78 Clinical Trials for Various Conditions
This is a single center, open-label, prospective, investigation to quantify the effects liver congestion and fibrosis has on hepatic drug metabolism and transport in children, adolescents, and young adults with Fontan circulation.
This is a pilot study using a novel, minimal risk, portable, hands-free oral-positive pressure device (oPEP) in patients with Fontan palliation that will examine whether using this device in both the acute and chronic phase will alter Fontan hemodynamics and create pulsatility in the Fontan circuit and thereby increasing cardiac output. This device is easy to use and poses no significant risk to human subjects. The investigators will measure this through echocardiographic measures including pulsatility in different aspects of the Fontan circuit including IVC, hepatic veins, the Fontan conduit, and pulmonary arteries and aortic blood flow measurements. After demonstration of how to use the device appropriately, the investigators will have patients use the device after their clinical echocardiogram for their clinic appointment. The investigators will ask them to use the device at home 3-4 times a day for 10-15 mins and have them return in approximately 4 weeks to have another echocardiogram done with the same measurements.
This study will validate a coordinate-based 3-dimensional echocardiographic technique for the assessment of cardiac size and function in children and young adults with functional single ventricles.
In this three-party collaboration, which includes The Texas Center for Pediatric and Congenital Heart Disease, the Value Institute for Health and Care, and the Design Institute for Health, all from University of Texas (UT) Dell Medical School, the investigators will explore how patient insights, data, storytelling, ideation, and prototyping can be employed to design a care delivery solution that enables patient outcome improvement. The goal of this project is to understand the life journey of patients with single-ventricle congenital heart disease and understand areas of that journey that can be improved. As such, the project intentionally does not start with a hypothesis, but instead seeks to gain insight of single-ventricle patients' care journey and based on that understanding identify opportunities for improvement.
This is a prospective, multi-center, open-label, PK and safety profile study of enteral digoxin in children \<6 months old at time of enrollment, post-surgical or hybrid stage 1 palliation, but prior to surgical stage 2 palliation.
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.
The purpose of this study is to provide a rich description of the parental perspectives of utilizing a mHealth application for data transfer during the single ventricle interstage monitoring period. A gap in the body of knowledge of single ventricle cardiac disease interstage will be explored with these research questions: 1) How do parents of children (\< 5 years of age) with single ventricle disease describe their experiences using a mHealth application during the interstage period (first 6-12 months of child's life)? And 2) How do these experiences influence their decision making for use of the mHealth application?
Patients under the age of 5, with a diagnosis of hypoplastic left heart syndrome (HLHS), unbalanced atrioventricular canal (uAVC), or borderline left heart who are undergoing staged LV recruitment following bidirectional Glenn (BDG) or undergoing BDG with plans for LV recruitment will be considered for enrollment in this study. Those patients enrolled in the study will be randomized to either the experimental arm or control arm of the study. Those patients randomized to the experimental arm will receive mesenchymal precursor cells (MPCs) injected directly into the LV endocardium during their LV recruitment or BDG procedure. Those patients randomized to the control arm will receive normal standard of care during their procedure with no injection of MPCs. It is believed that injection of MPCs will help improve the chances of those patients with single ventricle or borderline left ventricle being converted to biventricular circulation which could improve quality of life and longevity over palliation.
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.
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).
Patients 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).
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.
This study seeks to determine cerebral blood flow changes in single ventricle patients during staged surgical reconstruction as compared with normal children. Two general groups of single ventricle patients will be recruited for this study, corresponding to the two approaches used. An aged-match group of healthy subjects will be included as a control.
Purpose: To evaluate the pharmacokinetics, bioavailability and hemodynamic efficacy of ambrisentan after Fontan surgical palliation of single ventricle heart defects. Study activities and population group: Children undergoing Fontan surgical palliation for single ventricle defects will be eligible for the study. Up to 20 subjects will be enrolled (16 ambrisentan, 4 placebo) and will receive 3 days (3 doses) of ambrisentan starting on post-operative day #1 upon returning from the operating room. Ambrisentan plasma levels will be obtained at specified time points during treatment. Post-operative monitoring lines will be used to measure effects of ambrisentan on hemodynamics and pulmonary / systemic endothelial function.
The 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).
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.
Patients with single ventricle anatomy undergo staged surgical palliation. The result is an "in series" circulation with pulmonary blood flow and cardiac output directly related to pulmonary vascular resistance. While surgical outcomes have improved, the physiology of the single ventricle palliation results in continued long term attrition. Elevated pulmonary vascular resistance and impaired systemic ventricular function are important risk factors for failure of single ventricle palliation. Sildenafil is a pulmonary vasodilator and has been shown to improve cardiac contractility in the pressure overloaded right ventricle. The investigators will assess the safety, pharmacokinetics and hemodynamic efficacy of sildenafil in single ventricle patients following stage II and III surgical palliation.
Protein Losing Enteropathy (PLE) is a serious medical condition that may develop in children and adults with congenital heart disease for which a palliative procedure known as the "Fontan procedure" has been performed. The loss of serum proteins into the gastrointestinal tract that is associated with PLE can cause serious symptoms and life-threatening complications. A number of clinical studies have suggested that heparin administration can have clinical benefit in children with PLE, however the risk of bleeding associated with the administration of heparin is an important concern and commonly limits its administration. ODSH is a desulfated heparin with minimal anticoagulation properties but which, in pre-clinical studies, appears to have the potential to replace heparin and greatly reduce the risk of bleeding. This open label study is to assess the safety and evidence of therapeutic effect of the administration of ODSH as a 4-day continuous intravenous infusion in patients with an exacerbation of their PLE.
The 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.
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.
The objective of this research proposal is to perform an ongoing single institution prospective clinical trial examining the overall clinical outcome of children with single ventricle physiology.
Congenital heart disease affects 1 in 100 newborn babies each year and more than 2,000,000 Americans have a congenital heart defect. One common defect treated at Children's Healthcare of Atlanta at Egleston is single ventricle heart. Due to these overwhelming numbers, the use of diagnostic imaging technology to assess these defects and heart function is an important step in the evolving care of this patient group.
This study will assess 75-100 patients for feeding issues following surgery for single ventricle.
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.
The normal heart has four chambers. There are two upper (or filling) chambers and are called the atrium. There are two lower (or pumping) chambers and are called the ventricles. One of the ventricles pumps blood to the lungs to get oxygen. This oxygenated blood is returned to the heart then pumped to the rest of the body by the other ventricle. Sometimes babies are born with heart defects that only allow one of the lower chambers (ventricle) to work properly. This means that the one ventricle must pump blood to both the lungs and to the rest of the body. Babies born with this defect must undergo multiple surgeries, the first of which is usually done during their first week of life. There is a machine that is FDA approved that can measure how much oxygen is delivered to the brain. This non-invasive (outside the body) machine uses Near-infrared spectroscopy (NIRS), which is similar technology used in pulse oximetry and is routinely used to measure the level of oxygen in blood. At Children's Healthcare of Atlanta, we typically place the NIRS machine on the baby in the Cardiac Intensive Care Unit immediately after their first surgery to monitor oxygen delivery to the brain. The monitor has proven to be accurate in this situation. We now want to know if we can use this monitor to assess oxygen delivery to the brain before and during a routine heart catheterization prior to the child's second surgery. Once consent is obtained, we will place a probe on the child's forehead during the pre-catheterization visit and obtain readings for 5 minutes. The probes are about the size of a quarter and are self-stick (they look like the kind of leads used to measure the babies heart rate \[EKG\]). The machine will be disconnected for the rest of the Pre-catheterization visit, however the probes will stay on the child's forehead. Once the child is in the catheterization suite, the machine will be reconnected. A sheath or hollow tube is routinely placed in a blood vessel in the child's neck or groin for the catheterization. The doctor would place a special catheter in this sheath that measures oxygen levels in blood continuously. This monitor would be calibrated by using the results of a blood sample that is routinely drawn from the child's IV. During the same sampling, an additional 2cc's of blood would be collected to measure the lactate level. The lactate level is an indicator of how well the child is using oxygen. Once this monitor is calibrated, data will be collected for 5 minutes to compare it with the NIRS machine. The special catheter will be removed after the 5-minute time period, and the catheterization will proceed as usual. The NIRS probes will stay on the child's forehead during the entire catheterization with the data documented. At the end of the catheterization, the probes will be removed.
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.
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
This trial will evaluate the efficacy and safety of the modified Blalock-Taussig shunt (MBTS) compared to the right ventricle to pulmonary artery (RV-to-PA) shunt; compare the effect of the MBTS to that of the RV-to-PA shunt on the incidence of death or cardiac transplantation at 12 months post randomization; and compare the effect of the two shunts on intensive care unit (ICU) morbidity, unintended cardiovascular interventional procedures, right ventricular function, tricuspid valve regurgitation, pulmonary artery growth, and neurodevelopmental outcome.
This study will evaluate the efficacy and safety of administering an angiotensin converting enzyme inhibitor (ACE-I) (enalapril) to infants with a functional single ventricle. The study will also compare the effect of ACE-I therapy to placebo on somatic growth and compare the effect of ACE-I therapy to placebo on signs and symptoms of heart failure, neurodevelopmental and functional status, ventricular geometry, function, and atrioventricular (AV) valve regurgitation. In addition, the study will determine the relationship between genetic polymorphisms linked to ventricular hypertrophy (enlarged heart) and the response to ACE-I therapy and compare the incidence of adverse events in subjects treated with ACE-I with those in subjects treated with placebo.