View clinical trials related to Single-ventricle.
Filter by:This is an investigator initiated, prospective, single-center, double-blinded, randomized, placebo-controlled trial of post-operative low dose vasopressin infusions as an early treatment of low systemic perfusion in pediatric patients following Fontan 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.
This study evaluates the effect of carvedilol in patients who have undergone a Fontan heart operation. All participants will receive carvedilol and placebo for 12 weeks. Exercise tests will be performed at the end of each 12 week period.
Introduction: The Fontan operation is the surgical treatment in most patients with either anatomic or functional single ventricles. In this operation, the subpulmonary ventricle is bypassed, connecting the systemic veins directly to the pulmonary arteries. The lack of a subpulmonary ventricle is associated with a nonpulsatile pulmonary flow and triggers a sequence of adaptive mechanisms along the life of these patients. The most frequent consequence of these adaptative mechanisms is the reduction in functional capacity, objectively measured by the decrease in peak oxygen consumption (VO2). So, cardiovascular and pulmonary functioning and skeletal muscle alterations can explain exercise intolerance in these patients. Objectives: 1. To compare the cardiovascular, pulmonary, and musculoskeletal system variables in clinically stable Fontan patients with Healthy subjects; 2. To correlate the variables of the cardiovascular, pulmonary, and skeletal muscle with the functional capacity in Fontan patients; 3. To identify predictors of low functional capacity in this population; 4. To evaluate four-month aerobic exercise and inspiratory muscle training on functional capacity, pulmonary function, and autonomic control in patients after Fontan operation and compare to the group with no exercise training. Methods: All subjects were submitted to cardiovascular, pulmonary, and skeletal muscle evaluation at baseline to perform a cross-sectional study comparing Fontan Patients (FP) with Healthy Controls (HC). In addition, the FP accepted to participate in the longitudinal clinical trial to evaluate exercise programs were randomized into three groups: Aerobic Exercise Training (AET), Inspiratory Muscle Training (IMT), and Non-exercise Training Group (NET, a control group). All patient groups (AET, IMT, NET) were reassessed after four months of training or under usual care. Expected Outcomes: This study expects to demonstrate that impaired pulmonary function, altered neurovascular control, and reduced skeletal muscle could be an additional potential mechanism for reducing functional capacity in clinically stable Fontan patients. And this impairment could be diminished by exercise training, enhancing physical capacity, and exercise tolerance.
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.
The purpose of this protocol is to measure the relaxation of the heart in subjects with single ventricles who have undergone the surgical Fontan procedure. We will do this by measuring relaxation with MRI, echocardiography, and cardiac catheterization and compare to blood levels that measure heart scarring. We will also measure relaxation before and after boluses of intravenous (IV) fluids to see if the relaxation changes when there is more fluid in the heart. Measurements of heart relaxation will be obtained from the MRI, echocardiogram, and cardiac catheterization for each patient and compared to blood markers of heart scarring. We aim to compare all of these measurements to see if we can accurately identify heart scarring and, if present, how much it correlates with impaired heart relaxation.
This pilot study is aimed at assessing the short-term effects of Tadalafil on the hemodynamic response to exercise and exercise capacity in patients with Fontan circulation. Data regarding effect size and drug tolerability will be used in the design of a randomized multicenter trial. The long-term goal of this investigation is to systematically evaluate the effect of tadalafil therapy on exercise performance, quality of life, and delay of functional deterioration in patients with single ventricle physiology.
Hypoplastic left heart syndrome (HLHS) and related anomalies involved a single ventricle are characterized by hypoplasia of the left heart and the aorta with compromised systemic cardiac output. Infants with the syndrome generally undergo a staged surgical approach in view of an ultimate Fontan procedure. Although long-term survival in patients with HLHS and related single ventricle physiology has improved markedly with advances in medical and surgical therapies, a growing number of infants will ultimately require heart transplantation for end-stage heart failure due to several potential disadvantages include a negative effect on right ventricular function, arrhythmia, additional volume load via regurgitation from the nonvalved shunt, and impaired growth of the pulmonary artery. Risk factors for poor outcome of heart transplantation with HLHS and single ventricle physiology are older age at transplantation and previous Fontan operation. New strategies are needed to improve the underlying transplant risks proper for the Fontan failure patients. Emerging evidence suggests that heart-derived stem/progenitor cells can be used to improved cardiac function in patients with ischemic heart disease. In this trial, the investigators aimed to test the safety and feasibility of intracoronary injection of autologous cardiac progenitor cells in patients with HLHS and related single ventricle anomalies and that could improve ventricular function at 3 months' follow up.
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.