A new multiparameter approach to computational simulation for Fontan assessment and redesign.

Introduction: Despite an abundance of prior Fontan simulation articles, there have been relatively few clinical advances that are a direct result of computational methods. We address a few key limitations of previous Fontan simulations as a step towards increasing clinical relevance. Previous simulations have been limited in scope because they have primarily focused on a single energy loss parameter.

Sustained experience of emotion after loss of memory in patients with amnesia.

Can the experience of an emotion persist once the memory for what induced the emotion has been forgotten? We capitalized on a rare opportunity to study this question directly using a select group of patients with severe amnesia following circumscribed bilateral damage to the hippocampus. The amnesic patients underwent a sadness induction procedure (using affectively-laden film clips) to ascertain whether their experience of sadness would persist beyond their memory for the sadness-inducing films. The experiment showed that the patients continued to experience elevated levels of sadness well beyond the point in time at which they had lost factual memory for the film clips.

Majewski osteodysplastic primordial dwarfism type II (MOPD II): expanding the vascular phenotype.

Majewski Osteodysplastic Primordial Dwarfism, Type II (MOPD II) is a rare, autosomal recessive disorder. Features include severe intrauterine growth retardation (IUGR), poor postnatal growth (adult stature approximately 100 cm), severe microcephaly, skeletal dysplasia, characteristic facial features, and normal or near normal intelligence. An Institutional Review Board (IRB) approved registry was created and currently follows 25 patients with a diagnosis of MOPD II.

Periodic magnetic field as a polarized and focusing thermal neutron spectrometer and monochromator.

A novel periodic magnetic field (PMF) optic is shown to act as a prism, lens, and polarizer for neutrons and particles with a magnetic dipole moment. The PMF has a two-dimensional field in the axial direction of neutron propagation. The PMF alternating magnetic field polarity provides strong gradients that cause separation of neutrons by wavelength axially and by spin state transversely.

Perioperative complications in children with pulmonary hypertension undergoing general anesthesia with ketamine.

Background: Pulmonary arterial hypertension (PAH) is associated with significant perioperative risk for major complications in children, including pulmonary hypertensive crisis and cardiac arrest. Uncertainty remains about the safety of ketamine anesthesia in this patient population.

Aim: Retrospectively review the medical records of children with PAH to ascertain the nature and frequency of peri-procedural complications and to determine whether ketamine administration was associated with peri-procedural complications.

Congenital extrahepatic portosystemic shunt associated with heterotaxy and polysplenia.

Background: Heterotaxy with polysplenia is associated with many cardiovascular anomalies including the occasional occurrence of congenital extrahepatic portosystemic shunts (CEPS). Missing this anomaly can lead to inappropriate and ineffective therapy.

Objective: To emphasize the importance and associated anatomy of CEPS in conjunction with heterotaxy with polysplenia.

Non-congenital heart disease associated pediatric pulmonary arterial hypertension.

Recognition of causes of pulmonary hypertension other than congenital heart disease is increasing in children. Diagnosis and treatment of any underlying cause of pulmonary hypertension is crucial for optimal management of pulmonary hypertension. This article discusses the available knowledge regarding several disorders associated with pulmonary hypertension in children: idiopathic pulmonary arterial hypertension (IPAH), pulmonary capillary hemangiomatosis, pulmonary veno-occlusive disease, hemoglobinopathies, hepatopulmonary syndrome, portopulmonary hypertension and HIV.

Pulmonary arterial hypertension in children: Diagnostic work-up and challenges.

The diagnostic evaluation of a pediatric patient with suspected pulmonary arterial hypertension (PAH) is extensive but essential, given the rapid progression of the disease if left undiagnosed and untreated. The major goals of performing a complete diagnostic work-up are to confirm the diagnosis of PAH, assess disease severity, rule out associated diseases, and begin to formulate an individualized treatment plan for the pediatric patient with pulmonary hypertension. This article will provide a comprehensive review of the diagnostic work-up of the child with suspected PAH as well as a review of some of the challenges faced when assessing a child for PAH.

The pathways of interoceptive awareness.

A network of cortical brain regions, including the insula and anterior cingulate cortex (ACC), has been proposed as the critical and sole substrate for interoceptive awareness. Combining lesion and pharmacological approaches in humans, we found that the insula and ACC were not critical for awareness of heartbeat sensations. Instead, this awareness was mediated by both somatosensory afferents from the skin and a network that included the insula and ACC.

Bilateral limbic system destruction in man.

We report here a case study of a rare neurological patient with bilateral brain damage encompassing a substantial portion of the so-called "limbic system." The patient, Roger, has been studied in our laboratory for over 14 years, and the current article presents his complete neuroanatomical and neuropsychological profiles. The brain damage occurred in 1980 following an episode of herpes simplex encephalitis.

On coupling a lumped parameter heart model and a three-dimensional finite element aorta model.

Aortic flow and pressure result from the interactions between the heart and arterial system. In this work, we considered these interactions by utilizing a lumped parameter heart model as an inflow boundary condition for three-dimensional finite element simulations of aortic blood flow and vessel wall dynamics. The ventricular pressure-volume behavior of the lumped parameter heart model is approximated using a time varying elastance function scaled from a normalized elastance function.

Angina associated with left main coronary artery compression in pulmonary hypertension.

Chest pain is a common complaint in patients with pulmonary arterial hypertension (PAH). Left main coronary artery (LMCA) compression by an enlarged pulmonary artery trunk (PAT) has been associated with angina, but appropriate diagnostic and treatment approaches remain poorly defined. We present two cases of angina caused by LMCA compression from an enlarged pulmonary artery, one of which also presented with new, severe left ventricular systolic dysfunction attributed to myocardial ischemia.

Short- and long-term outcomes of necrotizing enterocolitis in infants with congenital heart disease.

Objective: Congenital heart disease is a significant risk factor for necrotizing enterocolitis in the term infant. We compared the short- and long-term necrotizing enterocolitis-specific outcomes of infants with congenital heart disease with those of neonates without congenital heart disease.

Patients And Methods: A retrospective study of 202 patients with necrotizing enterocolitis treated at our center from May 1999 to August 2007 was conducted.

Evaluation, risk stratification, and management of pulmonary hypertension in patients with congenital heart disease.

Surgical outcomes for patients with congenital heart disease have improved dramatically over the past few decades due, in part, to improvements in preoperative and postoperative management, cardiopulmonary bypass and intraoperative technique, and the development of pediatric cardiology as a subspecialty. Patients with pulmonary hypertension, however, remain a particularly challenging subgroup worldwide. Determination of operability and, when reasonable, timing of surgery, remains at times difficult.

Further lesion evidence for the neural basis of conceptual knowledge for persons and other concrete entities.

The neural underpinnings of conceptual knowledge have been studied intensively, but many unanswered questions remain. In a previous study examining recognition of persons, animals, and tools in 116 participants with unilateral brain lesions, we found no instance of a patient who manifested defective recognition in all three categories. We reasoned that the spatial distribution of the lesion loci critical for the appearance of recognition defects for these different categories explained why this 'three-way' defect could not be found in patients with unilateral lesions, and we proposed that only a suitable bilateral lesion would be likely to produce such a combined defect.

A novel non-invasive method of estimating pulmonary vascular resistance in patients with pulmonary arterial hypertension.

Background: The assessment of pulmonary vascular resistance (PVR) plays an important role in the diagnosis and management of pulmonary arterial hypertension (PAH). The main objective of this study was to determine whether the noninvasive index of systolic pulmonary arterial pressure (SPAP) to heart rate (HR) times the right ventricular outflow tract time-velocity integral (TVI(RVOT)) (SPAP/[HR x TVI(RVOT)]) provides clinically useful estimations of PVR in PAH.

Methods: Doppler echocardiography and right-heart catheterization were performed in 51 consecutive patients with established PAH.

Sailing through the Late Pleistocene: unusual historical demography of an East Asian endemic, the Chinese Hwamei (Leucodioptron canorum canorum), during the last glacial period.

Pleistocene climate fluctuations shaped the patterns of genetic diversity observed in extant species. In contrast to Europe and North America where the effects of recent glacial cycles on genetic diversity have been well studied, the genetic legacy of the Late Pleistocene for East Asia, a region of great topographical complexity and presumably milder historical climate, remains poorly understood. We analysed 3.

Prediction of pediatric death in the year after hospitalization: a population-level retrospective cohort study.

Background: The study of how the quality of pediatric end-of-life care varies across systems of health care delivery and financing is hampered by lack of methods to adjust for the probability of death in populations of ill children.

Objective: To develop a prognostication models using administratively available data to predict the probability of in-hospital and 1-year postdischarge death.

Methods: Retrospective cohort study of 0-21 year old patients admitted to Pennsylvania hospitals from 1994-2001 and followed for 1-year postdischarge mortality, assessing logistic regression models ability to predict in-hospital and 1-year postdischarge deaths.

Evaluation of a novel Y-shaped extracardiac Fontan baffle using computational fluid dynamics.

Objectives: The objective of this work is to evaluate the hemodynamic performance of a new Y-graft modification of the extracardiac conduit Fontan operation. The performance of the Y-graft design is compared to two designs used in current practice: a t-junction connection of the venae cavae and an offset between the inferior and superior venae cavae.

Methods: The proposed design replaces the current tube grafts used to connect the inferior vena cava to the pulmonary arteries with a Y-shaped graft.

Anterior insula reactivity during certain decisions is associated with neuroticism.

Neuroticism is a core personality trait that profoundly affects how individuals interpret and interact with their environment. Understanding neuroticism at a neurobiological level will be an important step toward identifying novel vulnerability factors for psychiatric illnesses such as depression and anxiety. Along these lines, recent work has identified neural activation patterns within the right anterior insula that correlates with an individual's degree of neuroticism.