Assessment of Atmospheric and Surface Energy Budgets Using Observation-Based Data Products.

Accurate diagnosis of regional atmospheric and surface energy budgets is critical for understanding the spatial distribution of heat uptake associated with the Earth's energy imbalance (EEI). This contribution discusses frameworks and methods for consistent evaluation of key quantities of those budgets using observationally constrained data sets. It thereby touches upon assumptions made in data products which have implications for these evaluations.

The Fontan immunophenotype and post-transplant outcomes in children: A multi-institutional study.

Background: Patients after Fontan palliation represent a growing pediatric population requiring heart transplant (HTx) and often have lymphopenia (L) and/or hypogammaglobinemia that may be exacerbated by protein-losing enteropathy (PLE, P). The post-HTx effects of this altered immune phenotype are not well studied.

Methods: In this study of the Pediatric Heart Transplant Society Registry, 106 Fontan patients who underwent HTx between 2005 and 2018 were analyzed.

Emerging Trends in Arctic Solar Absorption.

Recent satellite observations confirm that the Arctic is absorbing more solar radiation now than at the start of this century in response to declining Arctic sea ice and snow covers. Trends in the solar radiation input to Arctic ocean and land surfaces now each exceed interannual variability at the 95% confidence level, although all-sky trends have taken 20%-40% longer to emerge compared to clear-sky conditions. Clouds reduce mean solar absorption and secular trends over both land and ocean, but the effect of clouds on natural variability depends on the underlying surface.

Orthotopic heart transplant in a pediatric patient with mixed connective tissue disease.

Background: Patients with autoimmune inflammatory syndromes such as mixed connective tissue disease (MCTD) and systemic lupus erythematosus have previously been considered marginal candidates for orthotopic heart transplant (OHT).

Methods: A retrospective chart review was completed for this case report.

Results: We present the case of an 11-year-old girl with known MCTD who developed congestive heart failure refractory to medical therapy and underwent OHT.

Arctic Clouds and Precipitation in the Community Earth System Model Version 2.

The Arctic climate is changing rapidly, warming at about twice the rate of the planet. Global climate models (GCMs) are invaluable tools both for understanding the drivers of these changes and predicting future Arctic climate evolution. While GCMs are continually improving, there remain difficulties in representing cloud processes which occur on scales smaller than GCM resolution.

Reducing the aerosol forcing uncertainty using observational constraints on warm rain processes.

Global climate models (GCMs) disagree with other lines of evidence on the rapid adjustments of cloud cover and liquid water path to anthropogenic aerosols. Attempts to use observations to constrain the parameterizations of cloud processes in GCMs have failed to reduce the disagreement. We propose using observations sensitive to the relevant cloud processes rather than only to the atmospheric state and focusing on process realism in the absence of aerosol perturbations in addition to the process susceptibility to aerosols.

Pediatric heart transplant from an incompletely treated influenza A-positive donor.

There is a shortage of pediatric donor hearts for waitlisted children, and yet nearly 50% of organs offered are not transplanted. Donor quality is often cited as a reason for declining organs offered from donors infected with influenza, presumably due to concern about disease transmission at transplant leading to severe disease. We previously described an excellent outcome after heart transplant from a donor infected with influenza B that had been treated with a complete course of oseltamivir.

Trajectory of right ventricular indices is an early predictor of outcomes in hypoplastic left heart syndrome.

Background: Children with hypoplastic left heart syndrome (HLHS) have risk for mortality and/or transplantation. Previous studies have associated right ventricular (RV) indices in a single echocardiogram with survival, but none have related serial measurements to outcomes. This study sought to determine whether the trajectory of RV indices in the first year of life was associated with transplant-free survival to stage 3 palliation (S3P).

Pediatric heart transplantation from an influenza B-positive donor.

As heart transplantation demand is increasing without subsequent growth of the donor pool, need for expansion of acceptance criteria is paramount, particularly when considering critically ill, highly sensitized patients. We present a case report of a pediatric heart transplant recipient of an organ refused by 197 prior potential recipients due to the donor being infected with influenza virus. We perform a literature review of recent solid organ transplant cases from influenza-positive donors and conclude that the donor pool may be expandable by allowing donors with treatable infections to be included.

Cloud Occurrences and Cloud Radiative Effects (CREs) from CCCM and CloudSat Radar-Lidar Products.

Two kinds of radar-lidar synergy cloud products are compared and analyzed in this study; CERES-CALIPSO-CloudSat-MODIS (CCCM) product and CloudSat radar-lidar (RL) product such as GEOPROF-LIDAR and FLXHR-LIDAR. Compared to GEOPROF-LIDAR, CCCM has more low-level (< 1 km) clouds over tropical oceans because CCCM uses a more relaxed threshold of Cloud-Aerosol Discrimination (CAD) score for Cloud-Aerosol Lidar and Infrared Pathfinder Satellite Observations (CALIPSO) vertical feature mask (VFM) product. In contrast, GEOPROF-LIDAR has more mid-level (1-8 km) clouds than CCCM at high latitudes (> 40°).

Saharan dust, convective lofting, aerosol enhancement zones, and potential impacts on ice nucleation in the tropical upper troposphere.

Dry aerosol size distributions and scattering coefficients were measured on 10 flights in 32 clear-air regions adjacent to tropical storm anvils over the eastern Atlantic Ocean. Aerosol properties in these regions were compared with those from background air in the upper troposphere at least 40 km from clouds. Median values for aerosol scattering coefficient and particle number concentration >0.

Bacterial infections after pediatric heart transplantation: Epidemiology, risk factors and outcomes.

Background: Bacterial infections represent a major cause of morbidity and mortality in heart transplant recipients. However, data describing the epidemiology and outcomes of these infections in children are limited.

Methods: We analyzed the Pediatric Heart Transplant Study database of patients transplanted between 1993 and 2014 to determine the etiologies, risk factors and outcomes of children with bacterial infections post-heart transplantation.

An Observational View of Relationships Between Moisture Aggregation, Cloud, and Radiative Heating Profiles.

Data from several coincident satellite sensors are analyzed to determine the dependence of cloud and precipitation characteristics of tropical regions on the variance in the water vapor field. Increased vapor variance is associated with decreased high cloud fraction and an enhancement of low-level radiative cooling in dry regions of the domain. The result is found across a range of sea surface temperatures and rain rates.

Observing Convective Aggregation.

Convective self-aggregation, the spontaneous organization of initially scattered convection into isolated convective clusters despite spatially homogeneous boundary conditions and forcing, was first recognized and studied in idealized numerical simulations. While there is a rich history of observational work on convective clustering and organization, there have been only a few studies that have analyzed observations to look specifically for processes related to self-aggregation in models. Here we review observational work in both of these categories and motivate the need for more of this work.

Responses of Tropical Ocean Clouds and Precipitation to the Large-Scale Circulation:Atmospheric-Water-Budget-Related Phase Space and Dynamical Regimes.

An atmospheric-water-budget-related phase space is constructed with the tendency terms related to dynamical convergence (QCON ≡ -∇ · ) and moisture advection (QADV ≡ - · ∇) in the water budget equation. Over the tropical oceans, QCON accounts for large-scale dynamical conditions related to conditional instability, and QADV accounts for conditions related to lower-tropospheric moisture gradient. Two reanalysis products [MERRA and ERA-Interim (ERAi)] are used to calculate QCON and QADV.

Gain-of-function mutations in SMAD4 cause a distinctive repertoire of cardiovascular phenotypes in patients with Myhre syndrome.

Myhre syndrome is a rare, distinctive syndrome due to specific gain-of-function mutations in SMAD4. The characteristic phenotype includes short stature, dysmorphic facial features, hearing loss, laryngotracheal anomalies, arthropathy, radiographic defects, intellectual disability, and a more recently appreciated spectrum of cardiovascular defects with a striking fibroproliferative response to surgical intervention. We report four newly described patients with typical features of Myhre syndrome who had (i) a mildly narrow descending aorta and restrictive cardiomyopathy; (ii) recurrent pericardial and pleural effusions; (iii) a large persistent ductus arteriosus with juxtaductal aortic coarctation; and (iv) restrictive pericardial disease requiring pericardiectomy.

Dilated cardiomyopathy with cardiogenic shock in a child with Kearns-Sayre syndrome.

Kearns-Sayre syndrome (KSS) is a mitochondrial myopathy resulting from mitochondrial DNA deletion. This syndrome primarily involves the central nervous system, eyes, skeletal muscles and the heart. The most well-known cardiac complications involve the conduction system; however, there have been case reports describing cardiomyopathy.

Clouds enhance Greenland ice sheet meltwater runoff.

The Greenland ice sheet has become one of the main contributors to global sea level rise, predominantly through increased meltwater runoff. The main drivers of Greenland ice sheet runoff, however, remain poorly understood. Here we show that clouds enhance meltwater runoff by about one-third relative to clear skies, using a unique combination of active satellite observations, climate model data and snow model simulations.

Diastolic function in anthracycline-treated children.

Background: Anthracyclines are effective medications for childhood cancer. Their limitation is the risk of cardiomyopathy. Although diastolic dysfunction has been described in patients who received anthracyclines, cardiac monitoring has focused on systolic function, which is abnormal in up to 41% of the patients.

Bortezomib use in a pediatric cardiac transplant center.

Data are limited on the efficacy and safety of bortezomib for the treatment of AMR following OHT for pediatric acquired or CHD. Retrospective chart review identified patients who received bortezomib for acute (n = 3, within two wk of diagnosis) and chronic (n = 1, three months after diagnosis) AMR or as part of a desensitization regimen (n = 1). Bortezomib was associated with a 3-66% reduction in class I DSA and a 7-82% reduction in class II DSA.

Predictors of cardiac allograft vasculopathy in pediatric heart transplant recipients.

CAV remains a leading cause of late graft loss and mortality among survivors of pediatric heart transplantation. We sought to define the incidence of CAV and identify its predictors in pediatric heart transplant recipients. The OPTN/UNOS database was analyzed for pediatric recipients who underwent heart transplant between 1987 and 2011.

Outcome of heart transplantation in pediatric cancer survivors.

The aim of this study is to evaluate the outcome of heart transplantation in children surviving malignancies. Pediatric heart transplant recipients were identified using the UNOS database. Follow-up data including survival and rate of malignancy were analyzed.

Impact of pulmonary hypertension on transplant outcomes in pediatric cardiomyopathy patients.

Controversy exists over whether PHTN in heart transplant candidates increases post-transplant mortality. We performed analysis of data reported to UNOS for children who underwent primary heart transplantation for cardiomyopathy from January 1994 to June 2010. Patients were divided into two groups depending on their pre-transplant TPG: no-PHTN (TPG ≤ 12 mmHg) and PHTN (TPG >12 mm Hg).

Orthotopic heart transplant: a therapeutic option for unresectable cardiac fibroma in infants.

Primary cardiac tumors are rare lesions in childhood, with the two most common being rhabdomyoma and fibroma. We report two infants who successfully underwent orthotopic heart transplant for massive interventricular septal cardiac fibromas. For unresectable infantile cardiac fibroma, orthotopic heart transplant may be considered a therapeutic option.

Impact of donor cardiopulmonary resuscitation on pediatric heart transplant outcome.

Mortality is the highest of any solid organ in pediatric patients awaiting heart transplantation. Strategies to increase the donor pool are needed if survival to transplant is to improve. There can be reluctance to accept pediatric hearts for transplantation if the donor has received cardiopulmonary resuscitation (CPR).