Congenital Heart Disease

Adults living with congenital heart disease (CHD) commonly experience physical symptoms alongside emotional and social challenges, which can affect wellbeing and health-related quality of life. Access to psychological services varies, and evidence to guide the development and delivery of psychological interventions for this population is still emerging. Interoceptive-based approaches focus on increasing awareness of and responding adaptively to internal bodily sensations, such as breathing, heart rate and fatigue. These approaches have shown benefits in other long-term health conditions but have not yet been studied in adults with CHD. This study will evaluate the feasibility, acceptability, and preliminary effects of a newly adapted online, skills-based group programme for adults with CHD. Thirty-two participants will be recruited through the Scottish Adult Congenital Cardiac Service and randomly allocated to one of two cohorts: Cohort 1 (Immediate Intervention) and Cohort 2 (Delayed/Waitlist Intervention). The group programme will consist of eight online sessions delivered by a Principal Clinical Psychologist and a Trainee Clinical Psychologist. Weekly sessions will explore the relationship between interoception and key areas relevant to living with CHD, including emotions, behaviours, and physical symptoms. Group discussions and exercises will focus on increasing awareness and understanding of internal bodily sensations, developing strategies for responding to interoceptive cues and using bodily information to guide behaviours and everyday activities. Participants will complete online questionnaires before and after the intervention assessing interoception, anxiety, depression and health-related quality of life. Participants will also provide feedback regarding the acceptability and perceived usefulness of the programme. Participation is voluntary, and participants may withdraw at any time without affecting their clinical care. Appropriate emotional support will be available should participants experience distress related to the group materials or discussions. Findings from this feasibility randomised controlled trial will inform further refinement of the group programme, assess the acceptability and feasibility of study procedures, provide estimates of recruitment, retention and outcome variability, and support the design and sample size calculation of a future definitive trial. The findings may also contribute to the enhancement of psychological support for adults with CHD and be disseminated through scientific publications and conference presentations.

Advances in surgical and medical care have led to improved outcomes in patients with congenital heart disease (CHD). As a consequence, the majority of patients nowadays survives to adulthood (adults with CHD, that is, adult CHD \[ACHD\]) with good quality of life. Despite the surgical success, the morbidity and mortality of ACHD is higher than in the general population and is linked to the development of heart failure (HF) in adulthood. HF occurs in approximately 25% of patients with ACHD, even in those patients in whom the congenital mal-formation has been corrected successfully in childhood. The time course and presentation are heterogeneous owing to variable congenital malformation and limitation of treatment options. ACHD with an anatomic right ventricle as the systemic ventricle (e.g., atrial switch operation in patients with transposition of the great arteries \[TGAs\]) and those with a functional single ventricle (e.g., Fontan circulation) appear to be at higher risk of developing HF. Young age at initial corrective surgery-often in the first 2 years of life-and lack of specific medical therapies can contribute to a high and early demand for heart transplantation in patients with ACHD.

Wearable health technology, particularly smartwatches, has revolutionized personal health monitoring by enabling continuous, non-invasive tracking of various physiological parameters. The potential applications of these devices in cardiology are extensive, but it is crucial to validate their accuracy against established medical-grade devices. The Apple Watch Series 9 (Apple, USA) exemplifies the capabilities of modern smartwatches, offering a wide range of health tracking features. These include heart rate monitoring, detection of irregular heart rhythms suggestive of atrial fibrillation, electrocardiogram recording, blood oxygen level measurement, and comprehensive mobility metrics (including VO2 max, six-minute walk distance, walking speed, step length, double support time, and walking asymmetry). Many of these parameters are highly relevant for cardiovascular patients, potentially aiding in disease progression monitoring and adverse event prediction. However, there is a lack of comprehensive validation studies for these devices in specific cardiovascular disease populations. This investigator-initiated, prospective observational study will consist of two phases: a training phase which involves 50 participants and a prospective validation phase which involves 20 participants. The study aims to validate smartwatch-based assessments using medical-grade devices and techniques. The study will focus on adult patients with congenital heart disease, pulmonary hypertension, heart failure, and coronary artery disease, as well as athletes undergoing cardiovascular assessment. By comparing smartwatch data with established clinical measurements, we seek to determine the reliability and potential clinical utility of these wearable devices in diverse cardiovascular contexts. Anonymized smartwatch data collected during the two phases will be published to a publicly registry to facilitate further research. This research will help bridge the gap between consumer-grade wearable technology and clinical practice in cardiology.

The purpose of this study is to implement and externally validate an inpatient ML algorithm that combines pulse oximetry features for critical congenital heart disease (CCHD) screening.

Lay Summary This study tests two ways of measuring blood magnesium after heart surgery. Children who need heart surgery may have heart and kidney problems after surgery. The right amount of magnesium in blood reduces this risk. This study will test the best way to measure magnesium. This will let doctors choose the right dose of MgSO4. MgSO4 is a magnesium supplement. Taking MgSO4 after heart surgery helps children. For each child, it is best to personalize MgSO4 dose. This is based on the amount of magnesium in blood. This study will test two ways of personalizing MgSO4 dose. In the blood, there are two kinds of magnesium. Usually, blood magnesium tests measure both forms together. This does not say anything about active magnesium. This study will measure the two forms separately. Then, MgSO4 will be given based on either the active or whole magnesium. Measuring active magnesium is good. Active magnesium levels change faster than total. That means active magnesium tests may better protect children. Also, active magnesium has more of an impact on heart and kidney function. Focusing on the active form will help these organs stay healthy. To test how well the MgSO4 is working, heart and kidneys will be examined. After surgery, certain harmful heart rhythms can occur. The types and number of harmful rhythms will be studied. Kidney problems can also happen after heart surgery. Kidney health will be studied. To help understand how active magnesium works, further tests will be done. These tests will look for evidence of poor health in the cells that make up the heart, kidney, and blood.

The goal of this clinical trial is to learn whether a Nintendo Switch-based exergame physical activity program can help children and adolescents with congenital heart disease increase their physical activity. It will also learn about the safety, acceptability, and feasibility of this program. The study will include children and adolescents aged 8 to 18 years who have congenital heart disease, have received surgical or interventional treatment, and are clinically stable. The main questions it aims to answer are: Does the exergame-based program increase the amount of time participants spend in moderate-to-vigorous physical activity each day? Is the exergame-based program safe, acceptable, and feasible for children and adolescents with congenital heart disease? All participants will receive the same exergame-based physical activity program. Researchers will use different baseline observation periods to help understand whether changes in physical activity happen after the program starts. Participants will: Wear an activity monitor to measure daily physical activity. Complete a baseline observation period lasting 7, 14, or 21 days. Take part in a 12-week Nintendo Switch-based exergame physical activity program with guidance, goal setting, self-monitoring, feedback, and caregiver support. Complete a 2-week observation period after the program to see whether physical activity changes are maintained. Complete study assessments at screening, the end of baseline, week 4, week 8, week 12, and the end of the observation period. Researchers will also collect information on step counts, energy expenditure, self-reported physical activity, exercise capacity, quality of life, adherence, acceptability, and adverse events.

The purpose of this study is to compare the effect of high and ordinary energy density enteral nutrition for improving physical growth and brain cognitive development in infants with congenital heart disease after operation, as well as evaluate the safety of interventions.

The goal of this observational study is to gather more information on kidney oxygen levels in babies with a patent ductus arteriosus (PDA), and evaluate the relationships between kidney oxygen levels, PDA status and kidney injury. Researchers will do this by looking at ultrasound images of the heart, analyzing substances in the urine, and evaluating oxygen levels in the kidneys.

Congenital heart disease (CHD) is the leading cause of congenital malformations, representing 1% of live births. Progress in surgical care have led to the dramatic increase in the population of children and adults living with heart disease. As survival is no longer a concern, long-term outcomes have become the major public health issue. Prenatal diagnosis of CHD requiring open-heart surgery can be a traumatic event for expecting mothers and fathers. In the general population, maternal mental health distress is associated with fetal disturbances in the hypothalamic-adrenal-pituitary system axis, restricted intrauterine growth and adverse outcomes in the offspring. It is unknown whether prenatal maternal psychological distress have an impact on neurodevelopmental outcomes in CHD. Our national study seeks to (1) characterize the impact of prenatal maternal psychological distress on neurodevelopmental outcomes at age 1 for children with CHD who undergo neonatal open-heart surgery; (2) investigate the sociodemographic and medical determinants associated with prenatal maternal mental health of women carrying a foetus diagnosed with complex CHD; (3) explore the mediating role of prenatal risk factors (i.e., sociodemographic, medical and maternal coping mechanisms) in the association of prenatal maternal mental health (i.e., distress, anxiety and depression) and neurodevelopment in children with CHD; and (4) explore the impact of paternal or the co-parent's mental health impact on neurodevelopmental outcomes at age 1 in children with CHD. This study is a non-interventional, prospective, and longitudinal study of prenatal maternal mental health and subsequent child's neurodevelopmental and behavioural outcomes. It includes a follow-up period from the 3rd trimester of pregnancy until the child's first year of life. It will include children with a prenatally diagnosed heart defect requiring open-heart surgery within the first weeks of life. Understanding and preventing the neurodevelopmental sequelae of heart disease diagnosed in-utero is a public health priority.

Background: In Fontan Associated Liver Disease (FALD), congestion of blood in the liver causes cirrhosis. This condition can cause death. Researchers want to understand what triggers this process and find new treatments for it. Objective: To understand how long-term congestion of blood in the liver causes liver scarring that eventually leads to cirrhosis. Eligibility: People aged 18 and older who are at risk of developing FALD from the Fontan procedure. Design: Participants will be screened with: Medical history Physical exam Blood and urine tests Liver ultrasound. This uses sound waves to take pictures of the body. Participants will have an outpatient visit within 12 weeks after screening. Within 24 weeks later, they will have a 3-day hospital stay. About 2 weeks later, they will have a follow-up visit. Visits will include repeats of the screening tests and: Heart tests Stool collection Questionnaires MRI of the liver. Participants will lie on a bed that slides in and out of the scanner. They will receive a contrast agent injected into a vein. While in the scanner, they will also have an MRCP to view the bile ducts and the pancreatic duct. Fibroscan exam. This is an ultrasound that uses a special probe to look at the toughness of the liver. Upper endoscopy. This uses a thin scope to look inside the upper digestive tract. Liver biopsy. This will be taken through large vein in the neck or through the chest. Just before the biopsy, participants will have pressure measurements inside their liver. For this, a catheter will be inserted into a neck vein and guided into the liver.

The purpose of this clinical trial is to learn about the effects of a nursing support program designed for families of children aged 1 to 3 years who have undergone heart surgery. This program aims to improve parents' caregiving skills and support the healthy growth of their children. The primary questions the study aims to answer are: Does the nursing program positively affect the physical growth of children, such as their height and weight? Does the program improve children's scores on general development tests (Denver II)? Do parents' attitudes toward child feeding and their confidence in parenting skills increase? Researchers will divide participants into two groups: Experimental Group: Families who participate in the nursing education program and receive an informative booklet. Control Group: Families who only receive an informative booklet and continue with routine hospital follow-ups. The researchers will compare these two groups to see if participating in the nursing program leads to better results for the children's development and the families' skills. Participants will be asked to: Attend 5 different education and counseling sessions, starting one week before surgery and continuing until the child reaches 1 year of follow-up. Meet with the researcher at specific intervals (at 1, 2, 3, 6, 9, and 12 months) for one year to monitor the child's development (height, weight, head circumference) and skills. Complete surveys regarding child feeding attitudes and parenting skills at the 6th and 12th month follow-ups.

The goal of this study is to evaluate the impact of a clinical decision support system (CDSS) in children receiving mechanical ventilation (MV) after surgery for congenital heart disease (CHD). The main question it aims to answer is: -What is the impact of a CDSS designed to facilitate weaning and discontinuation of MV on the duration of MV in post-operative congenital cardiac surgery patients? Participants will be identified as eligible to initiate weaning from mechanical ventilation. Providers will decide whether or not to initiate weaning based on recommendations provided by the CDSS. Researchers will compare patients exposed to the CDSS with a historical cohort to see if the CDSS facilitated a decrease in MV duration.

Cardiovascular disease is the leading cause of death worldwide. Advanced cardiovascular imaging using Magnetic Resonance Imaging (MRI) has proven to be effective in providing gold standard myocardial tissue characterization. Moreover, the intrinsic advantage of MRI's lack of exposure to ionizing radiation is particularly beneficial. At the same time, blood work can be very useful in early detection of certain cardiomyopathy, such as amyloid. However, there is a lack of agreement of on which markers are the most sensitive. This multi-study will allow us the unique opportunity to form a more comprehensive understanding for various cardiovascular diseases. Our team has developed novel cardiac MRI techniques that leverages endogenous tissue properties to reveal a milieu of deep tissue phenotypes including myocardial inflammation, fibrosis, metabolism, and microstructural defects. Among these phenotypes, myocardial microstructure has proven to be most sensitive to early myocardial tissue damage and is predictive of myocardial regeneration. In this study, the investigators aim to further study the importance of cardiac microstructure revealed by MRI in patient and healthy population and compare this novel technology with conventional clinical biomarkers.

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.

The study includes patients with congenital heart disease (CHD), as well as their support persons and their providers, who are preparing to make the transition from pediatric to adult care for their CHD. The purpose of this study is to improve the tools available to help find doctors as patients enter adulthood. 200 people with CHD and support people will be enrolled.

Starlight Cardiovascular, Inc. is sponsoring a prospective, multi-center, study to evaluate safety and effectiveness of the Lifeline Ductus Arteriosus Stent System. The study device is a stent that is designed to maintain patency of the Ductus Arteriosus for children who need blood flow through that part of the heart.

This research study is being done to provide comparative data to the Mayo Clinic Adult Congenital Heart Disease Registry.

Due to successes in the last decades in pediatric heart surgery and cardiology, 90-95% of the children with congenital heart disease reach adult age.This results in an increasing number of adults or "grown-ups" with congenital heart disease (ACHD or GUCH patients) that require special health care organization and training programmes. Long term complications of these GUCH patients and optimum treatment strategies are still poorly known. The aim of this registry is to collect quantitative and qualitative data regarding GUCH patients treated in specialised centres in Switzerland.

The aim of this randomized interventional multi-center clinical trial is to determine whether a standardized lung volume optimization maneuver (LVOM), including PEEP titration, improves outcomes in children undergoing biventricular repair for congenital heart disease (CHD) with cardiopulmonary bypass. The primary hypothesis is that optimizing end-expiratory lung volume through a standardized PEEP titration maneuver improves cardiac performance and lung function. Secondary objectives are to evaluate whether this strategy reduces duration of mechanical ventilation, improves hemodynamics and ventilation-perfusion matching, and decreases the need for vasopressor support.

This study is a randomized controlled trial designed to evaluate the effects of high-calorie diets and specialized nutritional formulas on weight gain and clinical outcomes in underweight children aged 1 to 5 years with congenital heart disease (CHD). Children with CHD often experience growth failure due to increased energy needs and feeding difficulties, which can negatively impact their recovery, development, and overall health. Seventy-five children will be randomly assigned to one of three groups: a control group receiving a standard diet, a group receiving a high-calorie diet made from energy-dense foods, and a third group receiving both the high-calorie diet and a specialized high-calorie pediatric formula. The study will measure changes in weight, appetite, feeding tolerance, and other growth indicators over an 8-week period. The goal is to determine whether enhanced nutritional support can improve weight gain and health outcomes in this high-risk population.

This study has the goal to determine the best method of respiratory support following extubation after cardiac surgery (CS). After cardiac surgery for Congenital Heart Disease (CHD), patients remain intubated until the cardiac team determines it is safe for the patient to undergo a trial of extubation. Two common methods of respiratory support following extubation are High Flow Nasal Cannula (HFNC) and Non Invasive Positive Pressure Ventilation (NIPPV). There is currently a gap in data comparing High Flow Nasal Cannula and Non-Invasive Positive Pressure Ventilation in infants (age 0-1) in regard to extubation failure and overall outcomes. This study will monitor the health outcomes of 200 infants (0 - 1 year) with CHD following cardiac surgery in the Cardiac Intensive Care Unit (CICU) at Children's Healthcare of Atlanta (CHOA). This will be done by assigning the respiratory support method each child will receive following extubation after cardiac surgery. Health outcomes will be monitored until discharge or until the second instance of extubation failure. Both study arms are standard-of-care respiratory support methods in the CHOA CICU. The investigators aim to determine which of these two methods has fewer risk factors when used with infants.

Non-invasive tools for monitoring of course of disease are important and necessary in the treatment of pre-term/premature infants and sick neonates. For many years, the ultrasound group in Trondheim has been at the forefront in the development of new ultrasound technology for the diagnosis and monitoring of disease. Several methods previously developed in this research group are today widely used in hospitals around the world. In this project the investigators aim to test a new ultrasound technology that allows continuous monitoring of cerebral blood flow in sick neonates and pre-term children. This technology was CE-certified in October 2022, and in this project the investigators will test the CE-certified version with the newest available approved software.

This study will evaluate a virtual mental health parenting stepped-care intervention (I-InTERACT-North) to determine if the program works to improve positive parenting skills and child behaviour among families with children born with Congenital Heart Disease (CHD). Recruitment will target children ages 3-9 years old from SickKids. We will also evaluate the acceptability and feasibility of the program among children and families to inform future delivery and multi-site trials. Results will evaluate whether I-InTERACT-North can improve parenting and child behaviour in these families and inform future best clinical practices for this population.

The goal of this observational study is to learn about recovery after heart surgery for people with Complex Congenital Heart Disease (CCHD), build a comprehensive data platform for CCHD care across a person's whole life, and create a China-specific quality control system to improve CCHD surgical care. The main questions it aims to answer are: What are the main risk factors that affect how well people with CCHD recover after heart surgery? Can a whole life course, multi-dimensional data platform for CCHD care be built to support better clinical quality control? Can a China-specific system to evaluate and control the quality of CCHD heart surgery be developed to guide national health policies and improve patient outcomes? Participants in this study are people with CCHD who have had or will have heart surgery at participating medical centers. This includes two groups: A retrospective group: People who already had CCHD heart surgery and were included in the existing National Congenital Heart Disease Surgery Database. A prospective group: People who are scheduled to have CCHD heart surgery as part of related sub-studies of this project. People who are lost to follow-up or have more than 30% missing data will not be included. Participants will: For the retrospective group: Have their past medical records (from surgery and follow-up) analyzed by researchers to find risk factors linked to recovery after CCHD heart surgery. For the prospective group: Have their pre-surgery tests, surgery details, care during and after surgery, and follow-up data (including up to 1 year after surgery) collected as part of their regular medical care. Researchers will combine data from both groups into a new registry, work with a third-party committee to check data quality, and use this combined data to build a quality control and evaluation system for CCHD heart surgery. This system will help show the current state of CCHD care in China and guide national health decisions.

This is a single-center, prospective, randomized, open-label, parallel-controlled clinical study to evaluate the effectiveness of a large language model (LLM)-based intelligent health management assistant in the life-cycle health management of patients after cardiac surgery. A total of 500 adult patients who undergo cardiac surgery (including coronary artery bypass grafting, heart valve surgery, great vessel surgery, congenital heart disease correction, and other cardiac procedures) at Beijing Anzhen Hospital will be randomly assigned in a 1:1 ratio to an intervention group or a control group, stratified by age (\<65 vs ≥65 years) and surgery type. The intervention group will use the LLM-based mobile health management application in addition to standard postoperative care, while the control group will receive standard postoperative care alone. The application integrates multimodal clinical data into a personalized health profile and provides surgery-type-specific postoperative management recommendations, medication adherence reminders, complication early warning, and cardiac rehabilitation guidance. The primary outcome is the composite endpoint of major adverse cardiac and cerebrovascular events (MACCE), defined as all-cause death, non-fatal myocardial infarction, non-fatal stroke, or unplanned cardiovascular reoperation/reintervention, within 12 months after randomization. Secondary outcomes include health-related quality of life (EQ-5D-5L), cardiovascular rehospitalization rate, medication adherence (MMAS-8), postoperative complication rate, and cardiac rehabilitation achievement rate. Follow-up visits are scheduled at 1, 3, 6, 9, and 12 months post-randomization.