Sudden Cardiac Death in Systemic Right Ventricle
Sudden Cardiac DeathCongenital Heart Disease1 moreIn complete transposition of the great arteries (TGA) with previous atrial switch repair, and congenitally corrected transposition of the great arteries (ccTGA), the morphological right ventricle and its tricuspid valve continue to support the systemic circulation. This results in late complications including including sudden death. This retrospective multicentric study aims to evaluate the prevalence of SCD in a contemporary population of patients with a systemic RV and identify specific risk factors for SCD and hemodynamically significant ventricular arrhythmia This registry records demographics, clinical, imaging data, electrophysiological and laboratory of patients with a sRV and a transposition of the great arteries Primary end points are defined by sudden cardiac death, near-miss sudden death, as well as sustained VT requiring defibrillation.
Utility of Lung Ultrasound in the Estimation of Extravascular Lung Water in Pediatric Population...
Pulmonary CongestionCongenital Heart Disease1 moreIncreased extravascular lung water (EVLW) may increase mortality and morbidity in cardiopulmonary pathology. Many factors can cause increased extravascular lung water and pulmonary edema after cardiac surgery. This includes left ventricular failure, acute mitral regurgitation; systemic inflammatory response post-cardiopulmonary bypass, left to right shunts, transfusion associated acute lung injury, acute respiratory distress syndrome(ARDS) and sepsis. The clinical assessment of lung water ranges from auscultation to radiological methods to invasive measurements like dye dilution or thermodilution studies. Lung ultrasonography is the newest modality for noninvasive assessment of extravascular lung water. Lung ultrasound has been validated against auscultation, chest X-rays, CT chest as well as the bedside gold standard, transpulmonary thermodilution in adults. Critically ill children are more susceptible to complications and worsened outcomes from increased EVLW. Lung ultrasound correlates with clinical and radiological endpoints, but has not been validated against invasive objective measures like transpulmonary thermodilution. Evaluation of transpulmonary thermodilution setups in the pediatric population has shown different normal values and cutoffs compared to adults, possibly due to differential rates growth and development. It is aimed to investigate the correlation of Lung ultrasound based indices of extravascular lung water to invasive measures, assess optimum cutoffs to appropriate clinical endpoints and evaluate their sensitivity and specificity.
White Matter Hyperintensities Burden in Adult Patients With Cyanotic Congenital Heart Disease: a...
AgingPremature2 moreThe study aims at investigating the role of cyanotic congenital heart disease (cCHD) on brain aging. The investigators assume that due to congenital and acquired cardiovascular abnormalities, cCHD patients could show radiologic (and clinical) signs of precocious brain aging and eventual cognitive decline.
Imaging by 4 DFlow in Patients With Tetralogy of Fallot
Congenital Heart DiseaseConventionnal cadiac magnetic resonance (CMR) is the reference to assess changes in right heart flow and pulmonary artery hemodynamics in patients with repaired Tetralogy of Fallot. 4D Flow CMR Imaging is a new imaging modality able to assess all of these parameters faster (8 min vs 30 min) and more comfortably. The aim of this study is to compare conventionnal CMR and 4D Flow CMR for the assessment of these parameters (ventricular volume, ventricular systolic function, and regurgitation of the pulmonary pathway).
Leuven Growing Into Deficit Follow-up Study
Heart DefectsCongenital3 moreThe key objective of the Leuven growing-into-deficit (GID) follow-up-study is to test the hypothesis that children with a congenital heart disease (CHD) show more neurocognitive impairment at the second follow-up at 7 years old than at the first follow-up at the age of 4, compared to healthy controls.
The Predictability of Intraoperative Rotational Thromboelastometry on Postoperative Bleeding and...
Surgery for Congenital Heart DiseasePediatric PatientsViscoelastic hemostatic assay has been reported to be superior to predict perioperative bleeding in cardiac surgical patients compared with conventional blood coagulation test. However, the role of rotational thromboelastometry (ROTEM) in predicting perioperative bleeding and transfusion requirements in pediatric patients who undergoing surgery for congenital heart disease. Therefore, the investigators attempt to evaluate the predictability of intraoperative rotational thromboelastometry for perioperative bleeding and transfusion requirements in pediatric cardiac surgical patients by comparing with conventional coagulation test. The investigators also attempt to evaluate the correlation between ROTEM parameters, platelet count, and results of conventional coagulation test results.
Parenting After Infant Congenital Heart Defect Diagnosis
Congenital Heart DiseaseThe purpose of this research study is to learn about parents' experiences following diagnosis of a fetal/neonatal Congenital Heart Defect (CHD). Nurses, physicians, and other health-care clinicians will benefit from an improved understanding of what the diagnosis means to parents and what they expect concerning the infant, being a parent, and caregiving tasks and responsibilities. The investigators expect that the knowledge gained will increase clinicians' ability to respond to parents' needs.
Nutritional Failure in Infants With Single Ventricle Congenital Heart Disease
Congenital Heart DiseaseNutritional FailureThe purpose of this study is to understand the causes of growth failure and to define biochemical markers of nutritional failure in patients with single ventricle Congenital Heart Disease (CHD).
Use of ROTEM® in Pediatric Cardiac Surgical Patients
Congenital Heart DefectCardiac surgery requiring use of a heart-lung machine, also known as cardiopulmonary bypass (CPB) can induce abnormalities in blood coagulation (clotting) that lead to excessive blood loss during and after operation. While of significant concern in adults, this problem takes on even greater importance in children due to the simple fact that they have a smaller blood volume. It is well known that if a child has previously undergone a heart operation, they are at increased risk of bleeding should more cardiac surgery be required ("reoperation"). The processes regulating blood coagulation are extraordinarily complex, and little is known about the exact mechanisms that contribute to the increased bleeding associated with cardiac reoperation in children. Rotational thromboelastometry (ROTEM®) is a technology that can provide, at the bedside, detailed information about coagulation abnormalities. While not currently approved for general use in the United States, in Europe ROTEM® has been used to guide administration of the blood products in surgery based upon determination of specific coagulation abnormalities. Importantly, there is now evidence that guidelines for transfusion therapy based on ROTEM® reduce transfusion requirements thus decreasing patient exposure to blood products. Whether the use of ROTEM® has potential benefit for pediatric cardiac surgical patients has not been studied. Accordingly, the present study was designed to provide preliminary data comparing coagulation profiles between children undergoing cardiac reoperation to those having primary procedure. The investigators hypothesize that ROTEM® analysis will detect a pattern of impaired coagulation in children undergoing reoperation. The overall objective of the four assays of ROTEM® analysis therefore, is to provide information that can be used to devise a rational transfusion protocol for pediatric cardiac surgical subjects.
Retrospective Pulmonary Valve Replacement Imaging
Congenital Heart DiseaseThis 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.