Influence of Genetic Polymorphisms on Ventricular Structure and Function in Patients With Single...
Congenital Heart DiseaseThere are many kids born with congenital heart disease. Some of these defects may lead to the formation of a single ventricle (the heart having only one pumping chamber). These children normally undergo a series of corrective surgeries to help overcome the problems of having just one ventricle. However there are some differences in how well the patients respond to the surgeries. In the adult population, studies have shown that there may be a genetic link that may be responsible for the differences in how patients respond. The investigators would like to study the pediatric population by looking for certain genetic markers in the patients' blood. They will also collect basic health information on each patient.
Impact of Early Intervention on Maternal Stress in Mothers of Fetuses Diagnosed With Single Ventricle...
Heart DefectsCongenital4 moreThis study evaluates maternal psychological distress and the impact of early palliative care team consultation on maternal anxiety and depression symptoms, coping, and quality of life/family functioning in the care of neonates born with single ventricle physiology. Half of the participants will receive early palliative care team consultation, while the other half will receive usual care (no or late palliative care intervention). The investigators hypothesize maternal stress, anxiety, and depression will be lower in the palliative care intervention group compared with the control group, and maternal coping mechanisms and perceived quality of life and family functioning will improve at the pre-discharge assessment.
Adaptation of Alveolar-capillary Diffusion at Effort of Subjects Suffering From Complex Congenital...
Complex Congenital Heart DiseaseSingle Ventricle With Fontan Circulation or Bicavo-bipulmonary Bypass2 moreAn injury of haematosis in post ischemic chronic heart failure limits the clinic tolerance. There is a correlation between injury of pulmonary diffusing, chronic heart failure intensity and aerobic physic ability evaluated by an heart-rate maximal exercise tolerance test (VO2 max). This injury is a new follow-up parameter of cardiac function for the adult. The nature of damage (vascular or membrane) can be determined by the measure of double pulmonary diffusing capacity to carbon monoxide (CO) associated to nitric oxide (NO). Today, in chronic heart failure consecutive to a congenital heart disease, there is no data on evolution of membrane and capillar factors.It is impossible to predict if membrane damage will be the best factor correlated to the VO2max in patients suffering from complex congenital heart disease. Assessing these parameters could be an comparative evaluation of heart-rate exercise tolerance test with VO2max and an early control of his damage without risks related to heart-rate maximal exercise and independently of age, sex, hemoglobin, type of heart disease.These results would have an early prognostic value that would permit to refine the follow-up and the treatment. The main objective of this trial is to assess the statistic correlation between the membrane injury of alveolar-capillary diffusing at rest and aerobic physic ability restriction in children and adults suffering from complex congenital heart disease.
Biomarker Study for Heart Failure in Children With Single Ventricle Physiology
Tricuspid AtresiaHypoplastic Left Heart Syndrome1 moreThe purpose of this study is to determine if children with heart disease where there is only one pumping chamber("ventricle") have proteins (biomarkers") in the blood that can be used to monitor the function of their heart.
Plastic Bronchitis and Protein Losing Enteropathy in Children With Single Ventricle Physiology
Fontan Physiology Patients With PB or PLEFontan Physiology Patients Without PB or PLEThe investigators are studying what causes Plastic Bronchitis and Protein Losing Enteropathy. The investigators think that these problems are from too much of two small proteins called Vasoactive Intestinal Peptide (VIP) and Substance P. VIP and Substance P are important proteins in the body that normally tell the body to make small amounts of fluid and they help the intestines work. Normally, VIP and Substance P are made in the intestines and then destroyed in the lungs after they do their normal work. The investigators think that kids who have Plastic Bronchitis and/or Protein Losing Enteropathy who also had the Fontan surgery might have too much VIP and Substance P in their bodies. The investigators think this causes too much fluid to go in the lungs and too much protein in the intestines.
Global and Regional Myocardial Strain and Power Output In Patients With Single Ventricles Using...
Fontan ProcedureHypoplastic Left Heart Syndrome2 moreThe investigators are beginning a clinical research study here at UCSF to learn more about how hearts with single ventricles work. The investigators hypothesize that myocardial strain , as measured by cardiac MRI,in patients with single ventricles differs from normal hearts. This abnormality is associated with depressed cardiac power output and impaired exercise capacity. The study will utilize cardiac magnetic resonance imaging, exercise testing, and lung function testing to study how the single ventricle heart works so that in the future the investigators can improve how the investigators care for patients with this type of congenital heart disease. The research protocol involves: Cardiac magnetic resonance imaging (MRI of the heart), exercise tests, and lung function tests. Some of these tests are routinely used to follow patients with single ventricles. Some of these tests are part of the research protocol. If you agree to participate in this study, these tests will be performed at UCSF. The results of the routine tests will be shared with your doctor. Participation will require approximately 30 additional minutes to the cardiac MRI procedure to collect the research data. The exercise tests, and lung function tests time commitment will take about two hours.
Changes in Cerebral Oxygenation During Laparoscopy in Patients With Single Ventricle Anatomy
Congenital Heart DiseaseSingle VentriclePatients with single ventricle physiology (hypoplastic left heart syndrome, tricuspid atresia) frequently have feeding difficulties necessitating procedures such as Nissen fundoplication and G-tube placement. With advances in minimally invasive surgery, these are frequently performed using laparoscopic techniques. Although generally safe and effective, the increase in IAP during laparoscopy may increase systemic and pulmonary vascular resistance and decrease cardiac output. This prospective study will include 50 patients with single ventricle physiology presenting for laparoscopic procedures. There will be no change in the anesthetic or perioperative care of these patients. Tissue and cerebral oxygenation will be monitored using near infrared spectroscopy (NIRS).
Sleep Disordered Breathing in Children With Single Ventricle Physiology
Hypoplastic Left Heart SyndromeObstructive Sleep Apnea Syndrome1 moreThis is an exploratory study designed to evaluate the incidence of, and to quantify sleep disordered breathing following stage I Norwood reconstructive surgery. Sleep disordered breathing will be correlated with: Elevations in pulmonary vasculature resistance at the time of Stage II surgery. Risks of death
Exercise Capacity in Patients With Fontan Procedure
Single-ventricleFontan ProcedureIn literature, it has been reported that exercise capacity decreases in single-ventricle patients undergoing Fontan Procedure due to various factors related to surgery and underlying cardiac anomaly. Cardiopulmonary exercise test (CPET) was generally used to evaluate exercise capacity in children and adults with fontan circulation. The fact that CPET requires a clinically expert team and equipment and is more expensive does not always make it possible to perform routine cardiopulmonary exercise testing. In some cases, field tests evaluating submaximal and maximal exercise capacity may be more practical than cardiopulmonary exercise testing. Therefore, in this study, it is aimed to evaluate the exercise capacity of Fontan patients with 6-minute walking test and incremental shuttle walk test and to compare vital signs during the tests.
Nutritional Failure in Infants With Single Ventricle Congenital Heart Disease
Congenital Heart DiseaseNutritional FailureThe purpose of this study is to understand the causes of growth failure and to define biochemical markers of nutritional failure in patients with single ventricle Congenital Heart Disease (CHD).