Near-infrared Spectroscopy (NIRS) Neurodevelopmental Outcomes
Summary
We believe that how a baby with Hypoplastic Left Heart Syndrome (HLHS)does after a major open heart operation, measured by things like blood pressure, oxygen saturation, heart rate and others, may have an impact on development. Studying how post-operative condition impacts outcomes may help us to protect babies better when they undergo surgery.
This study will look at some of the long-term outcomes of children with HLHS, including both mental development and quality of life. We will use information from your child's medical record to see if early oxygen delivery has an impact on later development.
Description
Over the past twenty years, the field of congenital heart disease has been marked by globally improved survival after complex congenital heart surgery. These improved results are clearly multi-factorial and include advances in diagnostic technologies, surgical techniques, perfusion strategies, pharmacologic therapies and perioperative monitoring of tissue oxygen delivery.
Hypoplastic left heart syndrome (HLHS) represents an extreme form of complex congenital heart disease in which the infant has prolonged cyanosis and a single systemic right ventricle. Staged palliation beginning with surgery in the neonatal period is the most common approach to infants with HLHS. After initial surgical palliation, infants are critically ill due to ischemia/reperfusion injury from cardiopulmonary bypass, coronary ischemia attributed to diastolic runoff, reduced total ventricular mass, continued hypoxemia during a time of increased metabolic demands, and finally, the inherent inefficiency of parallel circulation. The presence of any one of these physiologic derangements places the infant with HLHS at great risk for ischemia, organ dysfunction, circulatory collapse and, even, death. Postoperative monitoring of venous oximetry and, more recently, near infrared spectroscopy, have identified periods of impaired oxygen delivery and ischemia, allowed for interventions that enhance oxygen delivery and improved survival after cardiac surgery. Although, an apparent relationship exists between oxygen delivery and survival after cardiac surgery, little data is available on the relationship between oxygen delivery and neurodevelopmental outcomes (inclusive of neurocognitive and neuropsychologic outcomes).
Preliminary data from our center has recently identified that reduced venous oximetry was associated with adverse neurodevelopmental outcome. These initial findings warrant further investigation of neurologic injury, specifically, how such morbidity relates to perioperative ischemia and cerebral oxygen delivery. Hence, the purpose of this study is to understand the relationship of perioperative tissue dysoxia and long-term neurodevelopmental morbidity in children subject to oxygen delivery at which neurodevelopment is compromised. Identification of a critical threshold that may be devastating to a child's neurodevelopment and quality of life would allow for early intervention, goal-directed therapy and ultimately, improve outcomes.
Study Design
Observational Model: Case Control, Time Perspective: Prospective
Conditions
Hypoplastic Left Heart Syndrome
Location
Children's Hospital of Wisconsin
Milwaukee
Wisconsin
United States
53226
Status
Active, not recruiting
Source
Medical College of Wisconsin
Results (where available)
Links
- Source: http://clinicaltrials.gov/show/NCT00464100
- Information obtained from ClinicalTrials.gov on August 30, 2012
Medical and Biotech [MESH] Definitions
Hypoplastic Left Heart Syndrome
A condition caused by underdevelopment of the whole left half of the heart. It is characterized by hypoplasia of the left cardiac chambers (HEART ATRIUM; HEART VENTRICLE), the AORTA, the AORTIC VALVE, and the MITRAL VALVE. Severe symptoms appear in early infancy when DUCTUS ARTERIOSUS closes.
Norwood Procedures
A set of surgical procedures performed to establish sufficient outflow to the systemic circulation in individuals with univentricular congenital heart malformations, such as HYPOPLASTIC LEFT HEART SYNDROME, and MITRAL VALVE atresia, associated with systemic outflow obstruction. Follow-on surgeries may be performed and consist of a HEMI-FONTAN PROCEDURE as the stage 2 Norwood procedure and a FONTAN PROCEDURE as the stage 3 Norwood procedure.
Persistent Fetal Circulation Syndrome
A syndrome of persistent PULMONARY HYPERTENSION in the newborn infant (INFANT, NEWBORN) without demonstrable HEART DISEASES. This neonatal condition can be caused by severe pulmonary vasoconstriction (reactive type), hypertrophy of pulmonary arterial muscle (hypertrophic type), or abnormally developed pulmonary arterioles (hypoplastic type). The newborn patient exhibits CYANOSIS and ACIDOSIS due to the persistence of fetal circulatory pattern of right-to-left shunting of blood through a patent ductus arteriosus (DUCTUS ARTERIOSUS, PATENT) and at times a patent foramen ovale (FORAMEN OVALE, PATENT).
Ventricular Dysfunction, Left
A condition in which the LEFT VENTRICLE of the heart was functionally impaired. This condition usually leads to HEART FAILURE; MYOCARDIAL INFARCTION; and other cardiovascular complications. Diagnosis is made by measuring the diminished ejection fraction and a depressed level of motility of the left ventricular wall.
Ventricular Function, Left
The hemodynamic and electrophysiological action of the left HEART VENTRICLE. Its measurement is an important aspect of the clinical evaluation of patients with heart disease to determine the effects of the disease on cardiac performance.
Clinical Trials
Neurodevelopmental Outcomes in Hypoplastic Left Heart Syndrome
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 longe...
Family Adaptation Study Following the Diagnosis of Hypoplastic Left Heart Syndrome in a Newborn
Survival for one of the most complex forms of congenital heart disease, hypoplastic left heart syndrome (HLHS), has improved dramatically. However, little is known about family stress, co...
Examining Developmental Changes in Heart Contractions of Children With Congenital Heart Defects
Children who are born with heart defects undergo surgery when they are infants to correct the defects. However, many treatments that are used in pediatric heart patients were originally de...
NIRS in Neonatal Cardiac Surgery
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. Th...
Sleep Disordered Breathing in Children With Single Ventricle Physiology
This is an exploratory study designed to evaluate the incidence of, and to quantify sleep disordered breathing following stage I Norwood reconstructive surgery. Sleep disordered breathing...
PubMed Articles
BACKGROUND: The status of the reconstructed aorta in hypoplastic left heart syndrome is considered an important determinant of long-term prognosis. Therefore, we assessed the anatomy, elastic properti...
Hypoplasia of the left side of the heart is the most common cause of death from congenital heart disease in the first weeks of life. Once considered a surgically fatal disease, hypoplasia has been suc...
A Fetus With Hypoplastic Left Heart Syndrome and Tricuspid Stenosis With Evolving Hydrops Fetalis.
Hypoplastic left heart syndrome (HLHS) is a severe congenital heart defect that results in death if not treated shortly after birth. In approximately 60 to 70% of cases, HLHS can be detected prenatall...
Catheter interventions in the staged management of hypoplastic left heart syndrome.
Aim To analyse the current practice and contribution of catheter interventions in the staged management of patients with hypoplastic left heart syndrome. METHODS: This study is a retrospective case no...
Potocki-Lupski syndrome (PTLS) is a rare genetic disorder associated with neurodevelopmental delay and heart defects. We report the first case of prenatal diagnosis of PTLS in a fetus with hypoplastic...
