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BACKGROUND AND OBJECTIVES Annual preparticipation physical evaluation (PPE) is used in the United States to screen adolescents for potential causes of sudden cardiac death. The American Heart Association recommends 14 screening elements of history and physical examination. This study sought to define the utilization of these screening elements by each of the 50 states before high school athletics. METHODS PPE forms were obtained from the public website of the high school athletics governing body in every state. Form content was analyzed to identify which of the 14 screening elements were explicitly fulfilled. Additional PPE forms provided by private/parochial schools, other professional societies, or independent groups were excluded from this study. RESULTS A total of 48 states (96%) had PPE forms posted online. The remaining 2 states (4%) deferred the specific method of PPE documentation to individual school districts and provided no standardized form. Of the 48 states providing PPE forms, 13 (27%) included all 14 American Heart Association screening elements. The median criteria included by each state was 11 (range 3–14). The 3 criteria most commonly absent were (1) the examination of femoral pulses to exclude coarctation (58%), (2) a family history of specific inherited cardiac disease (31%), and (3) personal history of hypertension (27%). CONCLUSIONS Annual preparticipation forms are important screening tools. Only a minority of states include all 14 cardiac screening elements recommended by the American Heart Association.

Williams syndrome (WS) is an arteriopathic derangement associated with supravalvular aortic stenosis and branch pulmonary stenosis. We describe double-outlet right ventricle with mitral atresia and aortic arch hypoplasia in an infant with WS. This case demonstrates the difficulty in managing patients with WS with complex cardiac defects. To our knowledge, this is the first reported single-ventricle physiology in a patient with WS. (Level of Difficulty: Advanced.), Graphical abstract, Williams syndrome (WS) is an arteriopathic derangement associated with supravalvular aortic stenosis and branch pulmonary stenosis. We describe…

Background Infants with heart failure remain at significant risk for wait list mortality, despite mechanical circulatory support (MCS). It is unclear if the outcomes are influenced by modality of support or underlying diagnosis. We sought to compare the outcomes of infants Methods and Results Using the Pediatric Heart Transplant Society database, we evaluated survival following first MCS device in children P =0.2; 5.4% versus 6.4%; P =0.4). However, there was a significant difference in the use of VAD both at listing (8% versus 2.4%; P P P P =0.01) for the CHD or the cardiomyopathy group ( P =0.38), but patients with cardiomyopathy demonstrated better survival on both forms of support. Conclusions Survival for patients

BackgroundCategorization of topographical features into landform type is a long-standing method for understanding physiographic patterns in the environment. Differences in forest composition between landform types are driven primarily by concurrent differences in soil composition and moisture, but also disturbance regime. Many studies have focused on the interaction between fire disturbance, succession, and landforms, but the effects of hurricane disturbance on compositional differences between landforms are poorly understood. In the study presented here, we assess compositional and structural differences between landform types in the tree community of a young sub-tropical forest that is frequently subjected to hurricanes. Specifically, we ask whether the tree community (1) changed structurally over the study period, (2) experienced compositional change over the study period, (3) is compositionally different between landform types, and (4) exhibits compositional change mediated by landform type.ResultsThe tree community experienced significant structural change over the course of our study, but compositional change was only significant for some landforms.ConclusionDespite large-scale, intense, and frequent hurricane disturbance to our study system, compositional change in the tree community was localized and only significant for some landform types.

ObjectiveTo enhance the understanding of cardiovascular care delivery in childhood cancer patients and survivors.Study designA 20-question survey was created by the Pediatric Cardio-oncology Work Group of the American College of Cardiology (ACC) Cardio-oncology Section to assess the care, management, and surveillance tools utilized to manage pediatric/young adult cardio-oncology patients. The survey distribution was a collaborative effort between Cardio-oncology Section and membership of the Adult Congenital and Pediatric Cardiology Section (ACPC) of the ACC.ResultsSixty-five individuals, all self-identified as physicians, responded to the survey. Most respondents (n = 58,89%) indicated childhood cancer patients are regularly screened prior to and during cancer therapy at their centers, predominantly by electrocardiogram (75%), standard echocardiogram (58%) and advanced echocardiogram (50%) (i.e. strain, stress echo). Evaluation by a cardiologist prior to/during therapy was reported by only 8(12%) respondents, as compared to post-therapy which was reported by 28 (43%,p n = 31,48%) and history of chemotherapy exposure (n = 27,42%). Of note, during post-treatment counseling, common cardiovascular risk-factors like blood pressure (31,48%), lipid control (22,34%), obesity & smoking (30,46%) and diet/exercise/weight loss (30,46%) were addressed by fewer respondents than was LV function (72%).ConclusionsThe survey data demonstrates that pediatric cancer patients are being screened by EKG and/or imaging prior to/during therapy at most centers. Our data, however, highlight the potential for greater involvement of a cardiovascular specialist for pre-treatment evaluation process, and for more systematic cardiac risk factor counseling in posttreatment cancer survivors.

Williams syndrome (WS) is an arteriopathic derangement most often associated with supravalvular aortic stenosis and branch pulmonary artery stenosis. WS has also been reported in conjunction with several types of congenital heart disease. We report a unique case of double outlet right ventricle with

Background— Pediatric data on the impact of pre–heart transplantation (HTx) risk factors on early post-HTx outcomes remain inconclusive. Thus, among patients with previous congenital heart disease or cardiomyopathy, disease-specific risk models for graft loss were developed with the use pre-HTx recipient and donor characteristics. Methods and Results— Patients enrolled in the Pediatric Heart Transplant Study (PHTS) from 1996 to 2006 were stratified by pre-HTx diagnosis into cardiomyopathy and congenital heart disease cohorts. Logistic regression identified independent, pre-HTx risk factors. Risk models were constructed for 1-year post-HTx graft loss. Donor factors were added for model refinement. The models were validated with the use of patients transplanted from 2007 to 2009. Risk factors for graft loss were identified in patients with cardiomyopathy (n=896) and congenital heart disease (n=965). For cardiomyopathy, independent risk factors were earlier year of transplantation, nonwhite race, female sex, diagnosis other than dilated cardiomyopathy, higher blood urea nitrogen, and panel reactive antibody >10%. The recipient characteristic risk model had good accuracy in the validation cohort, with predicted versus actual survival of 97.5% versus 95.3% (C statistic, 0.73). For patients with congenital heart disease, independent risk factors were nonwhite race, history of Fontan, ventilator dependence, higher blood urea nitrogen, panel reactive antibody >10%, and lower body surface area. The risk model was less accurate, with 86.6% predicted versus 92.4% actual survival, in the validation cohort (C statistic, 0.63). Donor characteristics did not enhance model precision. Conclusions— Risk factors for 1-year post-HTx graft loss differ on the basis of pre-HTx cardiac diagnosis. Modeling effectively stratifies the risk of graft loss in patients with cardiomyopathy and may be an adjunctive tool in allocation policies and center performance metrics.

Since the 1995 publication of its Core Cardiovascular Training Statement (COCATS),1 the American College of Cardiology (ACC) has played a central role in defining the knowledge, experiences, skills, and behaviors expected of all clinical cardiologists upon completion of training. Subsequent updates have incorporated major advances and revisions—both in content and structure—including, most recently, …

The kinetics of Fischer–Tropsch synthesis (FTS) over a 25%Co/Al2O3 catalyst was studied using a 1-L continuously stirred tank reactor (CSTR) under the conditions of 205–230 °C, 1.4–2.5 MPa, H2/CO = 1.0–2.5 and 3–16 NL/g-cat/h (XCO = 7–54%). Thirty-one sets of kinetic data collected at 220 °C with a low extent of deactivation were used for kinetic parameter regression. The CAER empirical kinetic model ( r F T = k P CO a P H 2 b / ( 1 + m P H 2 O / P H 2 ) ) was employed to study the kinetic effect of water. A positive kinetic water effect was first evidenced using the kinetic approach, consistent with the results of the effect of co-fed water on cobalt FTS in this work and the literature (e.g. Loegdberg et al., 2011 [21] ) for Co/Al2O3 catalysts. The current kinetic results are based on kinetic data taken following an initial catalyst induction period, where the CO conversion had stabilized. These data are different from our earlier investigations where reversible oxidation of small cobalt crystallites and/or catalyst support effects likely impacted the cobalt site densities, resulting in a negative water effect. Thus, in this study, we decoupled the effect of reversible oxidation from the kinetics, so that the effect of water on stable (i.e., presumably larger) metallic cobalt particles could be assessed. In this study, an additional eleven classical FT kinetic models for Co catalysts were tested using the kinetic data. Five of them were also found to adequately describe the kinetic data, including two mechanistic models developed based on carbide mechanisms. The CAER model containing a water effect term and the mechanistic model of Botes et al. (2009) [31] yielded comparable reaction orders for the partial pressures of H2 and CO, resulted in a better fit of the kinetic data.

Objectives Children with congenital heart disease may experience significant psychosocial morbidity related to impaired quality of life (QOL). The aim of this study was to evaluate the clinical utility of health-related QOL assessment in a pediatric cardiology outpatient clinic. Design The Pediatric Quality of Life Inventory (PedsQL) 4.0 Generic Core Scales were completed by a convenience sample of 176 patients, aged 8–18 years, being seen in a pediatric cardiology clinic. Three cardiologists enrolled in this study reviewed the completed PedsQL during the clinic visit and recorded their responses to items reported to be a problem “Often” or “Almost Always.” This utilization of the instrument was compared to standardized scoring and the practicality and perceived usefulness of the practice was evaluated by physician interview. Results PedsQL responses showed 38% of patients reporting significant (Often or Almost Always) problems on at least one domain (19% Physical Functioning, 18.2% Emotional Functioning, 11.4% Social Functioning, and 22.3% School Functioning problems). Using standardized scoring, the prevalence of scores below the cutoff score for clinically significant impaired QOL in each domain ranged from 10% to 20%, with agreement between scoring methods ranging from 89% to 93%, sensitivity 68% to 86%, and specificity 89% to 97%. Cardiologists reported interventions in 30.1% of patients. They found that the PedsQL was easy to use, did not interfere with clinic operations, required minimal time (1–5 minutes), and provided information that had an important impact on their practice in some patients. Conclusions This study demonstrates the clinical utility of health-related QOL assessment using the PedsQL in a pediatric cardiology outpatient setting. Identification of significant impairments in QOL can impact clinical decision making and may change psychosocial outcomes in children with congenital heart disease.

Malonyl coenzyme A (CoA) decarboxylase (MCD) deficiency is a rare autosomal recessive organic acidemia characterized by varying degrees of organ involvement and severity. MCD regulates fatty acid biosynthesis and converts malonyl-CoA to acetyl-CoA. Cardiomyopathy is 1 of the leading causes of morbidity and mortality in this disorder. It is unknown if diet alone prevents cardiomyopathy development based in published literature. We report a 10-month-old infant girl identified by newborn screening and confirmed MCD deficiency with a novel homozygous MLYCD mutation. She had normal echocardiogram measurements before transition to high medium-chain triglycerides and low long-chain triglycerides diet. Left ventricular noncompaction development was not prevented by dietary interventions. Further restriction of long-chain triglycerides and medium-chain triglycerides supplementation in combination with angiotensin-converting enzyme inhibitors helped to improve echocardiogram findings. Patient remained asymptomatic, with normal development and growth. Our case emphasizes the need for ongoing cardiac disease screening in patients with MCD deficiency and the benefits and limitations of current dietary interventions.

The Council of Pediatric Subspecialties (CoPS) was founded in September 2006 largely due to concerns about the nonuniformity of the fellowship application process. Working with the pediatric subspecialty community, CoPS has been successful in promoting a uniform process with many more pediatric fellowship programs now using a matching program and the Electronic Residency Application Service. More important, the organization has created a bidirectional network of communication among the pediatric subspecialties and has used this to accomplish a great deal more than improving the entry of residents into subspecialty training. CoPS has provided a united voice for the subspecialties in response to the Institute of Medicine’s Duty Hours report, participated in the development of educational conferences geared toward the subspecialist, promoted careers in the subspecialties, and worked with other pediatric organizations to advocate for improved health care for children. This article highlights CoPS’ many achievements and describes the methods it used to accomplish them, illustrating how pediatric subspecialists can develop a communication network and use this to work together to achieve common goals.

Background Cardiomyopathy is a heterogeneous disease with a strong genetic component. A research-based pediatric cardiomyopathy registry identified familial, syndromic, or metabolic causes in 30% of children. However, these results predated clinical genetic testing. Methods and Results We determined the prevalence of familial, syndromic, or metabolic causes in 83 consecutive unrelated patients referred for genetic evaluation of cardiomyopathy from 2006 to 2009. Seventy-six percent of probands (n = 63) were categorized as familial, syndromic, or metabolic. Forty-three percent (n = 18) of hypertrophic cardiomyopathy (HCM) patients had mutations in sarcomeric genes, with MYH7 and MYBPC3 mutations predominating. Syndromic (17%; n = 7) and metabolic (26%; n = 11) causes were frequently identified in HCM patients. The metabolic subgroup was differentiated by decreased endocardial shortening fraction on echocardiography. Dilated cardiomyopathy (DCM) patients had similar rates of syndromic (20%; n = 5) and metabolic (16%; n = 4) causes, but fewer familial cases (24%; n = 6) compared with HCM patients. Conclusions The cause of cardiomyopathy is identifiable in a majority of affected children. An underlying metabolic or syndromic cause is identified in >35% of children with HCM or DCM. Identification of etiology is important for management, family-based risk assessment, and screening.

There are limited data on the incidence or risk factors for IFI in pediatric heart transplant recipients. The purpose of this study was to describe the incidence and types of IFI, to determine risk factors for outcomes of IFI, and to assist in decision-making concerning the need for prophylactic strategies in pediatric heart transplant recipients. Data from a multi-institutional registry of 1854 patients transplanted between 01/93 and 12/04 were analyzed to determine risk factors and outcomes of children with IFI post-heart transplantation. One hundred and thirty-nine episodes of IFI occurred in 123 patients and made up 6.8% of the total number of post-transplant infections. IFI was most commonly attributed to yeast (66.2%), followed by mold (15.8%) and Pneumocystis jiroveci (13%). Ninety percent of the yeast infections were caused by Candida spp., and Aspergillus spp. was causative in 82% of the mold infections. There was a significantly increased risk of fungal infection associated with pretransplant invasive procedures (e.g., ECMO, prior surgery, VAD, mechanical ventilation) with an incremental risk with increasing numbers of invasive procedures (early phase 0 vs. 1, RR 1.3; 0 vs. 3, RR 2.3; p

Dilated (DCM), restrictive (RCM), and hypertrophic (HCM) cardiomyopathies (CM) in children have varying clinical courses and therapeutic options. Heart transplantation (HTx) offers a chance for long-term survival; but outcomes after listing have not been well defined.A multi-institutional registry of 3,147 patients listed for HTx (January 1993-December 2006) was used to compare outcomes of 1,320 children with CM (42%) and 1,827 with non-CM (58%) etiologies. Comparisons were made between sub-groups: 1,098 DCM (83%), 145 RCM (11%), and 77 HCM (6%).CM patients had a waitlist mortality of 17% vs 32% for non-CM patients (p0.0001), with no difference between the CM sub-groups. Risk factors were younger age, black race (relative risk [RR], 1.65; p = 0.009), mechanical ventilation (RR, 3.17; p0.001), and extracorporeal membrane oxygenation (RR, 2.16; p0.001). Ten-year survival after listing was 66% for CM vs 53% for non-CM (p0.0001). HCM and RCM patients aged1 year at the time of listing had the highest waitlist mortality and the lowest overall survival. CM patients had a better 10-year survival after HTx (68% vs 61%, p0.0001). Risk factors for death early after HTx included mechanical ventilation at HTx (RR, 3.07; p0.001), longer ischemic time (RR, 1.27; p = 0.01), and earlier era (RR, 1.77; p = 0.002). Late risk factors included black race (RR, 3.01; p0.001), HCM or RCM (RR, 1.93; p = 0.007), and older age (RR, 1.9; p0.001).Children with CM have a lower waitlist mortality and better survival post-HTx than children with a non-CM diagnosis. DCM patients have the best and HCM or RCM patients aged younger than 1 year have the worst overall outcomes.

Infantile cardiomyopathy is a genetically heterogeneous disorder with significant morbidity and mortality.This study aimed to identify the mutation present in four unrelated patients who presented as infants with isolated hypertrophic cardiomyopathy.In all four, a novel mitochondrial m.8528T--C mutation was identified. This results in a change of the initiation codon in ATPase 6 to threonine and a concurrent change from a highly conserved hydrophobic amino acid, tryptophan, at position 55 of ATPase 8 to a highly basic arginine. To our knowledge, this is the first report of a mutation affecting both mitochondrial genome-encoded complex V subunit proteins. Testing of the relatives of one patient indicated that the mutation is heteroplasmic and correlated with disease.Mitochondrial genome sequencing should be considered in patients with infantile hypertrophic cardiomyopathy.

We describe an open-chest epicardial approach to transcatheter pulmonary artery stenting in a critically ill infant following heart transplantation. Technical considerations, indications, and feasibility are discussed. This case provides another example of the value of a "hybrid" approach (combining surgery and interventional cardiology) to complex congenital heart disease.

Preamble 1523 1. Introduction 1524 2. General Standards 1526 3. Training Components 1526 4. Training Requirements 1528 5. Evaluation of Proficiency 1542 6. Maintenance of Competency 1543 References 1543 Appendix 1. Author Relationships with Industry and Other Entities (Relevant) 1545 Appendix 2. Reviewer Relationships with Industry and Other Entities (Relevant) 1548 Appendix 3. Abbreviations 1551 Since the 1995 publication of its Core Cardiovascular Training Statement (COCATS), the American College of Cardiology (ACC) has played a central role in defining …

Since the 2005 publication of the first “Training Guidelines for Pediatric Cardiology Fellowship Programs,”1 the field of pediatric cardiology has undergone significant growth and change, and thus, the Society of Pediatric Cardiology Training Program Directors (SPCTPD) in conjunction with the Joint Council of Congenital Heart Disease recommended the guidelines be revised accordingly. The SPCTPD board assembled a Steering Committee that nominated 2 chairs for each of the 8 Task Forces (7 as in the original document plus 1 for “advanced medical therapies,” [ie, heart failure, pulmonary hypertension, and cardiac transplantation]). Six to 8 members were selected from a list of potential committee members representing a wide range of program sizes, geographic regions, and subspecialty focuses. Representatives from the American College of Cardiology, American Academy of Pediatrics, and American Heart Association participated. These participants, along with 1 Steering Committee member, comprised each Task Force. A Steering Committee member was added to provide perspective to each Task Force as a “nonexpert” in that field. The authors developed the Task Force reports under guidance from the Task Force chairs, approved them for review by individuals selected by the participating organizations, and addressed the 258 comments submitted. The peer reviewers for each report are listed in an appendix in each Task Force report along with their employment information and affiliation in the review process. The final, complete document was approved by the Society of Pediatric Cardiology Training Program Directors, American Academy of Pediatrics, and the American Heart Association in February 2015 and by the American College of Cardiology in March 2015, and individual Task Force reports were endorsed by the organizations noted in each report. During the process of updating the guidelines, a paradigm shift in medical education occurred. The change to competency-based training now requires trainees to achieve an expected level of …

Long-term outcome after cardiac transplantation is limited by graft coronary vasculopathy (GCV). Hyperlipidemia may contribute to the development and progression of GCV. The purpose of this study was to determine the distribution of lipid levels in children after heart transplantation and to determine the incidence and risk factors for hyperlipidemia.We compared the distribution of lipid values 1 year after heart transplantation in children participating in Pediatric Heart Transplant Study to those of normal U.S. children and then performed a multivariable analysis to identify risk factors for hyperlipidemia.The median values of total and LDL-cholesterol in heart transplant patients were similar to those of normal U.S. pediatric population. However, total cholesterol level was200 mg/dl in 14% and170 mg/dl in 33% of transplant patients. LDL-cholesterol was130 mg/dl in 12%,110 mg/dl in 27% and100 mg/dl (greater than "optimal" levels) in 39% of transplant patients. The distribution curve for serum triglycerides was shifted to the right and that for HDL-cholesterol levels was shifted to the left of the normal U.S. pediatric curve. As a result, triglyceride levels were75th centile of U.S. normal distribution in 59% of the transplant patients. HDL-cholesterol level was25th centile of U.S. normal distribution in 61% of the transplant patients. Independent risk factors for elevated levels of total and LDL-cholesterol were patient age and use of cyclosporine and prednisone.Lipid levels considered to be in the intervention range in adults at high risk of coronary artery disease are a common finding after cardiac transplantation in children.

The objective of this study was to evaluate the safety and efficacy of carvedilol in pediatric patients with stable moderate heart failure. We performed a single-arm prospective drug trial at three academic medical centers and the results were compared to historical controls. Patients were 3 months to 17 years old with an ejection fraction

We present the anatomic constellation of mitral stenosis/aortic atresia variant of hypoplastic left heart syndrome, Ebstein's anomaly, and partial anomalous pulmonary venous return, an exceeding rare congenital heart defect. Prenatal echocardiography led to concern about the capacity of the right ventricle to increase cardiac output with lung expansion and pulmonary arterial runoff at birth, prompting the precaution of extracorporeal membrane oxygenator standby at delivery. Stage I palliation was not attempted, and control of pulmonary arterial blood flow was achieved with pulmonary artery banding, allowing sufficient ongoing hemodynamic stability. Orthotopic cardiac transplantation, repair of hypoplastic aortic arch, and primary sutureless repair of left pulmonary veins was performed, using dual-site arterial cannulation and continuous mild hypothermic cardiopulmonary bypass. We discuss how this unique echocardiographic anatomy influenced the surgical decision and point out how it guided therapy toward a strategy of primary transplantation rather than standard staged surgical palliation.

Duchenne muscular dystrophy leads to progressive deterioration in skeletal and cardiac muscle function. Steroids prolong ambulation and improve respiratory muscle strength. The authors hypothesized that steroid treatment would stabilize cardiac muscle function. Echocardiograms performed from 1997 to 2004 for 111 subjects 21 years of age or younger with Duchenne muscular dystrophy were restrospectively reviewed. The medical record was reviewed for steroid treatment. Untreated and steroids-treated subjects did not differ in age, height, weight, body mass index, systolic and diastolic blood pressure, or left ventricular mass. The shortening fraction was lower in the untreated group. Of those treated, 29 received prednisone and 19 received deflazacort. There was no difference in the shortening fraction between the two treated subgroups. Treated subjects not receiving steroids still had a normal shortening fraction, which was no different from the shortening fraction of those still receiving treatment. As compared with the treated subjects, the untreated subjects 10 years of age or younger were 4.4 times more likely to have a shortening fraction less than< 28% (p = 0.03), and the untreated subjects older than 10 years were 15.2 times more likely to have a shortening fraction less than< 28% (p < 0.01). This retrospective study suggests that the progressive decline in cardiac function of patients with Duchenne muscular dystrophy can be altered by steroid treatment. The effect appears to be sustained beyond the duration of treatment and independent of steroid type.

Pediatricians and other healthcare professionals need to be alert to the presence and progression of cardiac involvement in patients with MD. The signs and symptoms of cardiac involvement may be minimal, necessitating careful interval history, physical examination, and noninvasive cardiac testing. Available treatment strategies may reduce disease morbidity and mortality. It is reasonable to expect that a child who has skeletal muscle weakness from MD may have cardiac involvement, even if it is subclinical. Treatment of the muscular dystrophies through genetic engineering is a future dream. However, the improvements in clinical care, evaluation and treatment standards, and multidisciplinary supportive care are able to benefit the current generation of children.

Purpose: In circulations with pulsatile pulmonary artery flow the pulmonary venous wedge pressure (PVWp) has been validated as a good estimate of pulmonary artery pressure (PAp), when PAp is low. The purpose of this study was to validate PVWp estimates of PAp in the less-pulsatile pulmonary circulation of children after bidirectional Glenn shunts. Methods: A retrospective study was performed of 22 simultaneous measurements of PVWp and PAp made during 20 catheterizations in 19 children who had undergone bidirectional Glenn procedures. The PAp was measured directly from the branch PA ipsilateral to the side of the PVWp, or in the SVC. Pulmonary resistance (Rp) was calculated with both PAp and PVWp, to assess the impact of PAp estimates on Rp determinations. Results: Patients ranged in age from 5 months to 10.7 years. There were a variety of univentricular cardiac malformations in the study group. Two children had antegrade pulmonary blood flow in addition to a bidirectional Glenn shunt. The mean PAp ranged from 4 to 14 mmHg, while mean PVWp ranged from 3 to 15 mmHg. Mean PVWp never differed from mean PAp by more than 3 mmHg. There was a significant linear relation between mean PAp and PVWp: PAp = 0.86 (PVWp) + 2.0 (R2= 0.89; P < 0.0001). PVWp provided a good approximation of PAp regardless of the presence (n = 2) or absence (n = 19) of antegrade pulmonary flow. There was a good linear correlation between the Rp calculated by both methods (RpPAp = 0.9 (RpVWp) + 0.5; R2= 0.74; P < 0.0001). Conclusion: The mean PVWp provides a close approximation of mean PAp in children with a bidirectional Glenn shunt and provides valuable hemodynamic information in cases where direct PAp measurements are unavailable. (J Interven Cardiol 2003;16:367–370)

The purpose of this study was to explore the validity of diastolic indices derived from color M-mode Doppler and Doppler tissue imaging in a heterogeneous group of pediatric patients by comparing them with simultaneously obtained invasive indices of diastolic function.A total of 20 children undergoing left heart catheterization had echocardiographic images recorded simultaneously with high-fidelity left ventricular (LV) pressure tracings. Transmitral Doppler, pulmonary vein Doppler, Doppler tissue imaging, and color M-mode Doppler flow propagation velocity were recorded. LV peak negative dP/dt, the time constant of isovolumic relaxation, and LV end-diastolic pressure were compared with the echocardiographic indices.The ratio of peak E-wave mitral velocity/propagation velocity correlated significantly with LV end-diastolic pressure (r = 0.71; P.001). Propagation velocity correlated with the time constant of isovolumic relaxation (r = -0.56; P =.01) and peak negative dP/dt (r = 0.50; P.03). Septal mitral annular myocardial velocity correlated significantly with the time constant of isovolumic relaxation (r = -0.58, P =.01).The newer diastolic indices derived from color M-mode Doppler and Doppler tissue imaging appear to be a helpful adjunct in the noninvasive assessment of diastolic function in children.

Modeling of the supercritical fluid mixture indicated that an important increase in density occurs above a threshold of approximately 4 MPa for the reaction temperature of 220 °C studied. While transport parameters of the fluid are largely retained, the observed improvement in wax solubility was noteable. A cobalt catalyst (25%Co/γ-Al 2 O 3 ) was used in a fixed bed reactor under a pressure/density tuned supercritical fluid mixture of n -pentane/ n -hexane. By using inert gas as a balancing gas to maintain a constant pressure, the density of the supercritical fluid could be tuned near the supercritical point while maintaining constant space velocity within the reactor. The benefits of the mixture allowed for optimization of transport and solubility properties at an optimum reaction temperature for Fischer–Tropsch synthesis with a cobalt catalyst. Indeed, above 4 MPa, increases in wax yields from sampling and carefully controlled gas measurements using an internal standard demonstrated an important increase in conversion due to greater accessibility to active sites after extraction of heavy wax from the catalyst. Additional benefits included decreased methane and carbon dioxide selectivities. Decreased paraffin/(olefin+paraffin) selectivities with increasing carbon number were also observed, in line with extraction of the hydrocarbon from the pore. Faster diffusion rates of wax products resulted in lower residence times in the catalyst pores, and therefore, decreased probability for readsorption and reaction to the hydrogenated product. Even so, there was not an observable increase in the alpha value for higher carbon number products over that obtained with just the inert gas.

Children with end-stage cardiac failure are at risk of HA and PG. The effects of these factors on post-transplant outcome are not well defined. Using the PHTS database, albumin and growth data from pediatric heart transplant patients from 12/1999 to 12/2009 were analyzed for effect on mortality. Covariables were examined to determine whether HA and PG were risk factors for mortality at listing and transplant. HA patients had higher waitlist mortality (15.81% vs. 10.59%, p = 0.015) with an OR of 1.59 (95% CI 1.09-2.30). Survival was worse for patients with HA at listing and transplant (p ≤ 0.01 and p = 0.026). Infants and patients with congenital heart disease did worse if they were HA at time of transplant (p = 0.020 and p = 0.028). Growth was poor while waiting with PG as risk factor for mortality in multivariate analysis (p = 0.008). HA and PG are risk factors for mortality. Survival was worse in infants and patients with congenital heart disease. PG was a risk factor for mortality in multivariate analysis. These results suggest that an opportunity may exist to improve outcomes for these patients by employing strategies to mitigate these risk factors.

Iron oxide Fischer-Tropsch catalysts were prepared using a sol-gel method to incorporate varying amounts of silica (SiO2) and a constant amount of potassium oxide (K2O) promoters. The sol-gel slurries containing different Fe/Si ratios were dried using a spray dryer. The spray-dried materials were calcined at 300°C prior to the use as catalysts for the Fischer-Tropsch reaction. Scanning electron microscopy (SEM), combined with energy dispersive X-ray spectroscopy (EDS) and elemental dot mapping (EDM) techniques, was utilized to characterize the structural morphology for these promoted iron oxide catalysts. The results indicate that the promoters (SiO2, k2O) are evenly distributed throughout the catalysts, and no agglomerated regions of iron, silica or potassium could be observed. Thus, the sol-gel preparation, followed by spray drying produces industrial catalysts of fine particle morphology and a uniform distribution of the promoters.

Low-temperature (230°C), slurry phase Fischer-Tropsch synthesis (FTS) was conducted with precipitated iron-silicon catalysts under industrially relevant conditions (flow=3.1 Nl h −1 g-Fe −1 , H 2 :CO=0.7, P=1.31 MPa). The effects of activation gas (hydrogen, carbon monoxide, or syngas) and promoters (potassium and copper) on activity and selectivity were explored. Optimum potassium promotion was 4–5 at%, relative to iron. Promotion with copper lowered the reduction temperature and increased FTS activity, regardless of the activation gas used. Carbon monoxide activation gave the highest activity for a 100Fe/ 4.4Si/5.2K catalyst (atomic percent, relative to iron) while syngas activation was superior for a 100Fe/4.4Si/2.6Cu/5.2K catalyst. Selectivity of the FTS product was not affected by the activation gas employed or copper promotion; however, potassium promotion increased wax and alkene selectivity. A syngas activated 100Fe/4.4Si/2.6Cu/5.2K catalyst gave the best overall performance at 230°C. Alkene selectivity was >75% for the C 2 -C 11 fraction, methane selectivity was 12 + selectivity was >70 wt%.

Williams syndrome (WS) is a recognizable pattern of malformation with mental retardation, mild growth deficiency, characteristic facies and temperament, and cardiovascular disease. Sudden death is a recognized complication of WS; however, it is thought to be rare. The clinical features of 10 children with WS who died suddenly are reported here, doubling the number of unexpected deaths reported in the literature. We suggest that sudden death is a more common complication than has been assumed previously. Pathologic findings on the seven autopsy cases implicate two anatomic abnormalities that predispose individuals with WS to sudden death: coronary artery stenosis and severe biventricular outflow tract obstruction. The mechanisms for sudden death for both anatomic subgroups include myocardial ischemia, decreased cardiac output, and arrhythmia. We believe these observations warrant the development of strategies for monitoring patients with WS in an attempt to identify those at increased risk of sudden death.

Unsupported iron Fischer-Tropsch synthesis catalyst, formed to a spherical shape and calcined in air, were carbided at 270°C with CO for 24 hours. Following carbiding, the material retained the shape and size of the calcined metal oxide. During agitation by rolling of a slurry, the average particle diameter decreased by about 50% during 450 hours; the attrited material retained a spherical shape so that size reduction was not due primarily to particle fracture. The average particle size (1/d3) was approximately linearly related to the rolling time, and the particle size distribution was fitted by the Weibull Probability Function.

During the reaction of Fe(II) with oxygen, three regions of nearly constant pH are observed, each separated by two regions where there is an abrupt pH change. Data for the oxidation of a suspension with [Fe(II)] [OH −1 ] = 7 2 and 0.22 M Fe(II) indicate that the oxidation to Fe(III) and the associated hydrolysis to produce a proton are rapid compared to the reaction that consumes the proton generated during the first step. It is suggested that the oxidation occurs in the aqueous solution, and that the slow step is due to the proton reacting with a solid Fe(II) species to produce soluble Fe(II). Fe3O4 is formed at, or near, the first step in the oxidation-pH curve, and then redissolves as oxidation continues. The amount of Fe(II) and Fe(III) in the solid and solution have been determined at several stages of the oxidation, and these are consistent with the amount of oxygen consumed during the reaction.

Electron microscopy data reveal a pattern of changes in the particle shape/morphology during the preparation of γ-FeOOH. The initial shapeless material develops to form large thin hexagonal crystals which are shown to be Green Rust-II (GR-II). With the initial, sudden pH decrease from about 8 to 6.5 at about 33% conversion of the total amount of Fe 2+ that is oxidized, small, dense, hexagonal Fe 3 O 4 particles are formed in addition to, or instead of, the GR-II hexagonal crystals. The large thin hexagonal crystals of GR-II contain holes. The disappearance of Fe 3 O 4 and GR-II particles with further oxidation is accompanied by the development of needle-like γ-FeOOH particles. The formation of γ-FeOOH by a pathway involving only GR-II is therefore an oversimplification of the actual process.

Pleural effusions after the modified Fontan procedure are unpredictable, increase morbidity, and prolong hospital stay. To assess the relation between preoperative characteristics and postoperative pleural drainage, we performed a retrospective study of 71 patients who underwent Fontan procedures. Analyses revealed no significant relation between duration of effusion and age at Fontan, preoperative oxygen saturation, pulmonary artery pressure, ventricular end-diastolic pressure, type of Fontan, or prior cavopulmonary anastomosis. Patients with significant aortopulmonary collateral vessels evidenced by angiographic opacification of the pulmonary arteries or veins had more prolonged pleural drainage. The duration of the pleural drainage was significantly less in patients who had aortopulmonary collateral occlusion.

Slurry phase Fischer−Tropsch synthesis (FTS) was conducted with two precipitated iron catalysts (100 Fe/3.6 Si/0.71 K and 100 Fe/4.4 Si/1.0 K, atomic percent relative to Fe) at 543 K, 1.31 MPa, and a synthesis gas (H2/CO = 0.7) space velocity of 3.1 normal L h-1 (g of Fe)-1. The impact of activation gas (CO, H2/CO = 0.7, or H2/CO = 0.1), temperature (543 or 573 K), and pressure (1.31 or 0.10 MPa) on the long-term (>500 h) activity and selectivity of the catalysts was explored. Pretreatment with CO under the conditions employed gave highly active and stable catalysts. Catalyst performance when synthesis gas activation was used was found to be dependent upon the partial pressure of hydrogen in the activating gas, with low hydrogen partial pressures resulting in the highest catalyst activity. X-ray diffraction results indicate that carbon monoxide activations and synthesis gas activations with low hydrogen partial pressure result in the formation of the carbides χ-Fe5C2 and e‘-Fe2.2C, while activation with s...

Background The modified Fontan procedure has become the treatment of choice for patients born with a univentricular heart. Although the operative mortality has steadily decreased in recent years, the hospital stay is still prolonged in many patients due to fluid retention and pleural effusions. Methods We retrospectively analyzed subsets of patients undergoing the bidirectional cavopulmonary shunt (BDCPS) and modified Fontan procedure in an attempt to define factors influencing operative mortality and morbidity. Results Multivariate analysis of 64 patients undergoing BDCPS revealed that age 6 months or less, concomitant operation, mean pulmonary pressure of 15 mm Hg or less, and mean pulmonary artery ratio of 1.8:1 or less were not statistically significant indicators of risk. Abnormal pulmonary artery architecture was a significant predictor of early and late death (p ≤ 0.01). Retrospective analysis of 71 patients undergoing the modified Fontan procedure revealed no significant relationship between duration of pleural effusions and age at operation, preoperative oxygen saturation, pulmonary artery pressure, ventricular end-diastolic pressure, or prior BDCPS. Patients with important aortopulmonary collateral vessels defined by angiography had prolonged pleural drainage. Selective use of the total extracardiac conduit and the fenestrated Fontan resulted in low overall mortality with no statistical differences between subsets of patients undergoing different modifications of the Fontan procedure. Conclusions These data indicate that the operative risk for BDCPS or modified Fontan procedure is quite low if a procedure appropriate for the patient is selected. Abnormal pulmonary artery architecture is an important risk factor for death after the BDCPS. Aortopulmonary collateral vessels are associated with fluid retention and pleural effusions after a modified Fontan procedure. Important aortopulmonary collateral vessels should be occluded before or during the modified Fontan procedure.

The data presented in this study illustrate the similarities and differences in the yield and selectivities obtained from different types of coal liquefaction reactors. The results suggest that the comparison of data in the literature obtained from different reactors should be done with careful consideration. The differences and similarities in the yields obtained depend not only on the reactor type but also on the feedstock employed and the residence time in the reactors. Data generated using microreactors are adequate for the selection of operating conditions for conversion in larger scale reactors.

ABSTRACT A naphtha derived from the liquefaction of a subbituminous (Black Thunder) or a bituminous coal (III. #6) was hydrotreated on a pilot plant scale, to provide a feedstock sufficiently low in heteroatoms for further studies in reforming. Two commercial catalysts, a Ni/Mo and a Co/Mo on alumina, were employed in the processing of the naphtha samples. The Black Thunder naphtha was processed for over 120 hours, twice using the Co/Mo catalyst and once using the Ni/Mo catalyst. For this naphtha, the removal of nitrogen was extremely difficult using the catalysts and conditions employed in this study. An average of 51.6% of the nitrogen was removed during each of the three passes. The oxygen compounds in this naphtha were relatively easily removed. During the first pass over the Co/Mo catalyst, 94.0% of the oxygen was removed.