Clinical Research Directory

Evaluation of the GORE PV1 Device in Patients With Pulmonary Valve Dysfunction

This study will assess the safety and performance of the GORE PV1 Device for replacement of the pulmonary valve and reconstruction of the Right Ventricular Outflow Tract (RVOT).

Pathophysiologic Mechanism for Arrhythmias and Impaired Aerobic Capacity in Tetralogy of Fallot and Other Congenital Heart Diseases

This study is being done to determine the mechanism(s) contributing to the onset of symptoms (i.e. shortness of breath and/or palpitations) as well as changes in heart structure in patients with congenital heart disease (CHD)

High Intensity Interval Training in Patients With a Right Ventricle to Pulmonary Artery Conduit

The goal of this clinical trial is to learn if a specific type of exercise training (high intensity interval training) can improve exercise capacity in people with a congenital heart defect that required the creation of a new connection between the right ventricle and pulmonary artery. This includes people with a truncus arteriosus, pulmonary atresia with a ventricular septal defect or severe tetralogy of Fallot. This study focuses on people aged 12 to 45 years. The main questions it aims to answer are: * Can a 12-week home-based high intensity interval exercise training program increase the exercise capacity? * Can factors that predict whether or not the exercise training program can increase the exercise capacity in specific people be identified? Researchers will compare the results from the intervention group to the control group. Participants will be assigned to one of these two groups at inclusion. The control group will also receive the intervention, after the control period. Participants will: * Participate in a 12-week home-based exercise training program (3x30 minutes a week, digitally supervised); * Attend 2 or 3 study visits (which partially is standard care) (2 visits for the intervention group, 3 visits for the control group); * Each study visit includes: echocardiography, magnetic resonance imaging (MRI) of the heart, cardiopulmonary exercise testing (CPET), blood and feces sampling, and questionnaires on quality of life and physical activity.

PULSTA Transcatheter Pulmonary Valve Pre-Approval Study

The purpose of the study is to evaluate the safety and effectiveness of the PULSTA Transcatheter Pulmonary Valve (TPV) System for the treatment of congenital or acquired pulmonary valve stenosis and/or regurgitation who require pulmonary valve replacement.

Beta-blocker Administration for Cardiomyocyte Division

Heart failure is a common long-term complication in patients with congenital heart disease (CHD). Medical treatments to promote regeneration of new healthy heart muscle cells have the potential to provide new heart failure treatments for these patients. The development of such therapies is limited by the poor understanding of the ways in which heart muscles grow after birth. Investigators have learned that humans without heart disease generate new heart muscles cells up to the age of 20 years old and that this is decreased in patients with congenital heart disease like Tetralogy of Fallot. Investigators are trying to determine if treatment with a medicine called Propranolol can increase heart muscle cell proliferation and, with that, normalize heart growth. Investigators will examine discarded heart muscle tissue that is obtained during surgery for the presence of new heart muscle cells. Propranolol is approved by the Food and Drug Administration (FDA) to treat a certain kind of benign tumor in infants (hemangioma), but it is not currently approved by the FDA to increase heart muscle growth.

ALTERRA Post-Approval Study

This study will monitor device performance and outcomes in subjects undergoing implantation of the Edwards SAPIEN 3 Transcatheter Pulmonary Valve System with Alterra Adaptive Prestent in the post-approval setting

ALTERRA: SAPIEN 3 THV With the Alterra Adaptive Prestent

To demonstrate the safety and effectiveness of the Edwards Alterra Adaptive Prestent in conjunction with the Edwards SAPIEN 3 Transcatheter Heart Valve (THV) System in subjects with a dysfunctional right ventricular outflow tract/pulmonary valve (RVOT/PV) who are indicated for treatment of pulmonary regurgitation (PR). Following completion of enrollment, subjects will be eligible for enrollment in the continued access phase of the trial.

Role of 3D Echocardiography Knowledge-based Reconstruction in ACHD

The main objective of this study is to evaluate the reproducibility and accuracy of TTE-3DKBR in assessing RV volume and EF compared to the reference method of CMR in a group of ACHD patients with known moderate or severe PR after ToF repair. As part of the validation study, the reproducibility of the TTE-3DKBR in assessing RV volume and EF will also be tested in a group of healthy adult volunteers. Additionally, TTE-3DKBR evaluation will be compared in both groups to the conventional 2D echocardiography measurements used in routine clinical practice, such as tricuspid annular plane systolic excursion (TAPSE), tissue Doppler imaging (TDI), fractional area change (FAC), and global longitudinal strain (GLS). Another objective of the study is also to evaluate the effects of chronic RV volume overload with an interval of one-year follow-up using TTE-3DKBR and compare it with the conventional non-geometric echocardiography measurements, particularly with GLS. The study will end when the required number of patients have been enrolled and when the last subject undergoes the research dataset acquisition. The study may be terminated prematurely if it becomes apparent that the recruitment target cannot be met within the projected recruitment phase.

NIRS in Congenital Heart Defects - Correlation With Echocardiography

Neonatal patients with congenital heart defects (CHD) have changing physiology in the context of transitional period. Patients with CHD are at risk of low perfusion status or abnormal pulmonary blood flow. Near infrared spectroscopy has been used in neonatal intensive care units (NICU) to measure end-organ perfusion. The investigator plan on monitoring newborns with CHD admitted to the NICU with NIRS and echocardiography during the first week of life and correlate measures of perfusion from Dopplers to cerebral and renal NIRS.

Molecular and Cellular Characterization of Cardiac Tissue in Postnatal Development

The study team will use small pieces of human hearts which are removed as part of a required surgical procedure to study different objectives. One of the objective is how calcium ions pass through the membrane of heart cells in order to tell the heart cell how much force to contract with when the heart beats. Investigators will also study the proteins and RNA of these pieces to determine how the newborn heart cells control their force of contraction differently from adult heart cells. Investigators hypothesize that infant hearts have different regulation of calcium entry than adult hearts. The study team also wants to study combinations of 3D cardiac spheres with multiple environmental cues that can improve functional and metabolic maturation of Human pluripotent stem cell-derived cardiomyocytes (hPSC-CMs) and generate a more clinically relevant cell model.

Effects Branch PA Stenting d-TGA, ToF and TA

The goal of this randomized controlled trial is to identify the effects of percutaneous interventions for branch PA stenosis on exercise capacity in patients with d-TGA, ToF and TA. The main question\[s\] it aims to answer are: The primary study objective is to identify the effects of percutaneous interventions for branch PA stenosis on exercise capacity in patients with d-TGA, ToF and TA. The secondary objectives are 1) to assess the effects of percutaneous interventions for branch PA stenosis on RV function and 2) to define early markers for RV function and adaptation to improve timing of these interventions. Participants will undergo the same series of examinations at baseline and approximately 6 months follow-up (within 6 week time-range) as part of standard care: conventional transthoracic echocardiogram (TTE), cardiopulmonary exercise testing (CPET) and conventional Cardiac Magnetic Resonance (CMR) including a low dose dobutamine stress MRI to assess RV functional reserve. The low dose dobutamine stress MRI will be performed in the interventional group from the UMC Utrecht/WKZ and Erasmus MC because the LUMC and AUMC do not have a suitable infrastructure for the low dose dobutamine stress MRI and this cannot be achieved throughout the duration of this study. The baseline CMR in the interventional group will be performed as close as possible prior to the intervention but maximal 4 weeks prior to the intervention. In addition, the intervention group will undergo standard RV pressure measurements during the intervention. Quality of life (QoL) questionnaires will be obtained at baseline and 2 weeks post intervention (intervention group) or a similar time range in the control group, which is based on experts opinion. TTE, CPET and conventional CMR will be performed within 2-4 years follow-up to assess the long-term effects of percutaneous PA interventions. Researchers will compare the difference in VO2 max (% predicted) between the interventional group (TGA, ToF or TA patients with a class II indication for a PA intervention who will undergo a percutaneous intervention for a PA stenosis) and the control group (TGA, ToF or TA patients with a class II indication for a PA intervention who will undergo conservative management)

Repaired Tetralogy of Fallot Italian Registry

The CMR/CT WG of the Italian pediatric cardiology society set up a multi-center observational clinical database of repaired-TOF evaluated. This registry will enroll prospectively patients evaluated by CMR for clinical indication in most of the CHD Italian centers. Data collection will include surgical history, clinical data, imaging data, and also adverse cardiac events for a period of 6 years.

Repaired Tetralogy of Fallot: Risc Score From MRI & Clinical Data to Predict the Need for Change of Treatment

Patient with Tetralogy of Fallot need live-long surveillance after initial operative correction (rTOF). MRI is an established and important component of the standardized follow-up in this patient population. A new score will be used to summarize the multitude of MRI-based parameters into a single number. Individual modificators are assigned to the components of the score. The value of each modificatory will be determined as a result of this feasibility trial. This new compound score is designed to anticipate any deterioration of cardiac function, arrhythmias or sudden cardiac death. This feasibility study is the first step to establish this score and find initial values for the modifies that are assigned to the more than 40 biomarkers from MRI, clinical examinations and serum parameters that are included in this new score.

Canadian Adult Congenital Heart Disease Intervention Registry

The ACHDi Registry study will create a foundational database for adult congenital heart disease interventions. This Pan-Canadian Registry will collect clinical and patient-reported information that will enable the evaluation of care processes and outcomes in five most common ACHDi interventions by enabling prospective and retrospective registry-based studies to answer important clinical practice and policy-relevant questions.

Early Versus Later Re-valving in Tetralogy of Fallot With Free Pulmonary Regurgitation

Tetralogy of Fallot (ToF) is a congenital heart defect with four major features including right ventricular outflow tract obstruction. About 25 children are born with this condition in Denmark every year. Corrective surgery is usually performed within the first year. In 50 % of patients, enlargement with a patch is necessary to achieve relief of the outflow tract obstruction. This however results in severe pulmonary regurgitation, which eventually leads to volume overload, right ventricular dysfunction and arrhythmia. To avoid these late complications, pulmonary valve replacement with a prosthesis if performed when patients meet the current guideline criteria. Most patients meet the guideline criteria for revalving when they are between 20 and 30 years of age. The current guidelines however, are based solely on retrospective studies and novel research reveals that in more than 50 % of patients who are treated according to current practice, right ventricular volumes and function as well as exercise capacity and burden of arrhythmia do not normalize or improve. 500 patients with ToF will be enrolled in a multicentre, cross-sectional study, which will yield information about the long-term outcomes after initial repair of ToF, as well as suggestions about the optimal timing for re-valving. Among patients included in the cross-sectional study, 120 patients with free pulmonary regurgitation, will be randomized evenly for early or later re-valving with at least 10-years of follow-up, for evaluation of long-term efficacy and safety of early re-valving.

French National Registry of Patients With Tetralogy of Fallot and Implantable Cardioverter Defibrillator

National french registry of patients with tetralogy of Fallot and implantable cardioverter defibrillator.