of elderly male, characterized by concentric LV hypertrophy, preserved LVEF, and low QRS voltages. We sought to describe the characteristics of a large cohort of ATTRwt patients to better define the disease. Clinical findings of consecutive ATTRwt patients diagnosed at 2 centres were reviewed. ATTRwt was diagnosed histologically or non-invasively (LV hypertrophy ≥12 mm, intense cardiac uptake at 99mTc-DPD scintigraphy and AL exclusion). Mutations in TTR were excluded in all cases. The study cohort comprised 108 patients (78.6 ± 8 years); 67 (62%) diagnosed invasively and 41 (38%) non-invasively. Twenty patients (19%) were females. An asymmetric hypertrophy pattern was observed in 25 (23%) patients. Mean LVEF was 52 ± 14%, with 39 patients (37%) showing a LVEF < 50%. Atrial fibrillation (56%) and a pseudo-infarct pattern (63%) were the commonest ECG findings. Only 22 patients fulfilled QRS low-voltage criteria while 10 showed LV hypertrophy on ECG. Although heart failure was the most frequent profile leading to diagnosis (68%), 7% of individuals presented with atrioventricular block and 11% were diagnosed incidentally. Almost one third (35; 32%) were previously misdiagnosed. The clinical spectrum of ATTRwt is heterogeneous and differs from the classic phenotype: women are affected in a significant proportion; asymmetric LV hypertrophy and impaired LVEF are not rare and only a minority have low QRS voltages. Clinicians should be aware of the broad clinical spectrum of ATTRwt to correctly identify an entity for which a number of disease-modifying treatments are under investigation.This work was supported in part by the Spanish Society of Cardiology [Grant 2016 to E.G-L.] and by the Instituto de Salud Carlos III (ISCIII) [grants RD012/0042/0066 and CB16/11/00432] and by the Spanish Ministry of Economy and Competitiveness [grant SAF2015-71863-REDT]. Grants are supported by the Plan Estatal de IþDþI 2013-2016–European Regional Development Fund (FEDER) “A way of making Europe”.S

Reducing the environmental impact of surgery on a global scale: systematic review and co-prioritization with healthcare workers in 132 countries

Abstract Background Healthcare cannot achieve net-zero carbon without addressing operating theatres. The aim of this study was to prioritize feasible interventions to reduce the environmental impact of operating theatres. Methods This study adopted a four-phase Delphi consensus co-prioritization methodology. In phase 1, a systematic review of published interventions and global consultation of perioperative healthcare professionals were used to longlist interventions. In phase 2, iterative thematic analysis consolidated comparable interventions into a shortlist. In phase 3, the shortlist was co-prioritized based on patient and clinician views on acceptability, feasibility, and safety. In phase 4, ranked lists of interventions were presented by their relevance to high-income countries and low–middle-income countries. Results In phase 1, 43 interventions were identified, which had low uptake in practice according to 3042 professionals globally. In phase 2, a shortlist of 15 intervention domains was generated. In phase 3, interventions were deemed acceptable for more than 90 per cent of patients except for reducing general anaesthesia (84 per cent) and re-sterilization of ‘single-use’ consumables (86 per cent). In phase 4, the top three shortlisted interventions for high-income countries were: introducing recycling; reducing use of anaesthetic gases; and appropriate clinical waste processing. In phase 4, the top three shortlisted interventions for low–middle-income countries were: introducing reusable surgical devices; reducing use of consumables; and reducing the use of general anaesthesia. Conclusion This is a step toward environmentally sustainable operating environments with actionable interventions applicable to both high– and low–middle–income countries

"Nuclear medicine" session

En la sesión dedicada a la Medicina Nuclear (MN) se identificaron los cinco aspectos que se consideran más problemáticos en la protección radiológica en MN. Estos hacen referencia a: 1. Garantizar que la dosis administrada al paciente sea correcta; 2. Evitar en el trabajador la contaminación e irradiación de las extremidades superiores, cristalino y resto del cuerpo; 3. Asegurar la optimización de dosis en diagnóstico y tratamiento; 4. Promover la justificación de los exámenes en MN; 5. Prevenir incidentes y accidentes. Las soluciones aportadas a estos problemas fueron: 1. Implementar sistemas integrales de calidad y protocolos de control de calidad así como entrenar y capacitar adecuadamente a los trabajadores; 2. Mejorar la capacitación y formación del trabajador, uso sistemático de medios de protección y de protocolos de trabajo y adecuación de los procedimientos de trabajo; 3. Utilización de dosis estandarizadas en diagnóstico y planificar los tratamientos mediante dosimetría interna paciente específica; 4. Capacitación de médicos prescriptores y médicos nucleares y utilización de guías de adecuada solicitud de exámenes de MN; 5. Incorporación efectiva de sistemas de notificación de incidentes para su posterior análisis y aprendizaje mediante la utilización de técnicas de análisis de sucesos. Los indicadores propuestos para la adecuada valoración de los avances obtenidos en cada uno de los aspectos valorados fueron: 1. Número de centros con un Programa de garantía de calidad implementado y grado de cumplimiento del mismo en cada centro 2. Análisis de tendencias de los datos dosimétricos reportados 3. Cantidad de exámenes con protocolos de optimización de dosis o de estimación de la actividad paciente específico 4. Número de programas de estudio de pregrado de medicina que incluyen temas relacionados con Protección Radiológica y número de protocolos clínicos escritos con las indicaciones de cada estudio y porcentaje de exámenes que cumplen con estas guías. 5. Grado de implementación de de sistemas de notificación de incidentes de seguridad, grado de uso de herramientas de análisis pre dictivo y número de reuniones de evaluación de eventos Algunas de las soluciones propuestas son fácilmente incorporables a la práctica diaria. Sin embargo, otras requieren más tiempo y, sobre todo, acciones por grupos internacionales que trabajen conjuntamente para aportar soluciones concretas.In the session dedicated to Nuclear Medicine (NM) the five aspects considered the most problematic in radiation safety in NM were identified. These refer to: 1) Ensure the correct dose is delivered to the patient; 2) Avoid contamination and irradiation of the upper extremities, lens of the eyes and rest of the body; 3) Ensure the optimization of doses in diagnosis and treatment; 4 Promote the justification of the examinations in NM; and 5) Prevent incidents and accidents. The solutions provided to these problems were: 1) To implement quality management systems and quality control protocols as well as to educate and to train adequately the workers; 2) To improve the training and formation of workers, systematic use of personal pro tection equipment (PPE) and standard operation procedures (SOP’s) and adapta tion of working procedures; 3) To use standardized doses in diagnosis and planning each treatment by patient-spe cific dosimetry; 4) To train referring physicians and nuclear medicine physicians and to use referral guidelines for appropriate MN examinations; and 5) To incorporate effectively an incident reporting system for later analysis and learn ing through the use of event analysis techniques. The proposed indicators for an adequate evaluation of the obtained progress in each one of the assessed aspects were: 1) Number of centres with an implemented quality management system and its de gree of compliance in each centre; 2) Continuous trend analysis of dosimetric reports values; 3 Number of studies with dose optimisation protocols and/or patient-specific dosimetry; 4) Number of undergraduate medical programs that include subjects related to radia tion safety and number of written standard operation procedures with indications of each study and percentage of studies that comply with these guidelines; 5) Degree of implementation of security incident reporting systems, degree of use of predictive analysis tools and number of incident evaluation meetings; Some of the proposed solutions can be easily incorporated into daily practice. However, others require more time and, additionally, actions by international groups working together to provide concrete solutions.Na sessão dedicada à Medicina Nuclear (MN) consideraram-se cinco aspetos mais problemáticos na proteção contra as radiações em MN: 1. Garantia que a dose administrada ao paciente é correta; 2. Evitar a contaminação e irradiação das mãos, cristalino e resto do corpo do trablhador na área; 3. Assegurar a otimização das doses de diagnóstico e terapêutica; 4. Justificação de exames em MN; 5. A prevenção de incidentes e acidentes. Foram apresentadas as seguintes soluções para esses problemas: 1. Implementação de protocolos e regras de funcionamento bem definidas associadas a sistemas de controle de qualidade dos equipa mentos e à formação continua dos trabalhadores; 2. Melhorar a formação e treinamento dos trabalhadores na utilização sistemática dos meios de proteção e protocolos adequados aos procedimentos de trabalho; 3. Padronização das doses de diagnóstico e planificação das terapêuticas por dosimetria interna específica dos doentes; 4. Formação dos médicos prescritores e nucleares para utilização adequada de “Guidelines” para exames de MN; 5. Incorporação efetiva de sistemas de registo de incidentes e acidentes para posterior análise e melhoria das práticas utilizadas; Os indicadores propostos para avaliação dos avanços em cada um dos aspectos foram definidos como: 1. Número de centros com programa de garantia de qualidade utilizado e o grau de cumprimento em cada centro 2. A análise de tendência dos dados de dosimetria relatados. 3. Número de exames com os protocolos de otimização de dose ou estimativa da actividade específica para o paciente. 4. Número de currículos de graduação médica que incluam radioproteção, número de protocolos clínicos escritos com as indicações de cada estudo e porcentagem de testes que estejam em conformidade com estas diretrizes. 5. Grau de implementação da sistemas de informação sobre incidente, grau de utilização de ferramentas de análise preditiva e reuniões de avaliação número de eventos. Algumas das soluções propostas são fáceis de implementar na prática diária. Outras exigem mais tempo e, sobretudo, ações de grupos internacionais que trabalhem em conjunto para fornecer soluções concretas.UCR::Vicerrectoría de Docencia::Ciencias Básicas::Facultad de Ciencias::Escuela de FísicaUCR::Vicerrectoría de Investigación::Unidades de Investigación::Ciencias Básicas::Centro de Investigación en Ciencias Atómicas Nucleares y Moleculares (CICANUM