18 Clinical Trials for Various Conditions
All neonates with congenital heart disease undergoing stage one palliation (Norwood procedure, Damus-Kaye-Stansel procedure) at Texas Children's Hospital will be regularly monitored for B-type natriuretic peptide (BNP) and Troponin level before the surgical procedure, on arrival to the cardiac intensive care unit after their surgical procedure, every 6 hours during the first 24 hours of the post-operative period, followed by daily levels for the first week, and then weekly during patient's regular laboratory work up schedule. These cardiac biomarkers are linked to demographic, hemodynamic, respiratory, pharmacological data available via Sickbay.
A randomized, blinded, controlled trial to evaluate growth velocity and clinical outcomes in infants with single ventricle physiology fed an exclusive human milk diet prior to, and throughout the post-operative period following, surgical repair. Human milk is defined as expressed human milk or donor milk and its derivatives, human milk-based fortifier and human milk caloric fortifier. The study hypothesis is that infants fed an exclusive human milk diet will have short and long term benefits, with improved wound healing, growth, and neurodevelopmental outcomes while reducing episodes of feeding intolerance and necrotizing enterocolitis (NEC).
This 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.
The 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.
Recent studies have shown beneficial effects of sildenafil in patients with single ventricle congenital heart disease. The purpose of this study is to determine whether Bosentan, a drug with similar effects, will have similar benefit in this patient population.
The 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.
This 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: 1. Elevations in pulmonary vasculature resistance at the time of Stage II surgery. 2. Risks of death
The purpose of this study is to investigates serum and stool biomarkers as predictors for post-operative feeding intolerance in infant patients with complex congenital heart defects who undergo single ventricle staged palliation surgery.
To determine if an exercise program of passive range of motion (ROM) is safe and feasible in infants with single ventricle (SV) physiology after their Norwood procedure.
Purpose: The purpose of the Destination Therapy Evaluation for Failing Fontan Study (DEFINe Study) is to perform a single center physician-investigator led feasibility study to initiate examination of the safety and efficacy of implanting continuous flow circulatory support devices in 20 patients with failing Fontan physiology, not amenable to other surgical or medical therapy and who are not candidates for heart transplantation. Based upon the results of the DEFINe study, consideration would be given to a larger multicenter study. The primary endpoint is survival without a disabling stroke at two years. Safety, functional status, total days alive following hospital discharge and quality of life assessments will be evaluated as secondary endpoints. Background: Patients born with the congenital anomaly of a single ventricle often undergo Fontan procedures to improve oxygenation and circulation. As the patient matures, the benefits of the Fontan procedures become exhausted. These patients present as young adults with heart failure, and should be considered for heart transplantation. "Failed Fontan" physiology patients are now more commonly surviving into their 30s and 40s due to the advancement in surgical Fontan procedures over the last several decades. Yet, many patients are not appropriate transplant candidates due to progression of pulmonary failure that surpasses the pulmonary vascular resistance limits, end organ failure effects to the liver, and cardiac failure that does not present with left ventricular ejection fractions of less than 25%. Congenital heart disease is considered a higher risk indication for transplantation. Such patients present a complex anatomy, scar tissue from prior procedures, unique pathological states and limited ability to assess hemodynamics. Methods: Patients with failing Fontan physiology will be evaluated for surgical intervention, heart transplantation or participation in the study for long-term support, known as Destination Therapy (DT). Patients who do not meet the study criteria will be asked to participate in a sub-study to collect patient information. Consenting patients who meet the study criteria will be implanted with a HeartMate II Ventricular Assist Device (VAD) to improve cardiac output. Following VAD implantation, the patient will recover in the hospital, and then discharge to home or an approved facility. Study data will be collected as long as the patient receives VAD support. Placement of the VAD and continuing patient care should improve cardiac output and quality of life for this population of patients born with the congenital anomaly of a single ventricle. Outcomes: The primary endpoint of the study is to examine survival without a disabling stroke at two years (defined as a score of four or greater on the Modified Rankin Scale). Safety, functional status, total-days-alive following hospital discharge, and quality of life assessments will be evaluated as secondary endpoints.
Our aim is a retrospective chart review seeking to determine any differences in the main clinical and outcome variables as well as general outcome data such as functional recovery, readmission rates, somatic growth and mortality. An n size of 150 to 200 for each of the 2 groups will give us excellent power to detect potential differences.
This study is a 12-month (52 week) safety extension study to supplement the FUEL Phase III clinical trial to provide safety information regarding the long-term use of udenafil in adolescents with single ventricle congenital heart disease.
There 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.
Mechanisms that typically result in increased cardiac output, such as inotropic support, increased heart rate, and decreased afterload, have a blunted effect in Fontan circulation. The "thoracic pump" is a contributor to venous return that has been largely unexplored in patients with Fontan physiology. Inspiratory muscle training can improve the performance of competitive athletes across a range of sports and can improve quality of life and functional capacity in heart failure patients, presumably by reducing inspiratory muscle fatigue and possibly by improving peripheral blood flow during exercise. One could surmise that the effects of these changes would be particularly important in the Fontan population. The investigators propose to study the effects of inspiratory muscle training on exercise and pulmonary function parameters in a cohort of adult Fontan patients. The investigators hypothesize that a 12-week program of inspiratory muscle training with an inspiratory impedance threshold device will improve inspiratory muscle strength and endurance, and that this will translate into improved exercise performance in patients with Fontan physiology.
Ultrasound is a technique that can provide images of the blood vessels such as arteries. The size of the arteries, such as the main blood vessel in the arm, can change under different conditions. Using ultrasound we can see how arteries change with movement or even drugs. We want to use ultrasound to see how blood vessels look in patients with Congestive Heart Failure (CHF) and to also see how a drug called Spironolactone, commonly prescribed for patients with this disease, effects blood vessel function in patients with congestive heart failure. This information may be used to change the standard of care for patients with heart failure especially if we show that Spironolactone has a positive effect on vessel function in patients with CHF.
This trial is being conducted to evaluate the effect of a small-group wellness education program combined with a longitudinal, individualized prescription exercise program on the wellness, resiliency, and daily activity levels of pediatric patients with Fontan physiology. There will be two phases for this project. The first phase is the "WE BEAT Group Wellness Education Program" and participants will be transitioned into the phase two HEART Club following phase one. The trial will look at feasibility and acceptability of the program. Additional hypothesis include home whether exercise interventions can: * be delivered without any associated serious cardiac events; * will result in a decreased proportion of patients who are categorized as frail when compared to the cohort's pre-test baseline. * will result in increased measured peak oxygen consumption when compared with their pre-intervention baseline. * will result in increased step counts measured monthly from baseline to end of intervention * will improve self-reported quality of life from baseline to post-intervention. * will result in increased patient reported activity level from baseline to post-intervention
This study will evaluate the clinical efficacy and safety of udenafil, an orally administered, potent and selective inhibitor of PDE5, versus placebo for the treatment of adolescent subjects who have undergone the Fontan procedure.
To determine the pharmacokinetic profile and safety of udenafil in adolescents with Fontan physiology and to assess the short-term pharmacodynamic effect of udenafil on pharmacodynamic measures of exercise capacity, ventricular performance, and vascular function.