Active clinical trials for "Heart Defects, Congenital"

This study aims to determine what are some of the clinical characteristics and associations of Fontan patients who are doing well, as well as how accurate cardiology providers are at predicting the likelihood of future adverse event in their Fontan patients.

The aim of this study will be to evaluate vancomycin and cefoxitin blood levels during elective cardiopulmonary bypass (CPB) surgery in four pre-determined pediatric strata: neonates, infants, children weighting less than 40 kg and children weighting more than 40 kg.

Fibrinogen plays an important role in the coagulation cascade. Low levels of preoperative fibrinogen have been associated in adults with higher blood and transfusion requirements during surgery. Guidelines from the European Society of Anesthesiology and Intensive Care recommends fibrinogen substitution based on viscoelastic tests in adult cardiac surgery. Cardiopulmonary bypass results in a profound hemodilution and dilution of all coagulation factors. This is especially true in children where the priming volume of the cardiopulmonary bypass circuit is much higher compared to the patient's weight than in adults. Cardiopulmonary bypass generates high levels of tissue plasminogen activator resulting in a hyperfibrinolysis, further lowering fibrinogen levels. The aim of this retrospective study is to explore a possible relationship between preoperative fibrinogen levels and perioperative blood loss, as well as transfusion requirements in children undergoing cardiac surgery.

To answer the research question: "Would image-based modelling result in different clinical decisions as compared to clinical practice guidelines?", we will conduct a randomized controlled experiment in which we will compare the hypothetical decisions made by interventional cardiologists who are presented with imaging parameters currently recommended by clinical practice guidelines vs. hypothetical decisions made by interventional cardiologists receiving an expanded list of parameters, including simulation modelling.

Many toddlers and children are released home with relatively low hemoglobin levels following cardiac surgery, and with no careful follow up, at an age at which anemia is common. At this age, breast milk and its substitute do not provide enough iron, and parents are not adherent enough to iron supplements. Due to these facts, there is high importance, especially in children suffering from heart defects, for close follow up on their hemoglobin levels. Studies following hemoglobin trend post cardiac surgery were never done with children.

Previous studies have examined the usefulness of pulse oximetry or oxygen saturation to screen for left-sided cardiac lesions. These studies have shown that the occurrence of critical congenital cardiac malformations among asymptomatic newborns is high; the technique of pulse oximetry is reliable for detection of ductal dependant left-sided lesions, simple to operate(requires little time and can be done in the newborn nursery) and is cost effective; there is effective follow-up test (heart ultrasound) and available interventions have an effect on outcome for diagnosed newborns. The importance of this research project is to examine the overall helpfulness of measuring oximetry in newborn infants using somatic oximetry, as well ast to prepare for a population based study in the state of Florida.

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.

Lysine analogs, like tranexamic acid (TXA) or epsilon aminocaproic acid (EACA), are antifibrinolytic agents routinely administered in children undergoing different surgeries associated with a high bleeding risk (e.g. cardiac, craniofacial, and orthopedic surgeries). Although there is a growing literature regarding the pharmacokinetic characteristics of these drugs in children, the plasmatic concentration required to completely inhibit fibrinolysis remains to be determined. In this in vitro study, the investigators will use an experimental model of fibrinolysis designed for rotational thromboelastometry (ROTEM®) to determine the minimal concentration inhibiting fibrinolysis for both TXA and EACA. In addition, this study will be used to create and validate a new experimental assay to measure fibrinolysis and the effect of antifibrinolytic agents.

The purpose of this study is to find out whether a combination of new urine tests and blood tests can show kidney injury in its early stages, before kidney failure sets in. If the investigators find new tests that show kidney injury in early stages, the investigators hope to start treating people with kidney injury earlier, to prevent kidney failure. You/your child are at higher risk for kidney injury and kidney failure than most other people, because of having operations with cardiopulmonary bypass (a machine that pumps your/your child's blood during the operation). This research is being done because there are no tests yet proven to show kidney injury before it leads to kidney failure. The urine and blood tests the investigators are studying have each been shown to indicate some degree of kidney injury in certain people, but not with the accuracy needed to diagnose disease. The investigators think that the combination of urine and blood tests being tried in this research study may provide enough information to better diagnose kidney injury at an earlier stage. About 20 persons over 2 years old up to adults will take part in this study. All will be from the Herma Heart Center of Children's Hospital of Wisconsin.

Neonates and infants that have cardiac surgery with cardiopulmonary bypass (CPB) for congenital heart disease are at great risk for experiencing life-threatening low cardiac output syndrome (LCOS) in the first 24 hours after surgery. The poor perfusion and inadequate oxygen delivery that occurs may result in multiple organ failure and death. It is LCOS that is responsible for the majority of early postoperative deaths in this population of neonates and infants. Improved pediatric probes placed in peripheral locations using near infrared spectroscopy (NIRS) may permit continuous monitoring of venous saturations reflecting overall perfusion and oxygen balance in the tissues. Following parental or guardian consent, 30 neonates and infants scheduled to undergo surgery for congenital heart disease will be enrolled. At the end of surgery, four EQUANOX Advance 8004CB probes will be placed on the flank, lower extremities and the forehead. Continuous NIRS saturations will be monitored and stored for analysis. Point of care (POC) lactates will be obtained after admission to the intensive care unit (ICU) every 2 hours for the first 24 hours postoperatively, then once at 48 hours. The association between NIRS oxygen saturation and POC lactate values will be assessed using mixed linear models taking into account the repeated measures design. Exploratory analyses will be performed to assess whether NIRS oxygen saturation is associated with outcomes such as days in ICU, adverse events and mortality. The ability to use noninvasive, continuous monitoring for overall perfusion and cardiac output will allow better and earlier therapy for neonates and infants following cardiac surgery.