Técnicas de imagen en la coartación de aorta

Coarctation of aorta is a congenital malformation relatively frequent in the population. The clinical presentation varies widely, from symptoms at birth to incidental diagnosis in adulthood. Imaging techniques allow us to assess the anatomy of the stenosis, hemodynamics, left ventricle function and hypertrophy and the presence of associated complications. Transthoracic echocardiography is the first line technique but computed tomography and cardiac magnetic resonance are the tests of choice for surgical evaluation of the native coarctation and to followup the repaired coarctation.La coartación aórtica es una malformación congénita relativamente frecuente. Sus manifestaciones abarcan un amplio espectro de presentación, pudiendo mostrar síntomas desde el nacimiento, o bien diagnosticarse en la edad adulta. Para su manejo se dispone de una amplia gama de técnicas de imagen que permiten valorar la anatomía de la estenosis, las repercusiones estructurales en el ventrículo izquierdo, las alteraciones hemodinámicas y las complicaciones asociadas. La ecocardiografía transtorácica es la técnica de primera línea. Para la valoración quirúrgica en la coartación nativa y el seguimiento de la coartación reparada, la tomografía computarizada y la resonancia magnética son las técnicas de elección

MEAN-pinolla tehdyn järjestelmän yksikkötestaaminen

Tämä opinnäytetyö on laadittu tilaajayritys Mekiwi Oy:n tarpeisiin ja siinä tutkitaan yksikkötestejä sekä niiden merkitystä ja hyötyjä MEAN-pinolla tehdyssä järjestelmässä. Tarve opinnäytetyölle ilmeni, kun sen kohteena olevan järjestelmän testausta haluttiin helpottaa ja automatisoida. Tavoitteena oli tuottaa yritykselle tietoa yksikkötestauksesta ja yksikkötestien rakentamisesta sekä suunnitella ja toteuttaa yksikkötestit kattamaan järjestelmän koko lähdekoodi. Alusta asti oli selvää, että tutkimusta tarvittaisiin varsin vähän, sillä työn painopiste on toiminnallisuudessa. Lähteiden sisältö onkin suurimmaksi osaksi teoriapohjaa, jolla avataan tarvittavat käsitteet ja teknologiat itse toteutusosaa varten. Käytetyistä lähteistä suurin osa on kunkin käsiteltävän asian kotisivuja. Opinnäytetyön tuloksena on kaksi sarjaa yksikkötestejä, jotka testaavat järjestelmän asiakassovelluksen käyttäjiä koskevia tietokantatoimintoja ja HTTP-rajapintoja. Lisäksi sen tuloksena saatiin tietoa tarvittavista toimenpiteistä yksikkötestien tekemisestä kattamaan koko järjestelmän koodi. Pelkkä testien kirjoittaminen ei tule riittämään, sillä sekä järjestelmän palvelin- että asiakassovelluksessa on koodia, jonka yksikkötestaaminen ilman koodimuutoksia on vähintäänkin epäluotettavaa ellei jopa mahdotonta.This thesis was made for a company called Mekiwi Oy. The subject is to examine the purpose and benefits of unit tests in a software built with the MEAN stack. The need for this thesis arose when the company wanted to improve the testing of a software and to make it more automatic. The goal was to provide information to the company about unit testing and to design and produce unit tests to cover the whole of the software’s codebase. It was clear right from the start that the need for research would be limited because the main focus would be on the functional side. The majority of the source material used is information about the concepts and technologies described either in the theory section or in the implementation section. The vast majority of the references are links to a homepage of the subject in question. The end result was two sets of unit tests: one testing the database functionalities regarding users and one testing the HTTP application programming interfaces regarding users. Information on creating unit tests to cover the entire codebase was also uncovered. Mere writing the unit testing will not be sufficient because both the client and the server have code that will need refactoring to make unit testing it possible

Time course and predictors for neoaortic root dilatation and neoaortic valve regurgitation during adult life after arterial switch operation

[ES] Introducción y objetivos: Hay pocos datos sobre la evolución en adultos de la dilatación de la raíz neoaórtica (RAO) y la insuficiencia valvular neoaórtica (IA) tras la cirugía de switch arterial (SA) en la transposición de grandes arterias. Métodos: Análisis retrospectivo de 152 pacientes con transposición de grandes arterias, mayores de 15 años, intervenidos mediante SA y seguidos durante 4,9 ± 3,3 años en 2 centros de referencia. Se analizaron los cambios de diámetro de la RAO ajustados a superficie corporal y la progresión a grado moderado/grave de la IA con ecocardiografías seriadas. Se realizó un modelo de regresión de Cox para identificar factores predictores de progresión de la IA. Resultados: Inicialmente, 4 pacientes (2,6%) presentaban IA grave (3 habían precisado cirugía valvular) y 9 (5,9%) moderada. La RAO basal media era 20,05 ± 2,4 mm/m2, y al final del seguimiento, 20,73 ± 2,8 mm/m2 (p < 0,001), con un crecimiento medio de 0,14 (IC95%, 0,07-0,2) mm/m2/año. La IA progresó en 20 (13,5%) y 6 (4%) fueron intervenidos. La progresión de IA se asoció con válvula bicúspide, IA inicial, dilatación de la RAO inicial y crecimiento de la RAO. La válvula bicúspide (HR = 3,3; IC95%, 1,1-15,2; p = 0,037), la IA inicial (HR = 5,9; IC95%, 1,6-59,2; p = 0,006) y el crecimiento de la RAO (HR = 4,1; IC95%, 2-13,5; p = 0,023) resultaron predictores independientes. Conclusiones: La dilatación de la RAO y la IA progresan en el adulto joven intervenido mediante SA. La válvula bicúspide, la IA basal y el crecimiento de la RAO son predictores de progresión de IA.[EN] Introduction and objectives: There are limited data on the long-term development of neoaortic root dilatation (NRD) and neoaortic valve regurgitation (AR) after arterial switch operation (ASO) for transposition of the great arteries during adult life. Methods: We performed a retrospective longitudinal analysis of 152 patients older than 15 years who underwent ASO for transposition of the great arteries and who were followed-up for 4.9 ± 3.3 years in 2 referral centers. Sequential changes in body surface-adjusted aortic root dimensions and progression to moderate/severe AR were determined in patients with 2 or more echocardiographic examinations. Risk factors for dilatation were tested by Cox regression to identify predictors of AR progression. Results: At baseline, moderate AR was present in 9 patients (5.9%) and severe AR in 4 (2.6%), of whom 3 had required aortic valve surgery. Initially, the median neoaortic root dimension was 20.05 ± 2.4 mm/m2, which increased significantly to 20.73 ± 2.8 mm/m2 (P < .001) at the end of follow-up. The mean change over time was 0.14 mm/m2/y (95%CI, 0.07-0.2). Progressive AR was observed in 20 patients (13.5%) and 6 patients (4%) required aortic valve surgery. Progressive AR was associated with bicuspid valve, AR at baseline, NRD at baseline, and neoaortic root enlargement. Independent predictors were bicuspid valve (HR, 3.3; 95%CI, 1.1-15.2; P = .037), AR at baseline (HR, 5.9; 95%CI, 1.6-59.2; P = .006) and increase in NRD (HR, 4.1 95%CI, 2-13.5; P = .023). Conclusions: In adult life, NRD and AR progress over time after ASO. Predictors of progressive AR are bicuspid valve, AR at baseline, and increase in NRD

Predicting sudden cardiac death in adults with congenital heart disease

[Objectives] To develop, calibrate, test and validate a logistic regression model for accurate risk prediction of sudden cardiac death (SCD) and non-fatal sudden cardiac arrest (SCA) in adults with congenital heart disease (ACHD), based on baseline lesion-specific risk stratification and individual’s characteristics, to guide primary prevention strategies.[Methods] We combined data from a single-centre cohort of 3311 consecutive ACHD patients (50% male) at 25-year follow-up with 71 events (53 SCD and 18 non-fatal SCA) and a multicentre case–control group with 207 cases (110 SCD and 97 non-fatal SCA) and 2287 consecutive controls (50% males). Cumulative incidences of events up to 20 years for specific lesions were determined in the prospective cohort. Risk model and its 5-year risk predictions were derived by logistic regression modelling, using separate development (18 centres: 144 cases and 1501 controls) and validation (two centres: 63 cases and 786 controls) datasets.[Results] According to the combined SCD/SCA cumulative 20 years incidence, a lesion-specific stratification into four clusters—very-low (12%)—was built. Multivariable predictors were lesion-specific cluster, young age, male sex, unexplained syncope, ischaemic heart disease, non-life threatening ventricular arrhythmias, QRS duration and ventricular systolic dysfunction or hypertrophy. The model very accurately discriminated (C-index 0.91; 95% CI 0.88 to 0.94) and calibrated (p=0.3 for observed vs expected proportions) in the validation dataset. Compared with current guidelines approach, sensitivity increases 29% with less than 1% change in specificity.[Conclusions] Predicting the risk of SCD/SCA in ACHD can be significantly improved using a baseline lesion-specific stratification and simple clinical variables.Peer reviewe

The coronavirus disease pandemic among adult congenital heart disease patients and the lessons learnt - results of a prospective multicenter european registry.

BACKGROUND At the beginning of the COVID-19 pandemic, professionals in charge of particularly vulnerable populations, such as adult congenital heart disease (ACHD) patients, were confronted with difficult decision-making. We aimed to assess changes in risk stratification and outcomes of ACHD patients suffering from COVID-19 between March 2020 and April 2021. METHODS AND RESULTS Risk stratification among ACHD experts (before and after the first outcome data were available) was assessed by means of questionnaires. In addition, COVID-19 cases and the corresponding patient characteristics were recorded among participating centres. Predictors for the outcome of interest (complicated disease course) were assessed by means of multivariable logistic regression models calculated with cluster-robust standard errors. When assessing the importance of general and ACHD specific risk factors for a complicated disease course, their overall importance and the corresponding risk perception among ACHD experts decreased over time. Overall, 638 patients (n = 168 during the first wave and n = 470 during the subsequent waves) were included (median age 34 years, 52% women). Main independent predictors for a complicated disease course were male sex, increasing age, a BMI >25 kg/m2, having ≥2 comorbidities, suffering from a cyanotic heart disease or having suffered COVID-19 in the first wave vs. subsequent waves. CONCLUSIONS Apart from cyanotic heart disease, general risk factors for poor outcome in case of COVID-19 reported in the general population are equally important among ACHD patients. Risk perception among ACHD experts decreased during the course of the pandemic

Medical therapy for heart failure in adult congenital heart disease: does it work?

Heart failure (HF) is currently recognised as one of the most clinically important events resulting from the prolonged survival to adulthood of patients with congenital heart disease (ACHD). Hospitalisations involving HF have increased 91% over the last two decades,1 with an impact on rehospitalisations and deaths during follow-up. According to data extracted from the Dutch CONCOR Registry,2 patients with CHD first admitted for HF have a fivefold higher mortality risk than those with no admissions; more recently, a 25% risk of death within 1 year following first HF admission has been reported by Lal et al.3 Consequently, a steady increase in the proportion of HF-related deaths with ageing has been described and HF is currently cited as the leading cause of cardiac death in patients with ACHD.

Diseño y proposición de un sistema de control interno para las áreas de inventario y cartera del establecimiento de comercio Distribuidora Mayorista.

El presente proyecto de tesis es el diseño de un sistema de control interno para las áreas de inventario y cartera, aplicados al establecimiento de comercio Distribuidora Mayorista dedicada a la comercialización de licor, gaseosa, agua embotellada, y otros. La elaboración del sistema de control interno está apoyada en una serie de herramientas de auditoria tales como son: Cuestionarios de control interno, narrativas, diagramas de flujo, todo esto basado en los cinco componentes del control interno, obteniendo como resultado las debilidades que se deben corregir para una mejor administración del negocio. Ahora bien el cumplimiento de este objetivo depende en gran parte de la capacidad de la empresa para planificar y coordinar el uso de todos sus recursos y de medir que las actividades se estén realizando como han sido planificadas, así mismo, deben poseer los mecanismo de control para cada área a investigar

Adultos con cardiopatía congénita durante la pandemia de COVID-19: ¿población de riesgo?

En diciembre de 2019 se describió´ un nuevo coronavirus, causante del síndrome respiratorio agudo grave (SARS-CoV-2), en pacientes que presentaban síntomas de neumonía en la ciudad de Wuhan (China). Con una tasa global de mortalidad de un 2-3%, la enfermedad por coronavirus de 2019 (COVID-19) se ha convertido en pandemia mundial y se hizo endémica en Europa a comienzos de marzo de 2020. El 4 de junio de 2020, la infección por el SARSCoV-2 había afectado ya a más de 6,5 millones de personas y había causado mas de 380.000 muertes en el mundo. Los varones de mediana o avanzada edad son las personas afectadas con más frecuencia y mayor gravedad, pero pueden contraer la COVID-19 personas de cualquier edad. Aunque en su mayor parte no son graves, el espectro de la infección sintomática va de los casos leves a los críticos. En los pacientes con una evolución critica de la enfermedad, las principales manifestaciones clínicas consisten en neumonía bilateral, síndrome de disnea aguda, insuficiencia cardiaca, shock séptico y disfunción multiorga´nica3–5. Adema´ s, la COVID-19 evoluciona a menudo hacia una enfermedad inflamatoria sistémica que tiene repercusiones en el sistema vascular y hematopoyético, y la hipercoagulabilidad es frecuente en los pacientes con COVID-19 hospitalizado

Risk stratification of adults with congenital heart disease during the COVID-19 pandemic: insights from a multinational survey among European experts

OBJECTIVE Adults with congenital heart disease (ACHD) may be at a higher risk of a fatal outcome in case of COVID-19. Current risk stratification among these patients relies on personal experience and extrapolation from patients with acquired heart disease. We aimed to provide an expert view on risk stratification while awaiting results from observational studies. METHODS This study was an initiative of the EPOCH (European Collaboration for Prospective Outcome Research in Congenital Heart disease). Among nine European countries (Austria, Belgium, Denmark, France, Germany, Italy, the Netherlands, Spain and Switzerland), 24 experts from 23 tertiary ACHD centres participated in the survey. ACHD experts were asked to identify ACHD-specific COVID-19 risk factors from a list of potential outcome predictors and to estimate the risk of adverse COVID-19 outcomes in seven commonly seen patient scenarios. RESULTS 82% of participants did not consider all ACHD patients at risk of COVID-19 related complications. There was a consensus on pulmonary arterial hypertension, Fontan physiology and cyanotic heart disease as risk factors for adverse outcomes. Among different ACHD scenarios, a patient with Eisenmenger syndrome was considered to be at the highest risk. There was a marked variability in risk estimation among the other potential outcome predictors and ACHD scenarios. CONCLUSIONS Pulmonary arterial hypertension, Fontan palliation and cyanotic heart disease were widely considered as risk factors for poor outcome in COVID-19. However, there was a marked disparity in risk estimation for other clinical scenarios. We are in urgent need of outcome studies in ACHD suffering from COVID-19