Active clinical trials for "Heart Defects, Congenital"

This is a retrospective chart review examining children and adults with history of Tetralogy of Fallot or pulmonary stenosis who have undergone subsequent pulmonary valve replacement. The primary interest of the study is to analyze the routine pre- and post-operative imaging studies.

The purpose of this cross-sectional study was to determine the interrelationships between health status and measures of cardiac performance in children 6 to 18 years of age with congenital heart disease who have undergone a Fontan procedure as surgical treatment for functional single ventricle. The goal was to develop a data set that will permit identification of a clinically relevant endpoint for subsequent trials of medical management of the Fontan patient.

Genetically inherited heart diseases (familial cardiopathies) are conditions affecting the heart passed on to family members by abnormalities in genetic information. These conditions are responsible for many heart related deaths and illnesses. Researchers are interested in learning more about the specific genetic abnormalities causing heart diseases. In addition, they would like to find out how these abnormal genes can contribute to the development of other medical problems. In order to do this, researchers plan to study patients and family members of patients diagnosed with genetically inherited heart disease. Those people participating in the study will undergo a variety of tests including blood tests, echocardiograms, and magnetic resonance imaging studies (MRI). These tests will be used to help researchers find the genetic problem causing the familial cardiopathy. Researchers hope that the information gathered from this study can be used to develop better medical care through early diagnosis, management, and treatment plans.

Pulmonary homografts are standard substitutes for right ventricular outflow tract reconstruction in congenital heart surgery. Unfortunately shortage and conduit failure secondary to early calcifications and shrinking are observed particularly for small sized conduits in younger patients. In neonates, Contegra® 12mm could be a valuable alternative, but conflicting evidence exists. This retrospective study compared the outcome of these two conduits in a newborn population.

Patent ductus arteriosus (PDA) is a common problem in the neonatal intensive care unit and can be secondary to prematurity or congenital heart disease (CHD). PDA is the most common cardiovascular abnormality in preterm infants, and is seen in 55% of infants born at 28 weeks, and 1000 grams or less. In addition to producing heart failure and prolonged respiratory distress or ventilator dependence, PDA has been implicated in development of broncho-pulmonary dysplasia, interventricular hemorrhage, cerebral ischemia, and necrotizing enterocolitis (NEC). In an Israeli population study 5.6% of all very low birth weight infants (VLBW) were diagnosed with NEC, and 9.4% of VLBW infants with PDA were found to have NEC. In a retrospective analysis of neonates with CHD exposed to Prostaglandin E found that the odds of developing NEC increased in infants with single ventricle physiology, especially hypoplastic left heart syndrome. The proposed pathophysiological explanation of NEC and PDA is a result of "diastolic steal" where blood flows in reverse from the mesenteric arteries back into the aorta leading to compromised diastolic blood flow and intestinal hypo-perfusion. Prior studies have demonstrated that infants with a hemodynamically significant PDA have decreased diastolic flow velocity of the mesenteric and renal arteries when measured by Doppler ultrasound, and an attenuated intestinal blood flow response to feedings in the post prandial period compared to infants without PDA. Near Infrared Spectroscopy (NIRS) has also been used to assess regional oxygen saturations (rSO2) in tissues such as the brain, kidney and mesentery in premature infants with PDA. These studies demonstrated lower baseline oxygenation of these tissues in infants with hemodynamically significant PDA. These prior NIRS studies evaluated babies with a median gestational age at the time of study of 10 days or less. It is unknown if this alteration in saturations will persist in extubated neonates with PDA at 12 or more days of life on full enteral feedings. In the present study the investigators hypothesize that infants with a PDA, whether secondary to prematurity or ductal dependent CHD, will have decreased splanchnic and renal perfusion and rSO2 renal/splanchnic measurements will be decreased during times of increased metabolic demand such as enteral gavage feeding. To test this hypothesis the investigators have designed a prospective observational study utilizing NIRS to record regional saturations at baseline, during feedings, and after feedings for 48 hours.

Congenital heart disease (CHD) is the most prevalent congenital malformation affecting 1 in 100 newborns per year. Children with CHD are a known risk population for brain injury, with neurodevelopmental alterations shown over time in up to 50% of cases. No adequate description exists of the type of neurocognitive anomalies or risk factors associated with CHD, and consequently no prognostic markers that may allow identification of high-risk cases are available.

Cardiovascular Response to Maternal Hyperoxygenation in Fetal Congenital Heart Disease

Congenital heart disease is the most common congenital anomaly. The life expectancy of children with congenital heart disease has increased considerably in recent years. Nevertheless, the evolution of these patients is marked by an increased risk of complications. Arrhythmias, heart failure, pulmonary arterial hypertension (PAH) and endocarditis may be promoted by the absence or delay of management in childhood, by residual lesions or post-operative cardiac scars and by the presence of prosthetic materials. PAH is a common complication of congenital heart disease, especially in non-operated shunts. PAH corresponds to an increase in pulmonary vascular resistance and mean pulmonary arterial pressure that becomes greater than 25mmHg at rest, leading to right ventricular failure and ultimately to the patient's death. Eisenmenger's syndrome corresponds to a non-reversible pulmonary arterial hypertension with a left-right shunt initially left open, then right-left secondary to the increase in pulmonary vascular resistance, leading to cyanosis, polycythemia and multivisceral involvement. It is the most advanced form of PAH with congenital heart disease. PAH will be suspected during echocardiographic follow-up of any patient with congenital heart disease, on the analysis of the velocity of tricuspid and/or pulmonary regurgitation flow. Echocardiography allows the monitoring of the VD (right ventricle) function, which is the major prognostic element in PAH. Cardiac catheterization is systematically recommended and remains the gold standard to confirm the diagnosis of PAH, establish its pathophysiology and prognosis but also for the follow-up under medical treatment of these patients in tertiary centres every 6 months. Although this tool is the gold standard, rigorously performed, it remains an invasive examination often poorly experienced by patients. 4D Flow MRI is a promising imaging that allows the acquisition of anatomical, volume, right ventricular remodeling and intracardiac flow information in a single step with 2D (only 8 minutes extra), in free breathing and totally autonomous mode. Thus, at the same time as the realization of a 2D MRI, essential for the diagnosis and follow-up of PAH, with an additional 8 minutes for 4D flow, the investigators could have additional fundamental information on pulmonary cardiac output but also prognostic markers of right ventricular dysfunction turning dramatic in pulmonary vascular disease.

This is an international, cross-sectional and descriptive study that aims to investigate differences in patient-reported outcome measures (PROMs) and patient-reported experience measures (PREMs) and that aims to explore the profile and healthcare needs of adults with congenital heart diseases.

With an incidence of 0.8 %, congenital heart disease (CHD) is the leading cause of congenital anomalies at birth. Medical advances in CHD have transferred the mortality from childhood to adulthood and today there are more adults with CHD than children. After focusing on survival, more attention is being given to health-related quality of life and secondary prevention in this population where warning signals are launched on the risk of sedentary lifestyle, obesity, cardiovascular risk 1. The cardiopulmonary exercise test (CPET), which is a non-invasive and dynamic examination, is becoming the gold standard to the follow-up 2 of these patients by allowing to quantify disease severity, to evaluate the quality of life 3, to give important prognostic information on functional capacity and haemodynamic response 4, to facilitate a safe decision-making when prescribing exercise programmes and sport participation for these children with CHD 5. In this context, in a cross-sectional study from 2010 to 2015, the investigators evaluated the cardiopulmonary fitness of children with CHD by comparing them with healthy children 6. In this study, 496 children with CHD compared to 302 healthy children were included. It showed that maximum oxygen uptake (VO2max) and ventilatory anaerobic threshold (VAT) are decreased in CHD children compared to healthy children, clinical determinants of decreased VO2max have been defined for CHD children. This study was proposed, despite the cross-sectional nature, an average decrease in annual VO2max (0,84 ml/kg/min per year) to make pediatric and congenital cardiologist aware of the need to a regular follow up for these patients. In this new study, the main objective was to know the real evolution of VO2max in these patients from this same cohort, with a longitudinal design, by collecting a new CPET carried out between 2015 and 2020 and compared these results to healthy pediatric population. The secondary objectives were: to know the evolution of the VAT, to define the clinical determinants in relation to the annual decrease of the VO2max. And to describe the population lost to follow-up in this retrospective study which represents current practice.