Transposition of the Great Arteries

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 studying the use of sodium-glucose cotransporter 2 inhibitors (SGLT2i) in adults with congenital heart disease (ACHD), focusing on how these drugs are prescribed and their safety, tolerability, and possible benefits for heart failure-related outcomes. Heart failure is a leading cause of illness and death in this group, but treatment options are limited. SGLT2i are a new class of medications shown to help in conventional left ventricular heart failure and may work through multiple pathways to improve heart function. This international registry collects real-world data from adult ACHD patients who have been treated with any type and dose of SGLT2i. Data will be gathered from one year before starting the medication up to the most recent follow-up. There are no interventions or changes to care for participants since data collection uses routine clinical records. The registry aims to understand prescription patterns, side effects, discontinuation rates, and heart failure-related clinical and laboratory outcomes. Participants will contribute data from their medical records, including clinical parameters like weight, blood pressure, heart rate, oxygen saturation, laboratory values, echocardiography measurements, and exercise test results. Researchers will monitor safety and tolerability outcomes such as side effects, complications, and mortality over an average follow-up of one year. This study seeks to evaluate the impact of SGLT2i therapy on heart failure progression in ACHD patients through comprehensive real-world observation.

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.

Researchers are evaluating the impact of Cardiac Resynchronization Therapy (CRT) on functional capacity in adults with congenital heart disease and a systemic right ventricle (SRV). The study aims to provide the first evidence on CRT's effectiveness in patients with heart failure and SRV. This is a double-blind randomized cross-over trial involving 40 patients, with a follow-up period of 6 months. Participants who have heart failure with SRV and an implanted CRT device will be randomized to receive either an active CRT device (CRT ON) or an inactive device (CRT OFF) for 3 months. After this period, participants will switch to the other intervention for another 3 months. Two groups exist: one starts with CRT ON then switches to CRT OFF, and the other starts with CRT OFF then switches to CRT ON. During the study, participants will be monitored through various tests including the 6-minute walk test, peak oxygen uptake, and evaluation of heart function and symptoms. Quality of life, heart failure status, and device-related parameters will also be assessed. The study will track changes between the two 3-month treatment periods to determine the effects of CRT on functional capacity and other heart-related outcomes.

Children with chronic health conditions have a higher chance of developing heart and blood vessel diseases earlier than their peers due to faster aging of these organs. Experts recommend careful monitoring and early care, but the exact causes of this increased risk are not well understood. This research seeks to better understand why these children face greater cardiovascular risks and to support future prevention efforts. The study observes children and young adults aged 6 to 25 who have chronic conditions linked to higher cardiovascular risk. It uses non-invasive methods to check the health of large blood vessels like the carotid artery and small vessels in the retina. These assessments include measuring arterial stiffness, endothelial function of the brachial artery, and retinal vessel features, all done at the start and during yearly follow-ups. Participants will undergo regular evaluations including scans of blood vessels and retinal imaging to monitor changes over time. The study measures key indicators such as arterial stiffness and retinal vessel diameter to track early signs of vascular aging. This ongoing observation helps researchers identify those at risk before obvious heart or vascular disease appears. The study is sponsored by Semmelweis University and runs until 2029, involving close monitoring without intervention.

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.

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 investigating how abnormal blood flow regulation in the brains of fetuses with complex congenital heart disease (CCHD) relates to delays in neurodevelopment. The study also looks at how this relationship is influenced by patient factors like the type of heart defect, maternal-fetal environment, and social determinants of health in a diverse group of pregnant women. This study uses data from multiple centers and approaches pregnant women during their fetal cardiology visits. The study involves a single treatment group where pregnant women receive maternal hyperoxia (MH) therapy at around 28 weeks gestation during their scheduled fetal echocardiogram. MH involves breathing 100% oxygen through a face mask for 10 minutes, with imaging taken before, during, and after this oxygen exposure to observe changes in the fetal brain's blood flow. Following birth, neonates with congenital heart disease undergo a pre-operative neurobehavioral assessment using the Neonatal Intensive Care Unit Network Neurobehavioral Scale (NNNS). Participants will have their fetal brain blood flow measured via ultrasound at baseline, during MH, and after recovery. After birth, the neurobehavioral scale will be administered by certified therapists to evaluate attention and related functions within 30 days of life. Researchers will collect detailed patient and environmental information to analyze how these factors affect brain blood flow and neurodevelopment. The total study period includes prenatal evaluation and neonatal assessment within the first month of life, with no additional visits required beyond routine care.