View clinical trials related to Univentricular Heart.
Filter by:The lymphatics regulate the interstitial fluid by removing excessive fluid. It represents an extremely important step in the prevention of edema. The Fontan-Kreutzer procedure has revolutionized the treatment of univentricular hearts. However, it is associated with severe complications such as protein-losing enteropathy (PLE) and peripheral edema that may involve the lymphatic circulation. Our hypothesis is that patients with a univentricular circulation have a reduced functionality of the lymphatic vasculature, which predisposes them to developing complications such as edema and PLE. The functional state of lymphatics is investigated using near infrared fluorescence imaging, NIRF. The anatomy is described using non-contrast MRI and the capillary filtration rate is measured using plethysmography.
Individuals who have undergone the Fontan procedure are uniquely dependent upon respiratory mechanics to support pulmonary blood flow (PBF). The investigators hypothesized that enhanced respiratory muscle function via inspiratory muscle training (IMT) would improve performance on cardiopulmonary exercise stress testing (CPET).
Patients that have undergone a Fontan procedure (surgical correction for single ventricle congenital heart disease) may develop a complication known as protein-losing enteropathy (PLE). Some studies suggest PLE is primarily caused by impaired lymph flow. Use of continuous dopamine infusion can improve PLE. Evidence suggests the effect of dopamine may be through its effect on lymphatic function. This observational study looks at markers of lymph flow and PLE symptoms after treatment using dopamine and other standard therapies during disease exacerbations.
The goal of the Fontan Imaging Biomarkers (FIB) study is to identify the associations of blood and urine biomarkers to imaging parameters of ventricular mechanics.
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.
Frailty is a complex biologic syndrome of diminished physiologic reserve that leads to decreased resistance to stressors and is associated with adverse health outcomes. The syndrome has been well studied in adults and is quantified by the Fried criteria, which are comprised of five components- slowness, weakness, self-reported exhaustion, shrinkage and diminished physical activity. The concept of frailty is novel in children. A study in young adult childhood cancer survivors demonstrated increased incidence of frailty in this population along with increased risk of morbidity and mortality. This suggests that frailty as a phenotype has relevance outside of the geriatric age group. Pediatric patients with single ventricle physiology, heart failure and pulmonary artery hypertension- all represent populations with significantly increased risk of mortality, morbidity and decreased quality of life. Currently, such patients are monitored outpatient by serial echocardiograms and blood work that only gives information about end organ damage. But there is no validated tool available to measure global infirmity in such children. Better understanding of the relevance and applicability of frailty in pediatrics may allow for identification of the most vulnerable pediatric cardiac patients and be of value in optimizing their clinical management and improving health outcomes.
The purpose of this study is to determine whether Vasopressin following the Fontan operation will decrease chest tube output and duration.
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).
The purpose of this study is to evaluate the efficacy and safety of intracoronary injection of JRM-001 after reconstructive surgery in pediatric patients with functional single ventricle
The purpose of this study is to test the efficacy of treprostinil (TRE) in the perioperative non-fenestrated, extracardiac Fontan patients in order to reduce duration of chest tube drainage (in days). The Fontan operation is performed for patients with single ventricle physiology as the final palliation to create a series circulation, with passive systemic venous return to the pulmonary arteries and the single ventricle solely providing systemic output. Patients undergoing extracardiac Fontan tend to have elevation of Fontan pressures immediately following the operation with inflammation from surgery requiring additional fluid administration to maintain blood pressure. Increased Fontan pressures and fluid overload lead to prolonged chest tube drainage. The hypothesis is that treprostinil, a prostacyclin drug that dilates the pulmonary arteries, will improve immediate postoperative Fontan pressures. Treprostinil is not FDA approved for this use. Anecdotally and in a small case series, prostacyclin therapy has been shown to assist in transitioning patients off nitric oxide. The investigators believe that this improvement in hemodynamics will decrease duration of chest tube drainage resulting in a shorter length of hospital stay.