Clinical Research Directory

Learning by Heart: The Effectiveness of an EF Training Program for Pre-schoolers With Critical CHD

Advances in prenatal and neonatal care have improved outcomes in children with critical congenital heart disease (CCHD). With the increase in survival, neurocognitive problems such as executive functioning (EF) impairments have become more apparent in these children. EF problems have cascading negative effects on a child's development. New insights in EF development suggest that in otherwise physically healthy young children, EF can be improved by training. In a pilot study funded by Stichting Hartekind, the investigators studied the feasibility of a personalized EF training program called 'Kleuter Extra' and the results were promising. Therefore, the current study will investigate the effectiveness of this program in 4-6-year-old children with CCHD. The researchers will also explore interactions between the parent-child relationship and EF development of the child as psychosocial difficulties in these children and their parent(s) and/or caretaker(s) may impact EF-development. If found effective, EF training for children with CCHD will improve their developmental outcome.

Effect of Sacubitril-Valsartan on Cardiac Structure and Function

The purpose of this study is to compare changes in cardiac structure and function, biomarkers, and patient reported outcomes between adults with congenital heart disease (ACHD) randomized to angiotensin receptor-neprilysin inhibitor (ARNI) therapy vs placebo.

UltraTrac for Cardiac Output Monitoring in Congenital Heart Disease

This pilot feasibility study will evaluate UltraTrac, an investigational noninvasive wearable device for continuous cardiac output monitoring, in patients with congenital heart disease. UltraTrac integrates impedance cardiography and carotid Doppler ultrasound to estimate cardiac output. Participants undergoing clinically indicated cardiac catheterization, cardiac MRI, or cardiac intensive care unit monitoring will have UltraTrac measurements collected during routine clinical care. UltraTrac measurements will be compared with reference standard or clinical hemodynamic measurements, including thermodilution, direct Fick method, cardiac magnetic resonance imaging-derived cardiac output, and clinical hemodynamic assessments before and after vasoactive medication changes. UltraTrac data will not be used to guide clinical decision-making.

Echocardiographic Assessment of Valvular Dysfunction in Children With CHD

This cross-sectional study aims to assess the type and severity of valvular dysfunction by echocardiography in children with congenital heart disease.

Atrial Late Gadolinium Enhancement in Patients With Repaired Congenital Heart Disease

In this research study the investigators want to learn more about how well the investigators can visualize scar tissue in the heart by MRI. In patients with congenital heart disease who need a procedure in the electrophysiology laboratory, how the MRI findings match the findings in the electrophysiology laboratory is not known. This study works to answer these questions. Participants will undergo a cardiac MRI as part of the routine clinical care that was ordered by their doctors and additional imaging by cardiac MRI will be performed.

The Genetic Basis of Congenital Heart Disease in Africa

Recent advances in genomic techniques are making possible a new wave of genetic discovery in congenital heart disease (CHD). Existing data suggests that CHD occur in Sub-Saharan Africa at frequencies similar to the rest of the world. In this application, we propose to utilize the unique advantages of Sub-Saharan Africa - a combination of the most genetically diverse populations in the world and of diminished environmental background effects (i.e. low prevalence of smoking, alcohol abuse, obesity in comparison to western countries) - to better understand the genetic basis for congenital heart disease. We will couple next generation genomic techniques with more traditional gene discovery methods to investigate CHD in two African countries: Uganda and Nigeria. The inclusion of syndromic and non-syndromic CHD observed in these populations as well as careful phenotyping (including echocardiography) will greatly enhance our potential to provide insight into the genetic architecture of CHD in African populations. To accomplish this, we plan to enroll families, in whom members have congenital heart malformations consistent with an error of early human development in our research protocol. Patients will be enrolled at the Uganda Heart Institute in Kampala, Uganda, and at the Department of Pediatrics, College of Medicine, University of Lagos, Nigeria, with the potential to include other African sites. High throughput genomic studies will be done at the NIH.

The Effect of Telerehabilitation Exercises in Children With Congenital Heart Disease

This study aims to investigate the effects of functional inspiratory muscle training on physical performance and quality of life in pediatric patients with congenital heart disease.

Harmony TPV Post-Approval Study

The purpose of this study is to characterize the functionality of transcatheter implantation of the Medtronic Harmony Transcatheter Pulmonary Valve (TPV) achieved by real-world implanters.

Exercise Prehabilitation in Congenital Heart Disease

Around 8-12 in every 1000 babies are born with an abnormal heart, known as a congenital heart defect (CHD). Thanks to major advances in medical and surgical care, most babies born with CHD now live into adulthood. As people with CHD get older, many need repeated heart operations. It is known from other areas of medicine that "prehabilitation" - a short, structured exercise programme completed before surgery - can improve fitness and help patients cope better with major operations. However, this approach has never been tested in adults with CHD. The investigators are running a randomised controlled trial to find out whether a prehabilitation programme can improve physical fitness before heart surgery in adults with CHD. Participants will be randomly assigned to usual care or a prehabilitation group. Those in the prehabilitation group will follow a tailored, supervised exercise plan for several weeks before surgery. All patients undergoing cardiac surgery routinely complete a cardiopulmonary exercise test (CPET) as part of their standard pre-operative assessment. CPET is a well-established test that shows how the heart and lungs respond to increasing levels of exercise. During the test, participants exercise on a bike while their breathing, heart rate, and overall physiological response are continuously monitored. The results of each participant's routine CPET will be used to guide the individualised exercise programme in the intervention group. Exercise levels prescribed in the study will not exceed the intensity already safely achieved during the routine CPET, ensuring that activity remains within clinically assessed limits. Participants in the trial will complete an additional second CPET following the trial period. The key question is whether a tailored exercise programme can improve fitness in adults with CHD before heart surgery, and whether this approach is feasible, acceptable and practical to deliver, including successful recruitment and measurable physiological improvements.

Autus Valve Pivotal Study

Prospective, single-arm, multi-center study to evaluate safety and effectiveness of the Autus Size-Adjustable Valve in pediatric patients aged 18 months to 16 years requiring surgical pulmonary valve replacement. The Autus Valve may be expanded pre-implant to match the subject's body size. Subjects will be evaluated prior to the Autus Valve implant procedure, immediately post-implant, at hospital discharge, 30 days, 6 months, and annually through 10 years. The Autus Valve may be expanded post-implant via transcatheter balloon dilation to accommodate growth of the subject. In subjects who undergo a post-implant valve expansion, follow-up will continue for a minimum of 1 year after the post-implant valve expansion procedure.

Skin Pigment/Pulse Oximeter in Congenital Heart Disease (CHD)

Recent retrospective studies have demonstrated differences between pulse oximeter values (SpO2) and measured arterial oxygen saturation (SaO2) in patients identifying as Black or Hispanic. These retrospective studies have limitations because self-reported race is likely not an accurate metric for level of skin pigmentation and the retrospective nature of these studies may impact the accuracy of simultaneous measures of arterial oxygen saturation and pulse oximeter values. The few prospective studies that have evaluated this issue have utilized color-matching techniques to quantify skin pigmentation, and fewer studies have directly measured skin pigmentation in relation it to pulse oximeter accuracy. The aim of this study is to prospectively measure pulse oximeter accuracy in relation to measured levels of skin pigmentation in the congenital heart disease population.

PDA for Kidneys Study

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.

Comparison of the Best Method for Measuring Anti-Xa Activity in Children Receiving Unfractionated Heparin in Cardiac Intensive Care (COMPAXE-HNF)

This study aims to compare the accuracy of two different blood sampling methods from a central venous catheter (CVC) for measuring anti-Xa activity in children receiving unfractionated heparin (UFH) from this CVC. The results will be compared to a "gold standard" sample taken from an arterial catheter (KTA) whithout UFH. The objective is to identify a more reliable method for monitoring UFH, thereby reducing the risk of bleeding or thrombosis in these patients.

Perinatal Cardiology: Bridging Obstetric and Neonatal Care in CHD

This prospective observational study aims to evaluate the agreement between prenatal fetal echocardiographic diagnoses and postnatal diagnoses of congenital heart disease, and to assess the impact of prenatal diagnosis status on neonatal clinical outcomes at Assiut University Hospitals.

Pulmonary Determinants of Cardiorespiratory Fitness in Fontan Patients

This study will examine and record the patient's medical information and medical records (patient files, computer records, etc.). Physical examination, echocardiographic, electrocardiographic, laboratory, radiological, etc. evaluations are made during the routine controls of the patients. Within the scope of this study, no attempt will be made on the patients, no additional tests will be performed, or treatment changes will not be made in the patients because they are included in this study. After these records, KPET will be performed by a specialist doctor and physiotherapist for the patients who are directed to the Faculty of Physical Therapy and Rehabilitation Cardiopulmonary Rehabilitation Unit. Pulmonary function tests and respiratory muscle strength measurements will be performed on patients who have adequate rest after CPET.

Effect of Local Warming for Arterial Catheterization in Pediatric Anesthesia

The increase in internal diameter (ID) and cross-sectional area (CSA) may facilitate better arterial catheterization. Since an increase in body temperature can cause peripheral vasodilation, we aimed to determine if local warming of the radial artery (RA) catheterization site could improve the success rate of catheterization in pediatric patients.

International Multicenter Outcomes of Transcatheter Pulmonary Flow Restrictors in Congenital and Acquired Heart Disease

The international multicenter registry aims to collect real-world data on clinical outcomes and evaluate the net clinical success of transcatheter microvascular plug (MVP)-based pulmonary flow restrictors (PFRs) in patients with congenital and acquired heart disease.

Starlight Cardiovascular Lifeline Ductus Arteriosus Stent IDE Study

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.

Fontan Associated Liver Disease and the Evaluation of Biomarkers for Disease Severity Assessment

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.

SGLT2 Inhibition With Empagliflozin in Fontan Circulatory Failure

Some people are born with a birth defect where they only have one functioning ventricle (lower chamber) in their heart. This condition can be initially managed with a Fontan operation, but there is a risk of developing Fontan Circulatory Failure (FCF) later in life. FCF occurs when the single working heart ventricle is no longer strong enough to pump blood throughout the body. This also means the heart has difficulty supplying oxygen to keep up with the needs of the body. As a result, individuals living with FCF may have some challenges carrying out day to day activities. A heart transplant is currently the only therapeutic option for individuals living with FCF. The investigators are conducting this trial to determine whether a medication called empagliflozin can help these people have a better quality of life.

TORCH-LATAM: A Regional Pathway to Improve Transplant Evaluation for Congenital Heart Disease

Congenital heart disease can progress to advanced cardiac or cardiopulmonary failure requiring heart, heart-lung, or, in selected congenital conditions, bilateral lung transplantation with cardiac repair. Access to transplant evaluation and organ utilization can vary across regions and institutions, and potential candidates who are never referred or evaluated are not represented in conventional waiting-list statistics. TORCH-LATAM is a pragmatic multicenter stepped-wedge cluster-randomized effectiveness-implementation trial evaluating the TORCH Integrated Transplant Access Pathway (TORCH-ITAP) in Latin America. Regional network clusters transition in a randomized sequence from usual care to TORCH-ITAP. The pathway standardizes early identification and referral, a common recipient dataset, multidisciplinary candidacy adjudication, structured donor-organ assessment, immunologic and anatomic-physiologic matching, preservation and transport traceability, and navigation of clinical, regulatory, logistical, and financial barriers. The primary outcome is the proportion of enrolled recipient participants who complete a standardized multidisciplinary transplant evaluation and receive a documented candidacy decision within 90 days. Secondary outcomes include activation of an official transplant pathway or waitlist where applicable, transplantation within 12 months, pretransplant death or irreversible deterioration, donor-organ utilization, post-transplant safety and survival, and implementation outcomes. Recipient participants constitute the ClinicalTrials.gov enrollment; donors, organs, donor-recipient matches, preservation strategies, and transplant episodes are nested analytic units. TORCH-ITAP does not allocate organs and does not supersede national transplant laws, authorized transplant programs, or governmental requirements for cross-border organ movement.

EXCOR Active Driving System for the EXCOR Pediatric VAD IDE Study

The purpose of this study is to evaluate the device performance and monitor the safety and effectiveness of the Berlin Heart EXCOR Active Driving System while being used with the approved EXCOR Pediatric Ventricular Assist Device. EXCOR Active Driving System is intended for use with the approved EXCOR Pediatric VAD. The EXCOR Pediatric VAD is intended to provide mechanical circulatory support as a bridge to cardiac transplantation for pediatric patients. Pediatric candidates with severe isolated left ventricular or biventricular dysfunction who are candidates for cardiac transplant and require circulatory support may be treated using the EXCOR Pediatric. EXCOR Active is intended for use in a clinical setting. EXCOR Active can be used in any kind of hospital unit (e.g. OR, ICU, intermediate care unit or general care unit). The driving unit may be moved between clinical units using the caddy or baby buggy; however, a patient must always be accompanied by a person trained in the use of the manual pump and emergency procedures during transport in the event of an emergency. The driving unit can be transported during operation.

Heart2Heart for Teens With CHD

The goal of this clinical trial is to learn if teens living with congenital heart disease like and will use Heart2Heart and if it affects how they think and feel about e-cigarettes. Heart2Heart is a website that provides health information for teens with congenital heart disease. The main questions this trial aims to answer are: * Will teens enroll in the trial and complete the trial activities? * Do teens use and like Heart2Heart? * Does using Heart2Heart affect how teens think and feel about e-cigarettes? Researchers will compare the Heart2Heart group to a group that does not use Heart2Heart to see if it affects teens' thoughts and feelings about e-cigarettes. Participants will: * Complete online surveys * If assigned, explore the Heart2Heart website * If invited, share their opinions about Heart2Heart in an online discussion

Research on Body Voice AI Recognition System for Children's Health Management

The purpose of this research is to develop a body voice artificial intelligence (AI) recognition device, also referred to as an AI-assisted body sound identification device, by utilizing a deep learning-based novel AI algorithm in conjunction with a big body voice model. It could identify normal and abnormal heart, breath, and bowel sounds, and to provide early screening and auxiliary diagnosis of congenital heart disease (CHD), respiratory infections, diarrhea and other common multi-occurring diseases.