Active clinical trials for "Hypoplastic Left Heart Syndrome"

Single ventricle lesions are the leading cause of illness and death from congenital heart disease. The modified Fontan Operation is the corrective surgery for these lesions. The operation is done in stages over a few years and children who complete the operation are known to have greater neurodevelopmental (ND) deficits than the general population. The purpose of this study is to understand how blood flow to the brain (CBF) and brain lesions relate to ND outcome, as well as how CMRO2 relates to anatomic brain lesions. These relationships will be studied through Magnetic Resonance Imaging (MRI) and ND Testing.

This study is being conducted to determine what dexmedetomidine does to the body and in turn, what how the body handles the medication. This medication, for the purpose of this trial, is used as a short-term sedative for infants who are immediately post-operative from cardiac surgery and have a breathing tube and are breathing with the assistance of a mechanical ventilator or breathing machine.

Before birth, the placenta (a structure with many blood vessels attached to the inside of your womb) and the umbilical cord (the umbilical cord is attached to the placenta) are sending oxygen and nutrients from the mother's blood through the umbilical cord to the baby. After a baby is born the cord is clamped and babies have to start breathing and support themselves. At the moment when a baby with congenital heart disease is born they will have their cord clamped immediately (this is called immediate cord clamping (ICC)). After ICC the clinical team will start to help a baby transition by carefully monitoring their oxygen saturation (give oxygen if needed), provide warmth, and dry and stimulate. Several animal studies have shown that clamping the cord right after birth might causes the baby to miss the benefits of receiving blood from the umbilical cord / placenta. Delayed Cord Clamping (DCC) is when the baby stays attached to the cord for a longer time. Studies show that DCC has many benefits especially for a newborn baby, such as higher iron storage, less need for blood transfusions, and improved circulation. This can be done while the baby is breathing on its own or while we help you baby breath (this is called resuscitation). This study aims to examine whether DCC while providing resuscitation in infants with CHD is helpful compared to immediate cord clamping. Prior to the birth of your baby, a sealed envelope will be opened and your baby will be randomly assigned to either the DCC with resuscitation group or the ICC group. 40 babies will be enrolled into this study, 20 in each group. In the DCC group, the umbilical cord will be clamped after 120 seconds during which time your baby will receive the care he/she requires by the NICU team. In the ICC group, the umbilical cord will be clamped immediately and he/she will be brought over the resuscitation bed to be cared for by the same team.

Brain problems occur in neonatal open heart surgery with a frequency of 20-70%, seen on neurological examination, brain imaging such as magnetic resonance imaging (MRI), or long term development problems such as learning disorders and hyperactivity syndromes. This study aims to determine if erythropoetin, a natural hormone made in the body, protects the brain from damage when given in high doses before and during neonatal open heart surgery. We will use brain MRI, brain wave tests (EEG), neurological examination, and long term developmental outcome testing to see if erythropoetin is better than salt water injection (placebo) in protecting the brain.

Single ventricular (SV) heart was a uniformly fatal condition before the advances in surgical treatment in 1980. In the present era, 5-year survival rate with SV is 75%, with some centers, including the Stollery Children's Hospital reporting higher survival. Although mortality remains a major concern, the research focus has shifted to management of late complications as well as improving patient physical and mental health related quality of life issues. Children with SV have reduced exercise tolerance and this is progressive through adulthood. Recent advances in remote health assessment and telehealth systems have allowed the development of medically supervised home graduated physical training for adult cardiac patient rehabilitation. To our knowledge, the application of these technologies has not been applied to SV patients. The long term goal is to use this technology to improve patient exercise capacity and to positively influence parental and patient perceptions of the patient's physical ability.

The purpose of the Oxy-CAHN study is to improve the monitoring capabilities of newborn infants recovering from congenital heart surgery. Currently, we utilize important but unsophisticated measures, such as vital signs and lactate measurements, to monitor these patients. Although they are useful in categorizing patients as well or unwell, these signs currently lack the power quantify a patient's risk for cardiac arrest. More to the point, they are mostly indirect measures of what we really are assessing, which is tissue oxygen delivery. Our group has significant expertise with devices which quantify the amount of oxygen that a baby consumes every minute. Historically, these values are more commonly used in combination with other measures to assess nutritional and metabolism status. In critically ill patients, however, the volume of oxygen consumed by a patient may be limited by the amount of oxygen their circulation delivers. This may represent a critical relationship, which has been previously described, but not exploited for the purpose of identifying patients with critically low oxygen delivery. The aims of this study are therefore (1) to demonstrate that oxygen consumption can be safely and precisely measured continuously in newborns undergoing one of two common congenital heart surgeries, (2) to determine whether postoperative circulatory failure is associated with a precedent change in oxygen consumption, and (3) to determine whether the addition of the oxygen-based measurements (including oxygen consumption and venous oxygen saturations) to standardly measured parameters will add power in predicting which patients will experience postoperative circulatory failure. If successful, this study may improve our capacity to non-invasively and continuously monitor patients following the highest risk congenital heart surgeries, and in the future,to create an algorithm which quantifies a patients risk for having a cardiac arrest. This may permit providers to intervene on these patients earlier, improving the morbidity and mortality associated with congenital heart disease.

Neonates with a congenital heart defect are often in need of early cardiac surgery. In complex congenital heart defects, cardiopulmonary bypass is usually employed, with or without deep hypothermic circulatory arrest (DHCA). The brain is especially vulnerable to ischemic injury, which puts neonates undergoing complex operations at high risk of neurodevelopmental disorders. Selective antegrade cerebral perfusion (ACP) instead of DHCA during these complex operations may contribute to less cerebral damage, but literature is not conclusive on this issue. Therefore, the investigators will perform a randomised controlled trial comparing DHCA and ACP in neonatal aortic arch reconstructions, focusing on cerebral damage and neurological outcome.

All neonates with congenital heart disease undergoing stage one palliation (Norwood procedure, Damus-Kaye-Stansel procedure) at Texas Children's Hospital will be regularly monitored for B-type natriuretic peptide (BNP) and Troponin level before the surgical procedure, on arrival to the cardiac intensive care unit after their surgical procedure, every 6 hours during the first 24 hours of the post-operative period, followed by daily levels for the first week, and then weekly during patient's regular laboratory work up schedule. These cardiac biomarkers are linked to demographic, hemodynamic, respiratory, pharmacological data available via Sickbay.

Due to the small size of infants and the use of bypass machines, physicians have difficulty assessing whether the infant's brain and body is getting enough oxygen during heart surgery. This study compares continuous monitoring via the NIRS (Near Infrared Spectroscopy) to the traditional methods of determining oxygen saturation.

There have been many improvements in the care of children with hypoplastic left heart syndrome (HLHS). This has helped these children survive longer. Because these children now live longer, researchers are recognizing developmental disabilities (the children are behind in their thinking or physical activity) in children with hypoplastic left heart syndrome. The purpose of this research study is to help the investigators learn more about developmental disabilities in children with hypoplastic left heart syndrome. During the time of your child's first surgery for hypoplastic left heart syndrome, a monitor was placed on your child's forehead to measure the oxygen levels in the brain. This monitor is called Near Infrared Spectrometry (NIRS). The researchers involved in this study want to know if oxygen levels in the blood vessels of the brain have any effect on developmental disabilities later in life in children with hypoplastic left heart syndrome. The researchers plan to enroll 60 research subjects.