Individual quality of life in adults with congenital heart disease: a paradigm shift

Aims During the last decade, a paradigm shift has emerged in the measurement of quality of life, from the use of standard questionnaires towards a more individualized approach. Therefore, this study examined individual quality of life in adults with congenital heart disease and explored potential differences with those reported by matched, healthy control subjects. Methods and results We examined 579 adults with congenital heart disease. A subsample of 514 of these patients was matched for age, gender, educational level, and employment status with 446 healthy counterparts. Individual quality of life was assessed using the Schedule for the Evaluation of Individual Quality of Life-Direct Weighting (SEIQoL-DW). Twelve domains affecting patients' quality of life were identified. Family, job/education, friends, health, and leisure time were the most prominent quality of life domains. Significantly fewer patients than control subjects considered financial means and material well-being and future to be important determinants of quality of life. Conclusion Assessment of quality of life in adults with congenital heart disease that focusses on the individual is appropriate for obtaining in-depth information on issues relevant for patients' quality of life. This represents a paradigm shift in the measurement of this concep

PATHway: decision support in exercise programmes for cardiac rehabilitation

Rehabilitation is important for patients with cardiovascular diseases (CVD) to improve health outcomes and quality of life. However, adherence to current exercise programmes in cardiac rehabilitation is limited. We present the design and development of a Decision Support System (DSS) for telerehabilitation, aiming to enhance exercise programmes for CVD patients through ensuring their safety, personalising the programme according to their needs and performance, and motivating them toward meeting their physical activity goals. The DSS processes data originated from a Microsoft Kinect camera, a blood pressure monitor, a heart rate sensor and questionnaires, in order to generate a highly individualised exercise programme and improve patient adherence. Initial results within the EU-funded PATHway project show the potential of our approach

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

Mitral Valve Prolapse

Mitral valve prolapse (MVP) is the most common valvular abnormality, affecting 2.4% of the population. Usually MVP is a benign disease and remains asymptomatic. The diagnosis of MVP is based on clinical presentation, physical examination and echocardiography. Some atypical symptoms that are not correlated with mitral valve function, are described as the MVP syndrome. Potential complications such as infective endocarditis, thromboembolic events, atrial and ventricular arrhythmias, and progressive mitral valve regurgitation may occur. Management should concentrate on adequate guidance of the patients, relief of symptoms and avoidance of complications

Pregnancy outcomes in women with cardiovascular disease: evolving trends over 10 years in the ESC Registry Of Pregnancy And Cardiac disease (ROPAC)

Aims Reducing maternal mortality is a World Health Organization (WHO) global health goal. Although maternal deaths due to haemorrhage and infection are declining, those related to heart disease are increasing and are now the most important cause in western countries. The aim is to define contemporary diagnosis-specific outcomes in pregnant women with heart disease. Methods and results From 2007 to 2018, pregnant women with heart disease were prospectively enrolled in the Registry Of Pregnancy And Cardiac disease (ROPAC). Primary outcome was maternal mortality or heart failure, secondary outcomes were other cardiac, obstetric, and foetal complications. We enrolled 5739 pregnancies; the mean age was 29.5. Prevalent diagnoses were congenital (57%) and valvular heart disease (29%). Mortality (overall 0.6%) was highest in the pulmonary arterial hypertension (PAH) group (9%). Heart failure occurred in 11%, arrhythmias in 2%. Delivery was by Caesarean section in 44%. Obstetric and foetal complications occurred in 17% and 21%, respectively. The number of high-risk pregnancies (mWHO Class IV) increased from 0.7% in 2007–2010 to 10.9% in 2015–2018. Determinants for maternal complications were pre-pregnancy heart failure or New York Heart Association >II, systemic ejection fraction <40%, mWHO Class 4, and anticoagulants use. After an increase from 2007 to 2009, complication rates fell from 13.2% in 2010 to 9.3% in 2017. Conclusion Rates of maternal mortality or heart failure were high in women with heart disease. However, from 2010, these rates declined despite the inclusion of more high-risk pregnancies. Highest complication rates occurred in women with PAH

Three live-birth pregnancies in a woman with Williams syndrome

Objective. Maternal Williams syndrome with their associated (cardiac) malformations is rarely encountered during pregnancy.Methods. We report on a patient with Williams syndrome who has had 3 live-birth pregnancies.Results. Several noncardiac, mainly fetal, complications need to be anticipated: premature labor, intrauterine growth restriction with subsequent small-for-gestational-age offspring, and recurrence of congenital heart disease or similar syndromes.Conclusion. The present case report illustrates that multiple live-birth pregnancies are possible in women with Williams syndrome, and it advocates the need for regular multidisciplinary assessments prior to and during pregnancy.</p

Validity, reliability and responsiveness of the "Schedule for the Evaluation of Individual Quality of Life – Direct Weighting" (SEIQoL-DW) in congenital heart disease

BACKGROUND: The 'Schedule for the Evaluation of Individual Quality of Life – Direct Weighting' (SEIQoL-DW) is an instrument developed to measure individual quality of life. Although this instrument has been used in numerous studies, data on validity and reliability are sparse. This study aimed to examine aspects of validity, reliability and responsiveness of the SEIQoL-DW on data obtained in adults with congenital heart disease, by using the new standards of psychological testing. METHODS: We evaluated validity evidence based on test content, internal structure, and relations to other variables, as well as the stability and responsiveness of the SEIQoL-DW. Evidence was provided by both theoretical considerations and empirical data. Empirical data were acquired from two studies. Firstly, using a cross-sectional study design, we included 629 patients with congenital heart disease. Secondly, 130 of the 629 initially included patients readministered the questionnaires approximately one year after the first data collection. In addition to the SEIQoL-DW, linear analog scales were used to assess overall quality of life and perceived health. RESULTS: We found that the SEIQoL-DW is not a valid measure of quality of life, but rather assesses determinants that contribute to individuals' quality of life. The SEIQoL-DW consistently proved to be valid and reliable to assess those determinants. However, responsiveness in patients with congenital heart disease may be problematic. CONCLUSION: Based on theoretical and empirical considerations, the SEIQoL-DW cannot be considered as a quality of life instrument. Nonetheless, it is a valid and reliable instrument to explore determinants for patients' quality of life

Three live-birth pregnancies in a woman with Williams syndrome

Objective. Maternal Williams syndrome with their associated (cardiac) malformations is rarely encountered during pregnancy.Methods. We report on a patient with Williams syndrome who has had 3 live-birth pregnancies.Results. Several noncardiac, mainly fetal, complications need to be anticipated: premature labor, intrauterine growth restriction with subsequent small-for-gestational-age offspring, and recurrence of congenital heart disease or similar syndromes.Conclusion. The present case report illustrates that multiple live-birth pregnancies are possible in women with Williams syndrome, and it advocates the need for regular multidisciplinary assessments prior to and during pregnancy.</p

Paediatric and adult congenital cardiology education and training in Europe

Background:Limited data exist on training of European paediatric and adult congenital cardiologists.Methods:A structured and approved questionnaire was circulated to national delegates of Association for European Paediatric and Congenital Cardiology in 33 European countries.Results:Delegates from 30 countries (91%) responded. Paediatric cardiology was not recognised as a distinct speciality by the respective ministry of Health in seven countries (23%). Twenty countries (67%) have formally accredited paediatric cardiology training programmes, seven (23%) have substantial informal (not accredited or certified) training, and three (10%) have very limited or no programme. Twenty-two countries have a curriculum. Twelve countries have a national training director. There was one paediatric cardiology centre per 2.66 million population (range 0.87–9.64 million), one cardiac surgical centre per 4.73 million population (range 1.63–10.72 million), and one training centre per 4.29 million population (range 1.63–10.72 million population). The median number of paediatric cardiology fellows per training programme was 4 (range 1–17), and duration of training was 3 years (range 2–5 years). An exit examination in paediatric cardiology was conducted in 16 countries (53%) and certification provided by 20 countries (67%). Paediatric cardiologist number is affected by gross domestic product (R$^{2}$ = 0.41).Conclusion:Training varies markedly across European countries. Although formal fellowship programmes exist in many countries, several countries have informal training or no training. Only a minority of countries provide both exit examination and certification. Harmonisation of training and standardisation of exit examination and certification could reduce variation in training thereby promoting high-quality care by European congenital cardiologists

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

Adult congenital heart disease; Coronavirus disease 2019; Risk stratificationCardiopatia congènita de l'adult; Malaltia per coronavirus 2019; Estratificació del riscCardiopatía congénita del adulto; Enfermedad por coronavirus 2019; Estratificación del riesgoBackground 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.EPOCH is funded by internal grants without support from the pharmaceutical industry