Tetralogy of Fallot

Researchers are studying recovery after heart surgery for people with Complex Congenital Heart Disease (CCHD) to understand the main risk factors affecting recovery. The study also aims to build a comprehensive data platform to support CCHD care throughout a person's life and develop a China-specific quality control system to improve surgical outcomes and guide national health policies. The study includes two groups: a retrospective group of 2,494 patients who already had CCHD surgery and are in an existing national database, and a prospective group of 1,940 patients scheduled for surgery as part of related sub-studies. Data collected include pre-surgery tests, surgical details, care during and after surgery, and follow-up for up to one year. Researchers will combine data from both groups to build a new registry and quality control system. Participants will have their medical records analyzed or have data collected during their care, depending on their group. Researchers will monitor recovery and outcomes up to one year after surgery, using the combined data to identify risk factors and develop a surgical quality control system. The study spans up to 48 months, aiming to improve CCHD care across all ages in China.

Researchers are investigating whether an integrated prenatal and postnatal treatment model improves outcomes for newborns with critical congenital heart disease (CCHD) compared to traditional care. This study aims to see if early diagnosis during pregnancy, combined with coordinated delivery and surgical planning, can reduce preoperative complications, optimize surgery timing, lower postoperative mortality, and enhance heart healing, especially in cases involving the right heart system. The goal is to improve treatment capacity and create guidelines suited to China's healthcare needs. The integrated model involves diagnosing CCHD by ultrasound at 22 to 26 weeks of pregnancy, transferring the mother within the hospital system before birth, and planning surgical care after multidisciplinary discussion. Newborns are transferred to the pediatric heart center immediately after birth for either urgent or elective surgery depending on their condition. Traditional care involves transferring newborns from outside hospitals with routine interventions. The study compares these two approaches by following patients from birth through surgery and afterward. Participants will be monitored during hospitalization, with evaluations of heart function, metabolic status, and survival rates over up to five years post-discharge. Researchers will assess 30-day postoperative mortality as the primary outcome and also look at heart failure before surgery, the proportion receiving integrated care, and long-term survival and reoperation rates. This real-world study uses hospital data from multiple sites to validate if the integrated prenatal-postnatal model offers better outcomes for newborns with CCHD.

Pediatric patients undergoing cardiac surgery, particularly those with ventricular septal defect or tetrology of Fallot, experience significant physiological changes during surgery and cardiopulmonary bypass (CPB). Traditional monitoring focuses on large blood vessels, but this may not reflect the true state of smaller vessels. Researchers are evaluating a handheld device called the Cytocam incident dark-field (IDF) microcirculatory camera, which provides real-time video and data of the microvascular circulation in these patients. This observational study involves using the Cytocam device to monitor microvascular blood flow at specific times: after intubation in the operating room, 10 minutes after CPB starts, after the cross clamp is placed, hourly during CPB, after CPB ends, and every 4 hours during the first 48 hours in the intensive care unit. The study includes patients with cyanotic physiology related to tetrology of Fallot and those with acyanotic lesions undergoing ventricular septal defect repair. Participants will be monitored continuously using the Cytocam device during the surgical and recovery periods. Researchers will assess measures such as capillary density, microcirculatory flow index, tissue perfusion surrogates, and signs of acute kidney injury. Secondary outcomes include ventilator duration and adverse events. The study will follow patients for the first 48 hours after surgery to gather these data and understand microvascular changes during and after cardiac surgery.

Tetralogy of Fallot and double outlet right ventricle are congenital heart conditions that can lead to heart failure. Researchers are studying whether the medicine Propranolol can help increase the growth of new heart muscle cells in infants with these conditions. This study is a phase 1 randomized trial aiming to understand if Propranolol can reactivate heart muscle cell division and support normal heart growth in infants up to about 4 months old undergoing surgical repair. Participants will be randomly assigned to receive either Propranolol or a placebo starting at 1 month of age until 4 months or just before surgery. Propranolol will be given by parents in four daily doses based on the infant's weight. Additionally, patients will receive labeled N-thymidine by mouth for five days to help measure heart muscle cell growth. The study includes heart imaging using echocardiogram and cardiac MRI before and at surgery, as well as tissue and blood sample collection during surgery for detailed analysis. During the study, infants will have regular physical exams including vital signs and feeding and diaper diaries to monitor medication processing. Researchers will analyze heart tissue removed during surgery to measure new heart muscle cell growth using isotope mass spectrometry. The main outcome is cardiomyocyte division at surgery. Secondary outcomes include heart muscle size changes assessed by imaging and microscopy. Participants will be monitored from 1 month of age until surgery, with close follow-up and safety assessments during this time.

Researchers are studying the use of Cereset Research (CR), a device-based intervention, to improve autonomic cardiovascular regulation in people aged 11 and older who have symptoms of stress, anxiety, or insomnia. This exploratory, open-label study aims to assess how CR affects heart rate variability, baroreflex sensitivity, and blood pressure variability. Secondary goals include evaluating changes in self-reported symptoms such as insomnia, depression, anxiety, and stress, along with exploring impacts in subgroups with other conditions like traumatic brain injury or PTSD. Participants will receive between 4 and 12 sessions of CR, which delivers audible tones echoing brainwave activity through sensors placed on the scalp with eyes closed. The device uses a 64-bit processing system and switches between sensor pairs during sessions to reduce interruptions and session length. This single-arm trial does not use a control group, and participants continue their usual care throughout the study. Participants will be evaluated at enrollment and through follow-up visits up to 4 to 6 weeks after the final session. Assessments include physiological measures such as blood pressure and heart rate variability, plus questionnaires on sleep, mood, anxiety, stress, traumatic stress, and quality of life. Data will be analyzed to measure changes from baseline and to explore effects in participants with coexisting symptoms or conditions. The total study duration for each participant varies depending on the number of sessions and follow-up timing.

Researchers are evaluating the safety and effectiveness of the Edwards Lifesciences SAPIEN 3 and SAPIEN 3 Ultra RESILIA Transcatheter Heart Valve (THV) Systems in patients who have a dysfunctional right ventricular outflow tract (RVOT) conduit or a previously implanted valve in the pulmonic position that requires medical intervention. This study focuses on those with pulmonary valve dysfunction, a complex congenital heart defect, pulmonary valve insufficiency, or pulmonary valve degeneration. Participants will undergo transcatheter pulmonary valve replacement (TPVR) using either the SAPIEN 3 or SAPIEN 3 Ultra RESILIA THV devices placed in the pulmonic position. The study includes different groups based on valve type and prior valve implantation status. The procedure is designed as a non-randomized intervention. During the study, participants will be closely monitored for valve function and potential complications. Assessments include measuring THV dysfunction at one year, device success shortly after discharge, heart function and valve performance at six months, and safety events such as vascular complications, bleeding, infection, and mortality within one year. Study involvement may last up to one year with regular follow-up visits for evaluations and safety monitoring.

Tetralogy of Fallot (ToF) is a congenital heart defect characterized by four major features, including obstruction of the right ventricular outflow tract. About 25 children in Denmark are born with ToF each year, and corrective surgery is typically done within the first year of life. Many patients require enlargement of the outflow tract with a patch, which can lead to severe pulmonary regurgitation and later complications like right ventricular dysfunction and arrhythmias. This trial aims to evaluate the long-term outcomes after initial ToF repair and to determine the best timing for pulmonary valve replacement in patients with free pulmonary regurgitation. The study will enroll 500 patients in a multicenter, cross-sectional evaluation, with 120 patients who have free pulmonary regurgitation randomized into two groups. One group will undergo early pulmonary valve replacement within 3 months after randomization, while the other will have the procedure later when they meet current European guideline criteria. The valve replacement involves surgical implantation of an adult-sized homograft or Contegra graft under cardiopulmonary bypass through a sternotomy. Participants will be followed for at least 10 years to assess long-term safety and effectiveness. Participants will be monitored regularly with yearly assessments including heart function measurements by MRI, exercise testing, arrhythmia monitoring, quality of life evaluations, and health system contacts. Primary outcomes include right ventricular end-diastolic volume indexed to body surface area and mortality rates measured 3 years after randomization. Secondary outcomes cover additional cardiac function parameters, complication rates after surgery, stroke and mortality rates, and quality of life changes over 10 years. The study also tracks valve deterioration and other clinical events to gain comprehensive information on the impact of early versus later re-valving.

This research aims to evaluate the effects of steroids on the early postoperative inflammatory response in children undergoing elective congenital heart surgery using cardiopulmonary bypass (CPB). The study focuses on pediatric patients with cyanotic and acyanotic heart disease who require CPB during their first cardiac surgery. The trial is a phase 4 randomized controlled study designed to monitor inflammatory markers and clinical outcomes related to steroid use in this setting. Participants will receive either intravenous methylprednisolone at a dose of 30 mg/kg (up to 500 mg) or intravenous normal saline as a placebo. These treatments are administered during the surgery involving CPB. The study includes two groups: an experimental group receiving methylprednisolone and a placebo comparator group receiving saline. The trial uses triple masking to ensure unbiased evaluation. During the study, blood samples will be collected before the first dose, immediately after protamine infusion, and at 24 and 48 hours following CPB. These samples will measure serum levels of interleukin-6, C-reactive protein (CRP), and creatinine to assess inflammatory and kidney function responses. Participants will be monitored closely in the cardiac intensive care unit during the initial 24 hours and followed up as per protocol. The total duration of participation varies based on these assessments and clinical care needs.

Researchers are studying the effects of percutaneous interventions for branch pulmonary artery (PA) stenosis on exercise capacity in patients with dextro transposition of the great arteries (d-TGA), Tetralogy of Fallot (ToF), and Truncus Arteriosus (TA). This randomized controlled trial also aims to assess the impact of these interventions on right ventricular (RV) function and to identify early markers to improve the timing of treatment. The study responds to the need for clearer guidelines on when to perform these interventions, as current knowledge about their effects on exercise capacity and heart function is limited. Participants are divided into two groups: one undergoing percutaneous stent placement for PA stenosis and a control group receiving conservative management with potential delayed intervention. Both groups will receive standard care evaluations, including transthoracic echocardiogram (TTE), cardiopulmonary exercise testing (CPET), and cardiac magnetic resonance imaging (CMR). The interventional group will have additional low dose dobutamine stress MRI at certain centers and RV pressure measurements during the intervention. Quality of life questionnaires will be collected at baseline and shortly after intervention or corresponding time points in controls. Long-term follow-up with TTE, CPET, and CMR will occur within 2 to 4 years. During the study, participants will undergo examinations at baseline and approximately six months later to measure exercise capacity (VO2 max), RV function, and adaptation. Assessments include imaging, exercise testing, and questionnaires to evaluate heart performance and quality of life. Researchers will compare results between groups to determine the effects of the intervention. Safety and technical success will also be monitored through pressure and gradient measurements. The total study duration includes initial assessments, intervention or observation periods, and extended follow-up to understand long-term outcomes.

Researchers are studying individuals with repaired Tetralogy of Fallot (rTOF) who remain at risk for sudden cardiac death due to ventricular tachycardia (VT). This study aims to understand how transcatheter pulmonary valve replacement (TPVR) affects the heart's electrical pathways, specifically the right ventricular (RV) isthmuses, and to explore new technology for mapping heart electrical activity. The goal is to better identify and characterize areas at risk during TPVR procedures. Participants will undergo TPVR as part of their standard care. Before the valve is placed, a detailed 3-dimensional electroanatomic map of the RV will be created using a specialized high-density catheter and mapping system. This mapping includes overlaying prior imaging and assessing heart electrical activity during normal rhythm and paced heartbeats. No attempts will be made to induce arrhythmias or perform ablation during the procedure. After the procedure, participants will be followed for 10 years to track heart rhythm events such as ventricular tachycardia, ventricular fibrillation, ICD placement or shocks, and sudden cardiac arrest or death. Annual medical record reviews will support this long-term monitoring. The study measures the area of RV isthmuses affected by the valve immediately after TPVR and monitors arrhythmic events over time.