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Kommerell diverticulum (KD) is a dilated proximal aberrant right or left subclavian artery associated with either right or left aortic arches (RAA-ARSA or LAA-ALSA). Although case series suggest that KD may be a liability for vascular complications, the risk, pattern of dilation throughout the life span, and differences between arch sides are not known.This study was a single-center retrospective review of patients of all ages with KD on cross-sectional imaging. Maximal short-axis diameter of KD (KDmax), absolute and indexed to descending aortic diameter (DAo), was correlated with age. Comparisons were made between arch sides. Patients with vascular complications were described.A total of 104 patients with KD were included: 68 (65%) with RAA-ALSA, 36 (35%) with LAA-ARSA, 43 (41%) asymptomatic. Although KDmax was correlated with age (RAA-ALSA r = 0.84; [P.0001]; LAA-ARSA r = 0.51 [P = .001]), KDmax indexed to DAo was not (RAA-ALSA r = 0.14 [P = .27]; LAA-ARSA r = -0.22 [P = .21]). Patients with RAA-ALSA had larger KDmax indexed to DAo (1.02 ± 0.20 mm/mm vs 0.89 ± 0.18 mm/mm; P = .002) and more symptoms (75% vs 28%; P.0001), and they were younger (median, 9.5 years vs 61.7 years; P.0001). Six patients (58 to 80 years of age) had vascular complications, and all 6 had LAA-ARSA and risk factors for acquired aneurysms.In older patients, KDmax indexed to DAo was not larger, thus arguing against isolated KD dilation with age. Diverticula from RAA-ALSA and LAA-ARSA demonstrated different phenotypes, a finding suggesting different disease processes and likely different risk. The incidence of vascular complications was lower than in previous reports, and these complications occurred exclusively in patients with LAA-ARSA and aneurysm risk factors. This finding suggests that conservative management of asymptomatic KD is often reasonable, especially in patients with RAA-ALSA.

Introduction: To promote the rational use of cardiovascular imaging in the delivery of high-quality and cost-effective care to patients with congenital heart disease (CHD), the American College of Cardiology developed Appropriate Use Criteria (AUC). We aimed to evaluate the appropriateness of indications for cardiac CT and MRI in patients with conotruncal defects and to identify factors associated with Maybe or Rarely Appropriate (M/R) indications. Methods: Twelve US centers contributed a median of 147 studies performed prior to AUC publication (01/2020) on patients with conotruncal defects. Studies were excluded if the patient did not receive primary cardiac care at the center or were not seen > 5 years prior to study date. To account for the nesting of physicians within center, a hierarchical generalized linear mixed model was used to account for center-level variation with physicians nested within center. Results: Of the 1,753 studies (20% CT, 80% MRI) from 12 centers, 16% (range 4-39%) were rated M/R and 2.4% were unclassifiable (Figure 1). Patients age Conclusions: Most cardiac CTs and MRIs ordered for the follow-up care of patients with conotruncal defects were rated Appropriate and nearly all were able to be classified. However, there was significant center-level variation and higher annual MRI volume was independently associated with lower odds of M/R rating. These findings inform future quality improvement initiatives.

Elevated left ventricular end diastolic pressure is a risk factor for ventricular arrhythmias in patients with tetralogy of Fallot. The objective of this retrospective study was to identify echocardiographic measures associated with left ventricular end diastolic pressure >12 mmHg in this population. Repaired tetralogy of Fallot patients age ≥13 years, who underwent a left heart catheterisation within 7 days of having an echocardiogram were evaluated. Univariate comparison was made in echocardiographic and clinical variables between patients with left ventricular end diastolic pressure >12 versus ≤12 mmHg. Ninety-four patients (54% male) with a median age of 24.6 years were included. Thirty-four (36%) had left ventricular end diastolic pressure >12 mmHg. Patients with left ventricular end diastolic pressure >12mmHg were older (median 32.9 versus 24.0 years, p = 0.02), more likely to have a history of an aortopulmonary shunt (62% versus 38%, p = 0.03), and have a diagnosis of hypertension (24% versus 7%, p = 0.03) compared to those with left ventricular end diastolic pressure ≤12 mmHg. There were no significant differences in mitral valve E/A ratio, annular e’ velocity, or E/e’ ratio between patients with left ventricular end diastolic pressure >12 versus ≤12 mmHg. Patients with left ventricular end diastolic pressure >12mmHg had larger left atrial area (mean 17.7 versus 14.0 cm2, p = 0.03) and larger left atrium anterior–posterior diameter (mean 36.0 versus 30.6 mm, p = 0.004). In conclusion, typical echocardiographic measures of left ventricular diastolic dysfunction may not be reliable in tetralogy of Fallot patients. Prospective studies with the use of novel echocardiographic measures are needed.

Background:Fungal endocarditis classically involves dense heterogenous vegetations. However, several patients with fungal infections were noted to have myocardial changes ranging from focal brightening to nodular thickening of chordae or papillary muscles. This study evaluates whether these findings are associated with fungal infections.Methods:In a retrospective case–control study, paediatric inpatients with fungal infections (positive blood, urine, or catheter tip culture) in a 5-year period were matched 1:1 to inpatients without positive fungal cultures. Echocardiograms were scored on a 5-point scale by two independent readers for presence of myocardial brightenings, nodular thickenings, and vegetations. Clinical data were compared.Results:Of 67 fungal cases, positive culture sites included blood (n = 44), vascular catheter tip (n = 7), and urine (n = 29); several had multiple positive sites. “Positive” echo findings (score ≥ 2+) were more frequent in the Fungal Group (33 versus 18%, p = 0.04). Fungal Group patients with “positive” versus “negative” echo findings had similar proportion of bacterial infections. Among fungal cases, those with “positive” echo findings had longer hospital length of stay than cases with “negative” echos (median 58 versus 40 days, p = 0.03) but no difference in intensive care unit admission, extracorporeal membranous oxygenation support, or mortality.Conclusions:Myocardial and papillary muscle brightening with nodular thickening on echocardiogram appear to be associated with fungal infections. There may be prognostic implications of these findings as patients with “positive” echo have longer length of stay. Further studies are needed to better understand the mechanism and temporal progression of these changes and determine the prognostic value of this scoring system.

Background Fontan-associated liver disease (FALD) is universal in patients with a Fontan circulation. Hepatocellular carcinoma (HCC) is one of its severe expressions, and, though rare, frequently fatal. The purpose of this study was to describe the clinical presentation, risk factors, and outcomes of HCC in patients with a Fontan circulation. Methods A multicenter case series of Fontan patients with a diagnosis of HCC formed the basis of this study. The case series was extended by published cases and case reports. Clinical presentation, tumor characteristics, laboratory and hemodynamic findings as well as treatment types and outcomes, were described. Results Fifty-four Fontan patients (50% female) with a diagnosis of HCC were included. Mean age at HCC diagnosis was 30 ± 9.4 years and mean duration from Fontan surgery to HCC diagnosis was 21.6 ± 7.4 years. Median HCC size at the time of diagnosis was 4 cm with a range of 1 to 22 cm. The tumor was located in the right hepatic lobe in 65% of the patients. Fifty-one percent had liver cirrhosis at the time of HCC diagnosis. Fifty percent of the patients had no symptoms related to HCC and alpha-fetoprotein was normal in 26% of the cases. Twenty-six patients (48%) died during a median follow-up duration of 10.6 (range 1–50) months. Conclusions HCC in Fontan patients occurs at a young age with a 1-year survival rate of only 50%. Meticulous liver surveillance is crucial to detect small tumors in the early stage.

Background: Bicuspid aortic valve (BAV) is associated with an increased risk of aortic dissection, but little is known about pregnancy-associated outcomes. Methods: ICD codes and a text recognition program (EMERSE) were used to identify women with BAV and pregnancy (2000-2018) at a single center. Charts were manually reviewed to confirm the diagnoses, echocardiographic data, co-morbidities, and outcomes. Inclusion criteria were congenital BAV pathology and pregnancy. Primary endpoints included expansion of aortic aneurysm and aortic dissection during pregnancy and up to 6 weeks post-partum. Results: We identified 622 women with diagnoses of BAV and pregnancy. After detailed review, 72 women (87 pregnancies) met inclusion criteria. Mean gestational age at delivery was 36.9 weeks. Ten (11.5%) patients had hypertensive disorders of pregnancy, 7 (8.0%) had gestational diabetes, and 25 (28.7%) were treated with beta-blockers. Two (2.3%) pregnancies had intra-uterine growth restriction. Vaginal deliveries occurred in 56.5% and cesarean section in 36.3%. No Type A or B aortic dissections occurred. During pregnancy, there were no significant changes in the aortic dimensions. Prior to pregnancy, 6 women had aortic measurements > 40mm (mean pre-pregnancy 40.8 ± 3.2 mm; mean post-pregnancy 42.5 ± 3.9 mm). In this higher-risk group, mean gestational age was 38.7 weeks, 4 (66.7%) had vaginal delivery, 1 (16.7%) had preeclampsia, 4 (66.7%) were treated with beta-blockers, and all breastfed. There were no delivery complications, no aortic dissections, and no maternal or fetal deaths. Conclusions: Women with BAV appear to be at relatively low risk for acute aortic dissection during pregnancy.

Wetland restoration aims to recreate or enhance valuable ecosystem services lost during wetland destruction. Regaining wetland ecosystem services depends on restarting basic wetland functions, like carbon (C) storage, which are unmeasured in many Wetlands Reserve Program (WRP) restoration sites. We collected soil and plant data from 17 WRP sites in western New York that were used for tillage or non-tillage agriculture and then actively restored as isolated depressional wetlands by excavating basins and disabling drainage systems. Sites had been restored for 0–15 years when sampled in August-October 2010. We analyzed data as chronosequences and tested whether soil and vegetation parameters in restored wetlands, over time, (1) departed from pre-restoration baselines, estimated using active agricultural fields paired to each WRP site, and (2) converged towards “natural” benchmarks, estimated from four naturally-occurring wetlands. Restored WRP soils remained similar to agricultural soils in organic matter, density, moisture, and belowground plant biomass across chronosequences, indicating negligible C storage and belowground development for 15 years following restoration. Soil changes were limited in sites restored after both tillage and non-tillage agriculture and throughout the upland meadow, emergent shoreline, and open-water habitat zones that characterize these sites. Many plant metrics like aboveground biomass matched natural wetlands within 15 years, but recovered inconsistently among tilled and untilled sites and across all habitat zones, suggesting land-use history impacts and/or zonation effects. Disparities in recovery times exists between vegetation, which can respond quickly to wetland restoration, and underlying soils, which show limited signs of recovery 15 years after being restored.

Objective We sought to identify a time during cardiac ejection when the instantaneous pressure gradient (IPG) correlated best, and near unity, with peak-to-peak systolic ejection gradient (PPSG) in patients with congenital aortic stenosis. Noninvasive echocardiographic measurement of IPG has limited correlation with cardiac catheterization measured PPSG across the spectrum of disease severity of congenital aortic stenosis. A major contributor is the observation that these measures are inherently different with a variable relationship dependent on the degree of stenosis. Design Hemodynamic data from cardiac catheterizations utilizing simultaneous pressure measurements from the left ventricle (LV) and ascending aorta (AAo) in patients with congenital valvar aortic stenosis was retrospectively reviewed over the past 5 years. The cardiac cycle was standardized for all patients using the percentage of total LV ejection time (ET). Instantaneous gradient at 5% intervals of ET were compared to PPSG using linear regression and Bland-Altman analysis. Results A total of 22 patients underwent catheterization at a median age of 13.7 years (interquartile range [IQR] 10.3-18.0) and median weight of 51.1 kg (IQR 34.2-71.6). The PPSG was 46.5 ± 12.6 mm Hg (mean ± SD) and correlated suboptimally with the maximum and mean IPG. The midsystolic IPG (occurring at 50% of ET) had the strongest correlation with the PPSG ( PPSG = 0.97(IPG50%)–1.12, R2 = 0.88), while the IPG at 55% of ET was closest to unity ( PPSG = 0.997(IPG55%)–1.17, R2 = 0.87). Conclusions The commonly measured maximum and mean IPG are suboptimal estimates of the PPSG in congenital aortic stenosis. Using catheter-based data, IPG at 50%–55% of ejection correlates well with PPSG. This may allow for a more accurate estimation of PPSG via noninvasive assessment of IPG.

Background: Atrial fibrillation and atrial flutter (AF) during pregnancy may confer increased risk of perinatal complications, particularly since pregnancy is a known hypercoagulable and arrhythmogenic state. However, few studies of outcomes among this population exist, and the optimal management strategy is unclear. We sought to determine the risk of adverse outcomes among pregnant women with AF. Methods: In this single-center study, patients with documented AF in pregnancy (1/1998-9/2018) were retrospectively analyzed. Charts were manually reviewed for demographics, comorbidities, underlying cardiac disease, and medications. Outcomes included arrhythmias, need for cardioversion, and thromboembolic events. Results: Of 66 pregnant women with documented AF, 20 (30.3%) had a diagnosis of AF prior to pregnancy and 46 (69.6%) had new onset AF during pregnancy or immediately postpartum (within six weeks of delivery). Nine women (13.6%) had persistent AF. Thirty women (45.4%) had valve disease and/or congenital heart disease and/or preexisting cardiomyopathy, and eight (12.1%) had AF precipitated by active infection or severe nausea/vomiting. Comorbidities included diabetes mellitus (3%), hypertension (9%), thyroid disease (13.6%), and obesity (32.6%). Electrical cardioversion was performed in 10 women before or after pregnancy, and five women underwent electrical cardioversion during their first or second trimesters of pregnancy. Rate-controlling medications were prescribed in 75.7%, with the most common medication being metoprolol (39.3%). Anticoagulation strategies varied from no intervention (53%), low-dose aspirin monotherapy (18.2%), enoxaparin (13.6%), warfarin (13.6%), or warfarin plus aspirin (3%). AF with rapid rate occurred during labor/delivery in 6/44 (13.6%) women with available telemetry data. One woman had a stroke during pregnancy related to vertebral artery dissection; she was in sinus rhythm at presentation. No other thromboembolic complications occurred. Conclusion: Pregnant women with AF may require rate-controlling medication and/or cardioversion during pregnancy, but appear to be at low risk for thromboembolic complications when appropriately anticoagulated. Larger studies of pregnant women both with and without underlying structural heart disease are needed to further assess complications and thromboembolic risk to optimize management of AF in pregnancy.

We previously reported common knowledge deficits and lack of transition readiness in 13- 25-year-olds with congenital or acquired heart disease. The aims of this study were to re-evaluate transition readiness in this cohort at follow-up and to examine the relationship between changes in transition readiness and quality of life (QOL).In this prospective cohort study, patients completed the Transition Readiness Assessment and the Pediatric Quality of Life Inventory using an e-tablet, web-based format at a routine follow-up visit. Changes from initial to follow-up scores were evaluated.Sixty-five percent of patients (106 of 164) completed follow-up assessments at a median age of 18.7 years (IQR, 16.5-21.2 years) at a median follow-up of 1 year. The average perceived knowledge deficit score (percent of items with no knowledge) at follow-up was 18.0 ± 15.2%, which decreased from 24.7 ± 16.5% (P .0001). On a 100-point scale, the mean score for self-efficacy increased from 71.4 ± 17.0 to 76.7 ± 18.2 (P = .0004) and for self-management increased from 47.9 ± 18.4 to 52.0 ± 20.7 (P = .004). Although physical QOL did not change, the mean psychosocial QOL score increased significantly (P = .02). A decrease in the knowledge deficit score at follow-up was significantly associated with an increased psychosocial QOL score (P = .03). An increase in the self-efficacy score was associated with an increase in psychosocial QOL score (P = .04), especially social QOL (P = .02).Although deficits in knowledge and self-management skills persist, transition readiness assessment and recognition of deficits can improve transition readiness with improved psychosocial QOL.

Sinus tachycardia is common during and after pregnancy; however, elevated heart rate (HR) may also indicate underlying illness. It is unknown whether an elevated HR in the setting of pregnancy is associated with severity of cardiomyopathy. All patients with peripartum cardiomyopathy (PPCM) and EKG

Epicardial fat produces multiple proinflammatory cytokines and is associated with adverse cardiovascular events. Inflammation and resultant endothelial dysfunction may play a role in progressive myocardial dysfunction among adults with single ventricle physiology after Fontan palliation, but the potential impact of increased epicardial fat volume (EFV) has not been studied. This study sought to determine if there is greater EFV in Fontan patients compared with a group of repaired tetralogy of Fallot (rTOF) patients. We retrospectively measured EFV manually on cardiac magnetic resonance imaging in Fontan patients, ≥15 years, and 1:1 age, sex, and body mass index–matched patients with rTOF. EFV was indexed to body surface area. A random subset of studies was re-measured to assess intra- and interobserver reliability. Fontan patients (n = 63, median age 21.6 years, 51% male, mean body mass index 24.2 ± 5.6 kg/m 2 ) had a larger indexed EFV compared with matched rTOF patients (75.3 ± 29.2 ml/m 2 vs 60.0 ± 19.9 ml/m 2 , p = 0.001). In Fontan patients, indexed EFV was inversely correlated with ventricular ejection fraction (r = −0.26, p = 0.04) and cardiac index (r = −0.33, p = 0.01). Intra- and interobserver reliabilities of the indexed EFV measurements in both groups were excellent (intraclass correlation coefficient ranges from 0.93 to 0.97). In conclusion, indexed EFV is higher in Fontan patients compared with patients with rTOF and is associated with lower ventricular ejection fraction and cardiac index. Increased EFV could play a role in the failing Fontan circulation, but longitudinal studies are necessary to establish any causative role.

Objective. Valve repair for pediatric patients with Ebstein anomaly has historically yielded varied results. The cone reconstruction (CR) first described by Da Silva has revolutionized the surgical approach to these patients. This study reports our recent experience with CR in children and young adults with Ebstein anomaly. Design. Electronic medical records were reviewed for all patients 5 mm Hg) was present in 6 patients. There was one late death (motor vehicle accident) and one late re-repair of the tricuspid valve 4 years after initial operation. Conclusions. CR in children and young adults with Ebstein anomaly can be performed with low early mortality and excellent durability at short-term follow-up. CR represents an important surgical option for young patients. It is applicable to patients with a broad range of anatomic variability and precludes valve replacement in the vast majority. CR should be considered prior to the deleterious effects of chronic right ventricular volume overload and the development of systolic dysfunction, which hamper long-term prognosis. Therefore, early referral for surgical evaluation is recommended.

Background Although surgical patch arterioplasty is the conventional therapy for branch pulmonary artery (PA) stenosis, limited data exist on long-term outcomes. We examined the incidence of and risk factors for reintervention after surgical arterioplasty for branch PA stenosis in biventricular congenital heart disease. Methods This retrospective cohort study included patients with 2-ventricle physiology who underwent patch arterioplasty for PA stenosis at a single high-volume congenital heart center during a 10-year period. Freedom from surgical or percutaneous reintervention for recurrent PA stenosis was evaluated. Univariate and multivariable Cox regression analyses were performed to determine risk factors for reintervention. Results Among 135 patients, the median age at patch arterioplasty was 0.9 years. Survival to hospital discharge (or 30 days postoperatively) was 96%. During a median follow-up period of 4.0 years, reintervention for PA stenosis occurred in 38 of 115 patients (33%) at a median time to reintervention of 1.4 years. The overall 10-year reintervention rate was 54%. In univariate analysis, age less than 30 days at initial arterioplasty, congenital PA stenosis (vs acquired), and bilateral PA stenosis (vs unilateral) were significantly associated with reintervention. In multivariable analysis, neonatal age (adjusted hazard ratio, 3.6; p = 0.002) and bilateral PA stenosis (adjusted hazard ratio, 2.8; p = 0.005) remained independently associated with reintervention. Conclusions Long-term reintervention for recurrent PA stenosis after patch arterioplasty is common. Patients with bilateral PA stenosis or age younger than 30 days at the time of the index pulmonary arterioplasty are at higher risk for reintervention. These patients may benefit from frequent monitoring or novel approaches to repair.

Background and aims We determined the relationship between site N supply and decomposition rates with respect to controls exerted by environment, litter chemistry, and fungal colonization. Methods Two reciprocal transplant decomposition experiments were established, one in each of two longterm experiments in oak woodlands in Minnesota, USA: a fire frequency/vegetation gradient, along which soil N availability varies markedly, and a long-term N fertilization experiment. Both experiments used native Quercus ellipsoidalis E.J. Hill and Andropogon gerardii Vitman leaf litter and either root litter or wooden dowels. Results Leaf litter decay rates generally increased with soil N availability in both experiments while belowground litter decayed more slowly with increasing soil N. Litter chemistry differed among litter types, and

Woody plant expansion is a global phenomenon and has been demonstrated to have impacts on the global carbon (C) cycle as a substantial C sink. Land-cover change in western New York has followed a pattern common to the northeastern US as presettlement forests were extensively cleared for agriculture use. In the past several decades, a substantial portion of this agricultural land has been left to natural succession. This study investigates soil C dynamics across a chronosequence of habitats representing this land-cover change including old fields, shrublands, and early successional forests. Despite substantial shifts in plant community composition and structure, neither soil respiration nor soil organic matter changed significantly with habitat type. While consequences of this land-cover change in western New York remain inconclusive, the scale of this change could result in substantial shifts in regional ecosystem C dynamics.

Plant nutrient resorption, a ubiquitous mechanism of nutrient conservation, has often been proposed to be more pronounced in infertile than fertile habitats, and in species common to infertile compared to fertile habitats, because of the presumed advantage when nutrients are scarce. However, previous studies provide weak and inconsistent empirical support for these hypotheses, although few have examined intraspecific variation across well-quantified resource gradients. This study addresses intraspecific patterns of nutrient resorption for eight species across two N availability gradients on similar soils in an N-limited oak savanna ecosystem: a long-term fire frequency gradient with a negatively correlated N fertility gradient and a long-term N fertilization gradient. We hypothesized that both resorption proficiency (the minimum nutrient level retained in a senesced leaf) and efficiency (the proportional change in leaf nutrient concentration) would decrease with increasing soil N availability and plant N status. For the seven non-N fixers, either resorption proficiency or efficiency decreased modestly in treatments with higher N availability. In contrast, the legume Amorpha canescens Pursh had higher N levels in green and senesced leaves, and resorbed N much more weakly than the non-fixers, and did not respond in terms of proficiency or efficiency to soil N availability. Across all species and sites in each N fertility gradient, a scaling analysis showed greater resorption efficiency in plants with lower N concentrations. Our data suggest that species can have modest resorption responses reflective of soil nutrient availability and differences in resorption related to their N economy that represent mechanisms of nutrient conservation in nutrient-limited soils.

Lifelong specialized care is indispensable to optimize life expectancy and quality of life for a growing number of chronic conditions including congenital cardiac conditions. Provision of seamless continuous care along the patients’ life spectrum, from birth until death, is challenging. Transfers of care from pediatric, over adolescent, to adult-focused and even geriatric care make patients vulnerable for prolonged gaps in cardiac care or for discontinuation of cardiac care. Care gaps are observed in all chronic medical conditions, and among those with congenital heart disease (CHD) studies reported that up to 76 % of adolescents experienced care gaps or are even completely lost to follow-up. A median proportion of 42 % of young persons with CHD experience such discontinuous cardiac care. Care gaps in chronically ill populations are associated with an increased risk for serious comorbidities and a need for urgent interventions. In patients with CHD, such care gaps might aggravate disease burden, as the complex nature of the heart defects and its inherent high risk for comorbidity onset warrant close and sustained follow-up. This chapter describes the phenomenon of (dis)continuity of care by reviewing the current body of evidence on operational definitions for care gaps and the magnitude of this problem in adolescents with CHD. Furthermore, the clinical impact of discontinuation of cardiac care is described as well as predictors identified in empirical studies. Finally, as strategies are developed for improving CHD outcomes including quality of life, the patients’ perspectives on reasons for leaving or returning to cardiac care are described.

In recent decades, substantial areas of North American tallgrass prairie have been lost to the establishment and expansion of woodlands and forests, including those dominated by eastern redcedar (Juniperus virginiana). This shift in dominant plant life form, from C4 grasses to coniferous trees, may be accompanied by changes in productivity, standing stocks of biomass and nutrients and biogeochemical cycles. The goal of this study was to quantify and compare major pools and fluxes of nitrogen in recently established (≤ 80 y) redcedar forests and adjacent native grasslands. Three former grassland sites in the Flint Hills region of Kansas that developed closed-canopy redcedar forests in the recent past were paired with adjacent grassland sites on similar soil type and topographic position (n = 3 sites/land cover type), and selected soil and plant nitrogen pools and fluxes were measured in replicate plots (n = 6/site) along transects in each forest or grassland site over a 20-mo period. We found ...

Introduction: Epicardial fat produces multiple proinflammatory and proatherogenic cytokines and is associated with adverse cardiovascular events. Inflammation and resultant endothelial dysfunction may play a role in progressive myocardial dysfunction among adults with single ventricle physiology status post Fontan, but the potential impact of epicardial fat volume (EFV) has not been studied. This study aimed to quantify EFV in Fontan patients compared to a control group of repaired tetralogy of Fallot patients (rTOF) and to evaluate its association with ventricular ejection fraction and cardiac index. Methods: We retrospectively measured EFV on all patients post Fontan, 15 years or older, with cardiac magnetic resonance performed at the University of Michigan from 2007-2014, and 1:1 age, sex, and body mass index (BMI) matched patients with rTOF. EFV was measured manually on cine, steady-state free precession, short-axis images and was indexed to body surface area (BSA). Cardiac index was calculated as the sum of flow in the superior vena cava and inferior vena cava, indexed to BSA. A random subset of studies was re-measured >1 week after initial measurement to assess intraobserver variability. Results: Fontan patients (n=63, mean age 22.3±6.0, 52% male) had a larger indexed EFV compared to rTOF (mean age 22.2±5.8, 52% male) measuring 75.6±29.2 vs. 60.0±19.9 ml/m 2 (p= 0.0003). There was no difference in BMI between the two groups (24.2±5.2 vs 24.2±5.6, p= 0.81). In Fontan patients, indexed EFV was inversely correlated with ventricular ejection fraction (r = -0.26, p = 0.03) and cardiac index (r = -0.34, p = 0.01). Intraobserver variability of the indexed EFV measurements in both groups was excellent (intraclass correlation coefficient of 0.95 and 0.93 in the Fontan group and rTOF group, respectively). Conclusions: Indexed EFV is higher in individuals post Fontan compared to patients with rTOF and is associated with lower ventricular ejection fraction and cardiac index. Increased epicardial fat could play a role in progressive myocardial dysfunction in this population, but longitudinal studies are necessary to establish any causative role.

To evaluate transition readiness, including perceived knowledge deficits, self-efficacy, and self-management behaviors, in 13- to 25-year-olds with congenital heart disease or heart transplant and to examine the relationships between transition readiness assessment, information seeking behavior, and quality of life (QOL).In this cross-sectional study, patients (n = 164) completed the Transition Readiness Assessment and the Pediatric Quality of Life Inventory using an e-tablet, web-based format at a routine clinic visit.Median patient age was 18.1 years (range 13.0-25.5). Average perceived knowledge deficit score (% of items with no knowledge) was 25.7% (range 0%-75%). On a 100-point scale, the mean score was 72.0 ± 17.2 for self-efficacy and 49.7 ± 17.5 for self-management. Knowledge deficits were negatively correlated with self-efficacy (r = -0.45, P.0001) and self-management (r = -0.36, P.0001). Overall, 66% of patients requested information (73% ≥ 18 years old). Higher psychosocial QOL scores were correlated with lower knowledge deficit scores (r = -0.21, P = .01) and higher perceived self-efficacy scores (r = 0.41, P.0001).Transition knowledge deficits are common and associated with decreased self-efficacy and self-management skills in adolescents and young adults with heart disease. Greater transition knowledge and perceived self-efficacy are associated with better psychosocial QOL. Routine assessment of transition readiness is recommended to facilitate recognition of deficits and interventions to promote successful transition outcomes.

Background: A Kommerell Diverticulum (KD) is the dilated proximal portion of an aberrant subclavian artery as a consequence of abnormal embryonic vascular development. Concern has arisen for aneurysmal KD dilation, dissection and rupture, with a trend toward invasive prophylactic intervention. This

Objective: Transition is defined as “the process by which adolescents and young adults with chronic childhood illnesses are prepared to take charge of their lives and their health in adulthood”. We previously reported common knowledge deficits and lack of transition readiness (TR) in 13-25 year olds with congenital or acquired heart disease. The aims of this study were to re-evaluate TR in these patients at follow-up (F/U) and to examine the relationship between changes in TR and quality of life (QOL). Methods: Patients (n=106) completed the TR Assessment and Pediatric Quality of Life Inventory (PedsQL) utilizing an e-tablet, web-based format at a routine F/U clinic visit. Changes from initial to F/U scores were evaluated. Results: Median patient age was 18.7 yrs at a median F/U time of 1.02 yrs. Average perceived knowledge deficit score (% of items with no knowledge) at F/U was 18.0 ± 15.2%, decreased from 24.7 ± 16.5%, p Conclusion: While deficits in knowledge and self-management skills persist, TR assessment and recognition of deficits can improve transition readiness with improved psychosocial QOL. Routine TR assessment is important to identify transition needs. Further studies are needed to examine the relationship between TR and outcomes in young adults with heart disease.

An increase in woody plant abundance in regions historically dominated by grasses is a recent land cover change in grasslands worldwide. In tallgrass prairies of North America, this increase in woody plant cover includes the development of dense stands of eastern redcedar (Juniperus virginiana L.). To evaluate the consequences of this ongo- ing land cover change for ecosystem functioning, we developed allometric equations, using data from Kansas and Oklahoma, to estimate aboveground biomass and productivity in closed-canopy redcedar stands. We then applied these equations to three closed-canopy redcedar stands, 35-80 years old, which developed on sites formerly dominated by tallgrass prairie in eastern Kansas. Aboveground plant biomass for these redcedar-dominated sites ranged from 114 100 kg/ha for the youngest stand to 210 700 kg/ha for the oldest. Annual aboveground net primary productivity (ANPP) ranged from 7250 to 10 440 kg·ha -1 ·year -1 for the oldest and younger redcedar stands, respectively. Estimates of ANPP in comparable tallgrass prairie sites in this region average 3690 kg·ha -1 ·year -1 indicating a large increase in C uptake and aboveground storage as a result of the change from prairie to redcedar forests. Therefore, the widespread occurrence of redcedars across the woodland-prairie ecotone suggests that this land-cover change may have important consequences for regional net C storage. Resume : L'augmentation de l'abondance des plantes ligneuses dans des regions historiquement dominees par les gra- minees est un changement recent de type de couvert des prairies a l'echelle mondiale. Dans les prairies a herbes hautes de l'Amerique du Nord, cette augmentation du couvert des plantes ligneuses inclut le developpement de peuplements denses de genevrier de Virginie (Juniperus virginiana L.). De facon a evaluer les consequences de ce changement pro- gressif de type de couvert pour le fonctionnement de l'ecosysteme, nous avons developpe des equations allometriques a partir de donnees provenant du Kansas et de l'Oklahoma pour estimer la biomasse epigee et la productivite de ces peu- plements a couvert ferme de genevrier. Nous avons ensuite applique ces equations a trois peuplements fermes de genevrier, âges entre 35 et 80 ans et qui se sont developpes dans des sites anciennement domines par la prairie a her- bes hautes dans l'est du Kansas. La biomasse aerienne de ces sites domines par le genevrier variait de 114 100 kg/ha pour le peuplement le plus jeune a 210 700 kg/ha pour le plus vieux. La productivite primaire nette annuelle de la biomasse aerienne (PPNA) oscillait entre 7250 et 10 440 kg·ha -1 ·an -1 respectivement pour le plus vieux et le plus jeune peuplement. Les estimes de la PPNA dans des sites de prairie a herbes comparables atteignent en moyenne 3690 kg·ha -1 ·an -1 , ce qui in- dique une forte augmentation de la fixation du carbone et de son stockage aerien par la conversion des prairies en forets de genevrier. Par consequent, la presence abondante de genevriers dans l'ecotone prairie-foret semble indiquer que ce changement de type de couvert peut avoir des consequences importantes pour le stockage regional net de carbone. (Traduit par la Redaction) Norris et al. 1946

Valve repair for pediatric patients with Ebstein anomaly has historically yielded varied results. The cone reconstruction (CR) first described by Da Silva has revolutionized the surgical approach to these patients. This study reports our recent experience with CR in children and young adults with Ebstein anomaly.Electronic medical records were reviewed for all patients21 years old who had surgery to repair Ebstein anomaly at Mayo Clinic Rochester between June 2007 and December 2012. Clinical data including preoperative demographics, intraoperative procedures, and postoperative outcomes were recorded.Eighty-four patients initially had a cone reconstruction (54% male, mean age 10.1 ± 5.9 years). Indications for operation included cardiomegaly (42%), cyanosis (19%), and heart failure (19%). The preoperative echocardiogram demonstrated severe tricuspid regurgitation in 91% of patients. There was one early death and 3 early CR breakdowns requiring reoperation (2 re-repair, 1 tricuspid replacement). Eighty-two patients (98%) had successful CR at the time of hospital discharge. Patient age, gender, cardiopulmonary bypass time, and aortic cross-clamp time were not associated with early CR failure. Use of a partial or eccentric annuloplasty ring correlated with successful initial CR (P = .01). There have been no early CR breakdowns since 2010. Follow-up information was available for 77 patients (longest follow-up 6.5 years; mean 0.8 ± 0.2 years). The most recent postoperative echocardiogram demonstrated mild or no tricuspid regurgitation in 83%. Tricuspid stenosis (mean gradient5 mm Hg) was present in 6 patients. There was one late death (motor vehicle accident) and one late re-repair of the tricuspid valve 4 years after initial operation.CR in children and young adults with Ebstein anomaly can be performed with low early mortality and excellent durability at short-term follow-up. CR represents an important surgical option for young patients. It is applicable to patients with a broad range of anatomic variability and precludes valve replacement in the vast majority. CR should be considered prior to the deleterious effects of chronic right ventricular volume overload and the development of systolic dysfunction, which hamper long-term prognosis. Therefore, early referral for surgical evaluation is recommended.

Although sleep-disordered breathing has been extensively studied in patients with left-ventricular dysfunction, little is known of its prevalence in adults with congenital heart disease. Patients with d-looped transposition of the great arteries (d-TGA) who have undergone atrial switch procedures often develop progressive heart failure. The objective of this study was to determine the prevalence of patients at risk for sleep-disordered breathing in adults with d-TGA and atrial switch procedures compared with a control population. Thirty-two patients with d-TGA (66 % males, median age 31) were compared with 32 healthy controls. Baseline demographics and clinical characteristics were documented. The snoring, tiredness during daytime, observed apnea, and high blood pressure (STOP) questionnaire was used to identify subjects at risk for obstructive sleep apnea (OSA). There was no difference in baseline demographics between subjects and controls. For the STOP questionnaire, 14 subjects with d-TGA had scores predictive of OSA compared with three in the control group (44 vs. 9 %, p = 0.0038). There was no difference in functional status between d-TGA patients with or without OSA. There is a greater prevalence of risk for sleep disordered breathing in adults with d-TGA compared with controls. Further prospective investigation with sleep studies will be valuable to confirm these findings.

[Figure][1] [![Graphic][3] ][3] A 13-year-old young man with anxiety presented for echocardiogram after multiple emergency room visitations for atypical chest pain. An echocardiogram (A to E) displayed a fluid filled, tube-like structure posterior to the left atrium (LA) and parallel

Previous studies have noted the importance of vegetation in controlling run-off in terrestrial landscapes. Here, we hypothesize that ecologic succession from mowed grassland to old field to forest will impact run-off characteristics due to changing vegetation characteristics that affect soil surface processes. We test this idea by measuring depression storage and leaf litter water retention rates in 13 sites across this successional gradient. Sites of similar slope and soil type were also evaluated against one another to isolate the effect of vegetation diversity and management on depression storage. Results showed that average ideal depression storage increased with greater vegetation diversity and structure. Effective depression storage of unmanaged systems was significantly higher than mowed systems, implying that management activities retard the natural development of microtopography. Results of leaf litter water retention varied among land covers but contributed less than depression storage for water capture. These results demonstrate the tight linkages between vegetation succession and run-off. Understanding these characteristics is necessary for developing effective hydrologic models. Copyright © 2012 John Wiley & Sons, Ltd.

In the Great Plains region, eastern redcedar (Juniperus virginiana L.) abundance has increased dramatically in areas historically occupied by tallgrass prairie. This shift in dominant vegetation is likely to be accompanied by changes in ecosystem structure and function, including quantity, quality, and location (above- vs. below-ground) of litter inputs and subsequent effects on decomposition dynamics. The purpose of this study was to quantify and compare patterns of litterfall and decomposition in mature redcedar forests and adjacent prairie. Annual redcedar litterfall was 500 g·m-2·year-1, a large increase in foliar litter inputs compared with annually burned grassland (52 g·m-2·year-1). Using a reciprocal transplant decomposition study with four substrates, redcedar leaves and roots, and big bluestem (Andropogon gerardii Vitman) foliage and roots, we found marginal habitat effects, but greater differences between species, presumably owing to litter quality (including both C:N and lignin content). Decay rates were significantly higher in the prairie relative to the forest for each substrate. Additionally, within any particular habitat, grass litter had significantly faster decay rates than forest litter. These results suggest a shift in patterns of litter input and decomposition processes with afforestation of tallgrass prairie, which may result in long-term changes in C sequestration and storage.Key words: litter dynamics, eastern redcedar (Juniperus virginiana), big bluestem (Andropogon gerardii), decomposition, tallgrass prairie, nitrogen immobilization.

The population with adult congenital heart disease is expanding. Cardiac care retention and follow-up patterns were assessed in 153 adults with congenital heart disease (median age, 24.5 years), previously compliant as teenagers. The majority (125; 81.7%) were retained in care, most often by a pediatric cardiologist (69%). The rate of retention was surprisingly high.