Repaired Tetralogy of Fallot Italian Registry
Tetralogy of FallotCardiac Death3 moreThe 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.
Prospective Evaluation of Programmed Ventricular Stimulation Before Pulmonary Valve Replacement...
Congenital Heart DiseaseFallot Tetralogy2 moreSevere pulmonary regurgitation is common in patients with Tetralogy of Fallot and results in progressive right ventricular dilatation and dysfunction. Pulmonary valve replacement is frequent in this population, and percutaneous procedures are increasing. Ventricular arrhythmias are a frequent late complication in patients with tetralogy of Fallot. The most common critical isthmus of ventricular tachycardias is between the pulmonary valve and the ventricular septal defect patch. While an electrophysiology study is sometimes performed in expert centers before surgical pulmonary valve replacement to guide a surgical ablation if needed, this approach is not recommended in current guidelines. An electrophysiology study should also be considered before percutaneous pulmonary valve replacement, as a part of the critical isthmus may be covered by the prosthetic pulmonary valve. Moreover, ablation after percutaneous pulmonary valve insertion exposes patients to the risks of traumatic valve or stent injury and infectious endocarditis. At present, reliable predictors to identify high-risk patients in whom an electrophysiology study should be performed before pulmonary valve replacement are lacking. The aim of this study is to assess prospectively the yield of systematic electrophysiology study and programmed ventricular stimulation before surgical and percutaneous pulmonary valve replacement in patients with tetralogy of Fallot.
Early Right Ventricular Function After Repair Of Tetralogy Of Fallot , An Evidence Based Study....
Tetralogy of FallotThis study aims to assessing right ventricular function early after surgical repair of tetralogy of Fallot and identifying the risk factors associated with development of RV dysfunction.
Pediatric REPlAcement of the PulmonaRy ValvE in Tetralogy of Fallot -
Tetralogy of FallotTetralogy of Fallot (TOF) is the most common cyanotic congenital heart defect with the vast majority of survivors of corrective surgery left with some degree of right ventricular (RV) volume overload due to pulmonary regurgitation (PR) which cause RV enlargement with right heart failure, diminished biventricular function, ventricular arrhythmia, sudden death and decreased exercise performance over time. Pulmonary valve replacement (PVR) has been thought to ameliorate these complications but the timing of replacement has yet to be determined with equipoise at the moment in this decision making process. As nearly all studies in this regard are retrospective with much less data in pediatric TOF than adults, this pilot trial sets the stage to create a prospective randomized trial in the teenage years.
Repaired Tetralogy of Fallot: Risc Score From MRI & Clinical Data to Predict the Need for Change...
Tetralogy of FallotCongenital Heart DiseasePatient 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.
The Effects of Eplerenone on Markers of Myocardial Fibrosis in Adult Congenital Heart Disease
Tetralogy of FallotTransposition of the Great Vessels With an Arterial Switch1 moreHypothesis: By blocking aldosterone signaling in patients with Tetralogy of Fallot, Transposition of the great vessels with a prior atrial switch, and single ventricle "Fontan" patients, incident heart failure will be delayed, symptoms of heart failure ameliorated, and risk of arrhythmias decreased through decreases in myocardial fibrosis. Half of enrolled patients will complete an SF-36 quality of life questionnaire, perform a 6 minute walk, and have blood drawn for biomarker analysis at enrollment, again after 3 months without therapy, after 6 months on therapy, then finally after 12 months of eplerenone therapy. Half of enrolled patients will have the 3 month drug free period at the end of 12 months on therapy. Patients will be randomly assigned to drug free period up front versus at the conclusion of the trial period. Eplerenone will be started at a dose of 25mg and titrated up to 50mg at 4 weeks if tolerated. Blood will be drawn for basic metabolic panel analysis at enrollment, 3 months, 4 months to allow for dose titration, and at 6 and 12 months for monitoring.
Exercise Training Strategies for Children With Repaired Tetralogy of Fallot
Tetralogy of FallotCongenital Heart Defects3 moreThe investigators will explore the feasibility and safety of two exercise interventions delivered both in the hospital and in participants' homes. Preteens and adolescents who have had repaired Tetralogy of Fallot (TOF), will be grouped by age and randomly assigned to either an aerobic or strength training exercise program supervised for 12 weeks. An exploratory aim of this study will be to examine changes in fitness level, muscle strength, muscle oxygen extraction and quality of life after the intervention, and compare these measures between groups. At the end of the study, participants and parents will be interviewed to assess their satisfaction and ideas for improvements in the program.
Effects of rhBNP in Pediatrics After Corrective Repair of Tetralogy Of Fallot
Congenital Heart DefectsTetralogy Of FallotThe purpose of this study is to determine the effects of rhRNP on urine output and hemodynamics following corrective repair of Tetralogy Of Fallot.
A Phase I Study of Dexmedetomidine Bolus and Infusion in Corrective Infant Cardiac Surgery: Safety...
Transposition of the Great ArteriesTetralogy of Fallot1 moreThe purpose of this Phase I study is to determine the safety of a drug called dexmedetomidine (DEX) as part of a balanced general anesthetic and sedative strategy for neonates and infants undergoing corrective cardiac surgery that requires the use of cardiopulmonary bypass for congenital cardiac problems. This study will also design and validate a dosing schema for the use of DEX as described above.
Pulmonary Artery Repair With Covered Stents
Pulmonary StenosisPulmonary Regurgitation1 moreThe Covered Cheatham-Platinum Stent (CCPS) is being study for repair of tears that occur in the pulmonary artery during dilation (enlargement) of a conduit (passageway) connecting the right ventricle of the heart to the pulmonary arteries. Patients undergoing replacement of their pulmonary valve by transcatheter technique Melody Valve) are at risk of developing such tears in the process of preparing the conduit to accept the new valve. In order to implant such a valve, the connection between the right ventricle and the pulmonary arteries often needs to be enlarged. High pressure balloons may be needed and these balloons can sometimes cause tears in or even rupture of the connecting conduit. Such tears can allow blood to flow into the chest and rarely this can lead to a life-threatening emergency. Experience suggests that such tears can be closed by implanting into the conduit a metallic stent with an outer covering, rebuilding the wall and allowing continuation of the valve implant.