Impaired Maternal-Fetal Environment and Risk for Preoperative Focal White Matter Injury in Neonates With Complex Congenital Heart Disease.

Background Infants with congenital heart disease (CHD) are at risk for white matter injury (WMI) before neonatal heart surgery. Better knowledge of the causes of preoperative WMI may provide insights into interventions that improve neurodevelopmental outcomes in these patients. Methods and Results A prospective single-center study of preoperative WMI in neonates with CHD recorded data on primary cardiac diagnosis, maternal-fetal environment (MFE), delivery type, subject anthropometrics, and preoperative care.

Association of Ongoing Cerebral Oxygen Extraction During Deep Hypothermic Circulatory Arrest With Postoperative Brain Injury.

Cardiac surgery utilizing circulatory arrest is most commonly performed under deep hypothermia (∼18°C) to suppress tissue oxygen demand and provide neuroprotection during operative circulatory arrest. Studies investigating the effects of deep hypothermic circulatory arrest (DHCA) on neurodevelopmental outcomes of patients with congenital heart disease give conflicting results. Here, we address these issues by quantifying changes in cerebral oxygen saturation, blood flow, and oxygen metabolism in neonates during DHCA and investigating the association of these changes with postoperative brain injury.

Preoperative cerebral hemodynamics from birth to surgery in neonates with critical congenital heart disease.

Background: Hypoxic-ischemic white matter brain injury commonly occurs in neonates with critical congenital heart disease. Recent work has shown that longer time to surgery is associated with increased risk for this injury. In this study we investigated changes in perinatal cerebral hemodynamics during the transition from fetal to neonatal circulation to ascertain mechanisms that might underlie this risk.

Surgical and Catheter-Based Reinterventions Are Common in Long-Term Survivors of the Fontan Operation.

Background: There are limited follow-up studies examining surgical and catheter-based reinterventions in long-term survivors of the Fontan operation.

Methods And Results: All 773 patients who underwent Fontan at our institution between 1992 and 2009 were retrospectively reviewed. Current information regarding post-Fontan intervention was available for 70%.

Systemic Atrioventricular Valve Excision and Ventricular Assist Devices in Pediatric Patients.

Background: Continuous-flow ventricular assist devices (CF VADs) designed for adults are increasingly used in pediatric patients. However, there is greater risk of device inflow obstruction as a result of size and anatomy.

Methods: We reviewed all cases of systemic atrioventricular valve (AVV) excision with HeartWare HVAD (HeartWare, Framingham, MA) implantation in the systemic ventricle performed at our institution from November 2015 to May 2016.

Long-Term Outcomes of Pediatric Cardiac Patients Supported by Extracorporeal Membrane Oxygenation.

Objective: Extracorporeal membrane oxygenation is an important form of short-term mechanical support in children with cardiac disease, but information on long-term outcomes and quality of life is limited. The primary objective of this study was to determine the long-term outcomes of children previously supported by extracorporeal membrane oxygenation for cardiac etiologies.

Design: A retrospective analysis was performed on patients with cardiac disease managed with extracorporeal membrane oxygenation between January 1, 1995, and December 31, 2012, at the Children's Hospital of Philadelphia.

Effect of Fontan-Associated Morbidities on Survival With Intact Fontan Circulation.

Although survival after the Fontan operation has improved, little is known about the burden of major medical morbidities associated with the modern total cavopulmonary connection (TCPC). A total of 773 consecutive patients who underwent a first Fontan operation at our institution between 1992 and 2009 were retrospectively reviewed. All subjects underwent TCPC (53% lateral tunnel, 47% extracardiac conduit).

Long-term survival after the Fontan operation: Twenty years of experience at a single center.

Objective: Existing studies of patients palliated with the Fontan operation are limited by heterogeneous patient populations and incomplete follow-up. This study aimed to describe long-term post-Fontan survival in a modern patient cohort.

Methods: All 773 patients who underwent a first Fontan operation at our institution between 1992 and 2009 were reviewed.

Neurological Injury and Cerebral Blood Flow in Single Ventricles Throughout Staged Surgical Reconstruction.

Background: Patients with a single ventricle experience a high rate of brain injury and adverse neurodevelopmental outcome; however, the incidence of brain abnormalities throughout surgical reconstruction and their relationship with cerebral blood flow, oxygen delivery, and carbon dioxide reactivity remain unknown.

Methods: Patients with a single ventricle were studied with magnetic resonance imaging scans immediately prior to bidirectional Glenn (pre-BDG), before Fontan (BDG), and then 3 to 9 months after Fontan reconstruction.

Results: One hundred sixty-eight consecutive subjects recruited into the project underwent 235 scans: 63 pre-BDG (mean age, 4.

Mechanical Circulatory Support as Bridge to Transplantation for the Failing Single Ventricle.

Background: Circulatory failure necessitating cardiac transplantation will ultimately develop in many patients with functional single-ventricle physiology. Interest in the use of mechanical circulatory support (MCS) in this population is growing.

Methods: This was a retrospective case series of patients with functional single-ventricle physiology who underwent MCS with a ventricular assist device or a total artificial heart as a bridge to cardiac transplantation between January 2006 and December 2014.

Relationship of cerebral blood flow to aortic-to-pulmonary collateral/shunt flow in single ventricles.

Objective: Patients with single ventricle can develop aortic-to-pulmonary collaterals (APCs). Along with systemic-to-pulmonary artery shunts, these structures represent a direct pathway from systemic to pulmonary circulations, and may limit cerebral blood flow (CBF). This study investigated the relationship between CBF and APC flow on room air and in hypercarbia, which increases CBF in patients with single ventricle.

Brain magnetic resonance immediately before surgery in single ventricles and surgical postponement.

Background: Single-ventricle patients undergoing surgical reconstruction experience a high rate of brain injury. Incidental findings on preoperative brain scans may result in safety considerations involving hemorrhage extension during cardiopulmonary bypass that result in surgical postponement.

Methods: Single-ventricle patients were studied with brain scans immediately preoperatively, as part of a National Institutes of Health study, and were reviewed by neuroradiology immediately before cardiopulmonary bypass.

Time to surgery and preoperative cerebral hemodynamics predict postoperative white matter injury in neonates with hypoplastic left heart syndrome.

Objective: Hypoxic-ischemic white mater brain injury commonly occurs in neonates with hypoplastic left heart syndrome (HLHS). Approximately one half of HLHS survivors will exhibit neurobehavioral symptoms believed to be associated with this injury, although the exact timing of the injury is unknown.

Methods: Neonates with HLHS were recruited for pre- and postoperative monitoring of cerebral oxygen saturation, cerebral oxygen extraction fraction, and cerebral blood flow using 2 noninvasive optical-based techniques: diffuse optical spectroscopy and diffuse correlation spectroscopy.

Successful use of the total artificial heart in the failing Fontan circulation.

Typical left ventricular assist devices are often ineffective for the failing Fontan circulation. We report the first successful use of a total artificial heart as a bridge to transplant in a patient who had previously undergone a Fontan operation.

Design, synthesis, radiolabeling and in vivo evaluation of potential positron emission tomography (PET) radioligands for brain imaging of the 5-HT₇ receptor.

Here we describe the design, synthesis, and pharmacological evaluation of a set of compounds structurally related to the high affinity serotonin 5-HT7 receptor agonist N-(4-cyanophenylmethyl)-4-(2-diphenyl)-1-piperazinehexanamide (6, LP-211). Specific structural modifications were performed in order to maintain affinity for the target receptor and to improve the selectivity over 5-HT1A and adrenergic α1 receptors. The synthesized compounds have chemical features that could enable labeling with a positron emitter radioisotope (carbon-11 or fluorine-18) and lipophilicity within the range considered optimal for brain penetration and low non-specific binding.

Cerebral oxygen metabolism in neonates with congenital heart disease quantified by MRI and optics.

Neonatal congenital heart disease (CHD) is associated with altered cerebral hemodynamics and increased risk of brain injury. Two novel noninvasive techniques, magnetic resonance imaging (MRI) and diffuse optical and correlation spectroscopies (diffuse optical spectroscopy (DOS), diffuse correlation spectroscopy (DCS)), were employed to quantify cerebral blood flow (CBF) and oxygen metabolism (CMRO(2)) of 32 anesthetized CHD neonates at rest and during hypercapnia. Cerebral venous oxygen saturation (S(v)O(2)) and CBF were measured simultaneously with MRI in the superior sagittal sinus, yielding global oxygen extraction fraction (OEF) and global CMRO(2) in physiologic units.

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).

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.

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.

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.

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.