Assessing Neurodevelopment in Congenital Heart Disease.
Congenital Heart DiseaseCongenital heart disease (CHD) is the most prevalent congenital malformation affecting 1 in 100 newborns per year. Children with CHD are a known risk population for brain injury, with neurodevelopmental alterations shown over time in up to 50% of cases. No adequate description exists of the type of neurocognitive anomalies or risk factors associated with CHD, and consequently no prognostic markers that may allow identification of high-risk cases are available.
Contegra Versus Pulmonary Homograft for Right Ventricular Outflow Tract Reconstruction in Newborns...
Congenital Heart DefectRight Ventricular Outflow Tract ReconstructionPulmonary homografts are standard substitutes for right ventricular outflow tract reconstruction in congenital heart surgery. Unfortunately shortage and conduit failure secondary to early calcifications and shrinking are observed particularly for small sized conduits in younger patients. In neonates, Contegra® 12mm could be a valuable alternative, but conflicting evidence exists. This retrospective study compared the outcome of these two conduits in a newborn population.
Ultrafiltration in Pediatric Cardiac Surgery: An Observational Cohort Study
Congenital Heart DiseaseInflammationInfants and children undergoing cardiac surgery with cardiopulmonary bypass (CPB) can experience systemic inflammation that prolongs post-operative recovery. Ultrafiltration is an intra-opreative technique that is hypothesized to extract circulating inflammatory mediators during the CPB time. There have been only a few small studies looking at a limited number of inflammatory marker profiles in this context. Our institution uses an innovative form of ultrafiltration "subzero-balance simple-modified ultrafiltration" (SBUF-SMUF) throughout the entire CPB time. SBUF-SMUF has been our standard of care for the last 5 years. This observational seeks to describe the clinical and immunologic outcomes of infants and children undergoing cardiac surgery with CBP and SBUF-SMUF.
Motor Development of Children That Have Surgery as Newborns for Complex Congenital Heart Disease...
Heart DefectsCongenitalInfants requiring surgery in the neonatal period for complex congenital heart diseases are at risk for developmental problems. For infants with congenital heart diseases with admixture physiology and single ventricles, optimal circulation is associated with signs of adequate systemic perfusion and a systemic arterial oxygen saturation typically between 75% to 90%. Infants are often unable to withstand standardized developmental testing during early infancy due to medical fragility and sternal precautions after surgery. Evaluation of the quality of spontaneous movements and movement variability is a good alternative. The quality of general movements in early infancy is a valid predictor of neurological disorders in high risk infant groups and is assessed with short periods of video-recorded observations. This methodology has yet to be studied in infants with complex congenital heart disease that require surgery as neonates. For older infants, the Infant Motor Profile (IMP) is a promising tool to document developmental outcome.
Quality of Life in Young Adults With Congenital Heart Disease
Congenital Heart DiseaseThis study aims to evaluate the psychosocial situation of adult congenital heart disease (CHD) patients in terms of health-related quality of life, mental health, academic achievement, and employment status. By assessing a wide variety of medical (e.g., disease severity) and psychosocial (e.g., life events, coping strategies, personality) risk factors it will be possible to better understand the variables that influence psychosocial outcome of young adults with congenital heart disease. This will further improve the understanding of the lifelong consequences of a congenital heart malformation. Factors that proof to be predictors of favorable outcome represent a resource of resilience and therefore should play an important role in the care of CHD patients. By implementing those results in patient care the investigators aim to achieve an improved psychosocial outcome among adult congenital heart disease (ACHD). Hypothesis 1: It is expected that perceived health status, health-related quality of life and psychological adjustment will not differ between the patient and the control group. However, academic achievement and employment status are expected to be poorer in young adults with congenital heart disease compared to healthy controls. Hypothesis 2: It is assumed that parental socioeconomic status, problems in emotional regulation and impaired social support will be related to a negative psychosocial outcome and health-related quality of life. Moreover, the investigators hypothesize that disease severity is associated with academic outcome and employment status.
Fluid Balance During Closure of Atrial Septal Defect
Heart Defects,CongenitalHeart Septal Defects1 moreThis project will evaluate fluid balance and oedema formation in children with the same congenital heart disease (Atrial Septal Defect) who will either go through heart surgery with the use of Cardio Pulmonary Bypass and hypothermia or through interventional catheterization. The investigators will measure interstitial colloid osmotic pressure, distribution of proteins and cytokines. The study hypothesis is that "Oedema developed during heart surgery is caused by reduced colloid osmotic pressure gradient through the capillary membrane".
National Registry of Genetically Triggered Thoracic Aortic Aneurysms and Cardiovascular Conditions...
Marfan SyndromeTurner Syndrome14 moreThe National Registry of Genetically Triggered Thoracic Aortic Aneurysms and Cardiovascular Conditions (GenTAC) was initiated in 2006 by the National Heart, Lung, and Blood Institute (NHLBI) and the National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS). GenTAC established a registry of 3706 patients with genetic conditions that may be related to thoracic aortic aneurysms and collected medical data and biologic samples. The study ended in September 2016. Data and samples are available from NHLBI and requests should be made to BioLINCC. See the NHLBI website for more information: https://www.nhlbi.nih.gov/research/resources/gentac/.
Patient-Reported Outcomes in Adults With Congenital Heart Disease
Heart DefectsCongenitalThe purpose of this study is to examine the differences in perceived health, psychosocial functioning, behavioral outcomes and quality of life of adults with congenital heart disease who are living in different areas of the world, and how these differences can be understood (e.g., differences in sense of coherence or illness perceptions).
Genetic Determinants of Congenital Heart Disease Outcomes
Congenital Heart DiseaseHypoplastic Left Heart Syndrome2 moreThe purpose of this study is to examine the role of genetic variation in the oxidative stress response on critical perioperative and short-term outcomes after neonatal heart surgery. The goals will be to determine 1) if the oxidative stress pathway is an important one for therapeutic intervention in neonates with severe congenital heart defects and 2) if variants in the oxidative response pathway can be used to identify patients at increased risk for adverse outcomes.
Prenatal Effects of Congenital Heart Disease (CHD) on Neurodevelopmental Outcome
Congenital Heart DiseaseThe purpose of this study is to investigate the prenatal impact of abnormal cardiac structure on neurodevelopmental outcomes in children with congenital heart disease.