Medium-term outcome after anomalous aortic origin of a coronary artery repair in a pediatric cohort.

Background: Anomalous aortic origin of a coronary artery with an interarterial and intramural course (AAOCA) is a rare anomaly with increased risk of sudden cardiac death during or just after exercise among otherwise healthy youth. Risk stratification and management remain controversial, especially for the asymptomatic child with an anomalous right coronary artery from the left coronary sinus (ARCA). Medium-term surgical and quality-of-life (QOL) outcome data are lacking in this population.

Pediatric heart transplantation from donors with depressed ventricular function: an analysis of the United Network of Organ Sharing Database.

Background: Wait-list mortality for children awaiting heart transplantation remains high. Potential donor hearts with depressed ventricular function are often declined. We aimed to test the hypothesis that pediatric heart transplant recipients of grafts with depressed ventricular function would have comparable survival with those with normal function.

Risk factors for preoperative periventricular leukomalacia in term neonates with hypoplastic left heart syndrome are patient related.

Background: Preoperative brain injury is common in neonates with complex congenital heart disease. Increasing evidence suggests a complex interaction of prenatal and postnatal risk factors for development of brain white matter injury, called periventricular leukomalacia (PVL), in neonates with complex congenital heart disease. To date, there remains a limited understanding of the risk factors contributing to preoperative PVL in hypoplastic left heart syndrome (HLHS).

Hospital variation in postoperative infection and outcome after congenital heart surgery.

Background: Several initiatives aim to reduce postoperative infection across a variety of surgical patients as a means to improve overall quality of care and reduce variation across centers. However, the association of infection rates with hospital-level outcomes and resource utilization has not been well described. We evaluated this association across a multicenter cohort undergoing congenital heart surgery.

Postoperative electroencephalographic seizures are associated with deficits in executive function and social behaviors at 4 years of age following cardiac surgery in infancy.

Objective: The occurrence of an electroencephalographic (EEG) seizure after surgery for complex congenital heart defects has been associated with worse neurodevelopmental (ND) outcomes. We previously identified postoperative seizures documented by 48-hour EEG monitoring in 11% of 178 neonates and infants. Evaluation at 1 year of age did not identify an adverse effect of an EEG seizure on ND outcomes.

Results of elective repair at 6 months or younger in 277 patients with tetralogy of Fallot: a 14-year experience at a single center.

Objective: To report practice and outcomes in infants undergoing elective repair of tetralogy of Fallot.

Methods: A review of a retrospective cohort of elective complete repair of infants age 6 months or younger from 1995 to 2009 was performed. Patients were excluded because of previous interventions, hypercyanotic episodes, intensive care admissions, additional major cardiac defects, or if they were not discharged after birth.

Mechanical support as failure intervention in patients with cavopulmonary shunts (MFICS): rationale and aims of a new registry of mechanical circulatory support in single ventricle patients.

It is now recognized that a majority of single ventricle patients, those with functionally univentricular hearts, who have survived palliative cavopulmonary connection will experience circulatory failure and end-organ dysfunction due to intrinsic inadequacies of a circulation supported by a single ventricle. Thus, there are an increasing number of patients with functional single ventricles presenting with failing circulations that may benefit from mechanical circulatory support (MCS). The paucity of experience with MCS in this population, even at high volume cardiac centers, contributes to limited available data to guide MCS device selection and management.

Supplemental tube feeding does not mitigate weight loss in infants with shunt-dependent single-ventricle physiology.

Infants with shunt-dependent single-ventricle (SV) physiology are at risk for poor weight gain before superior cavopulmonary connection (SCPC). Lower weight-for-age z-score is a risk factor for prolonged length of stay (LOS) after SCPC. We sought to characterize infant growth and feeding and determine the effect of method of feeding on outcomes.

Sodium bicarbonate causes dose-dependent increases in cerebral blood flow in infants and children with single-ventricle physiology.

Background: Sodium bicarbonate (NaHCO3) is a common treatment for metabolic acidemia; however, little definitive information exists regarding its treatment efficacy and cerebral hemodynamic effects. This pilot observational study quantifies relative changes in cerebral blood flow (ΔrCBF) and oxy- and deoxyhemoglobin concentrations (ΔHbO2 and ΔHb) due to bolus administration of NaHCO3 in patients with mild base deficits.

Methods: Infants and children with hypoplastic left heart syndrome (HLHS) were enrolled before cardiac surgery.

Centre variation in cost and outcomes for congenital heart surgery.

Although overall outcomes for children undergoing heart surgery have improved, there is a significant variation in outcomes across hospitals. This review discusses the variation in cost and outcomes across centres performing congenital heart surgery, potential underlying mechanisms, and efforts to reduce variation and improve outcome.

Transesophageal echocardiography probe insertion failure in infants undergoing cardiac surgery.

Background: The use of intraoperative transesophageal echocardiography (iTEE) in neonates ≤4 kg has not been systematically described. We sought to describe the use of and determine risk factors for iTEE probe insertion failure in small infants. We also sought to develop an algorithm for predicting the likelihood of iTEE probe insertion failure.

Differential case ascertainment in clinical registry versus administrative data and impact on outcomes assessment for pediatric cardiac operations.

Background: Administrative datasets are often used to assess outcomes and quality of pediatric cardiac programs; however their accuracy regarding case ascertainment is unclear. We linked patient data (2004-2010) from the Society of Thoracic Surgeons Congenital Heart Surgery (STS-CHS) Database (clinical registry) and the Pediatric Health Information Systems (PHIS) database (administrative database) from hospitals participating in both to evaluate differential coding/classification of operations between datasets and subsequent impact on outcomes assessment.

Methods: Eight individual benchmark operations and the Risk Adjustment in Congenital Heart Surgery, version 1 (RACHS-1) categories were evaluated.

Early postoperative changes in cerebral oxygen metabolism following neonatal cardiac surgery: effects of surgical duration.

Objective: The early postoperative period following neonatal cardiac surgery is a time of increased risk for brain injury, yet the mechanisms underlying this risk are unknown. To understand these risks more completely, we quantified changes in postoperative cerebral metabolic rate of oxygen (CMRO(2)), oxygen extraction fraction (OEF), and cerebral blood flow (CBF) compared with preoperative levels by using noninvasive optical modalities.

Methods: Diffuse optical spectroscopy and diffuse correlation spectroscopy were used concurrently to derive cerebral blood flow and oxygen utilization postoperatively for 12 hours.

Results of genome-wide analyses on neurodevelopmental phenotypes at four-year follow-up following cardiac surgery in infancy.

Background: Adverse neurodevelopmental sequelae are reported among children who undergo early cardiac surgery to repair congenital heart defects (CHD). APOE genotype has previously been determined to contribute to the prediction of these outcomes. Understanding further genetic causes for the development of poor neurobehavioral outcomes should enhance patient risk stratification and improve both prevention and treatment strategies.

Protecting the infant brain during cardiac surgery: a systematic review.

Prevention of brain injury during congenital heart surgery has focused on intraoperative and perioperative neuroprotection and neuromonitoring. Many strategies have been adopted as "standard of care." However, the strength of evidence for these practices and the relationship to long-term outcomes are unknown.

Association of impaired linear growth and worse neurodevelopmental outcome in infants with single ventricle physiology: a report from the pediatric heart network infant single ventricle trial.

Objectives: To describe neurodevelopmental outcomes in infants with single ventricle (SV) physiology and determine factors associated with worse outcomes.

Study Design: Neurodevelopmental outcomes for infants with SV enrolled in a multicenter drug trial were assessed at 14 months of age using the Bayley Scales of Infant Development-II. Multivariable regression analysis was used to identify factors associated with worse outcomes.

A predictive model for neurodevelopmental outcome after the Norwood procedure.

Neurodevelopmental outcomes after the Norwood procedure for single right ventricular lesions are worse than those in the normal population. It would be valuable to identify which patients at the time of Norwood discharge are at greatest risk for neurodevelopmental impairment later in childhood. As such, this study sought to construct and validate a model to predict poor neurodevelopmental outcome using variables readily available to the clinician.

Neurodevelopmental outcomes in children with congenital heart disease: evaluation and management: a scientific statement from the American Heart Association.

Background: The goal of this statement was to review the available literature on surveillance, screening, evaluation, and management strategies and put forward a scientific statement that would comprehensively review the literature and create recommendations to optimize neurodevelopmental outcome in the pediatric congenital heart disease (CHD) population.

Methods And Results: A writing group appointed by the American Heart Association and American Academy of Pediatrics reviewed the available literature addressing developmental disorder and disability and developmental delay in the CHD population, with specific attention given to surveillance, screening, evaluation, and management strategies. MEDLINE and Google Scholar database searches from 1966 to 2011 were performed for English-language articles cross-referencing CHD with pertinent search terms.

Coronary ostioplasty for congenital atresia of the left main coronary artery ostium.

Background: Coronary ostial atresia is a rare anomaly, most frequently managed with coronary artery bypass grafting. This condition is often misdiagnosed as anomalous left coronary artery from the pulmonary artery until the time of surgical intervention, leaving the surgeon little time to contemplate the best operative technique.

Methods: In this single-center experience, three cases of congenital atresia of the left main coronary artery presenting during childhood were surgically managed with homograft patch ostioplasty.

18 years of the Fontan operation at a single institution: results from 771 consecutive patients.

Objectives: The aim of this study was to evaluate Fontan peri-operative outcomes for 771 consecutive patients.

Background: Since the initial description by Fontan, mortality associated with the Fontan operation has declined substantially. However, postoperative effusions remain a significant challenge.

Non-replication of an association of Apolipoprotein E2 with sinistrality.

A recent report found that left-handed adolescents were more than three times more likely to have an Apolipoprotein (APOE) ϵ2 allele. This study was unable to replicate this association in young adults (N=166). A meta-analysis of nine other datasets (N=360 to 7559, Power > 0.

Variation in outcomes for risk-stratified pediatric cardiac surgical operations: an analysis of the STS Congenital Heart Surgery Database.

Background: We evaluated outcomes for groups of risk-stratified operations in The Society of Thoracic Surgeons Congenital Heart Surgery Database to provide contemporary benchmarks and examine variation between centers.

Methods: Patients undergoing surgery from 2005 to 2009 were included. Centers with more than 10% missing data were excluded.