Orthotopic Heart and Combined Heart Liver Transplantation: the Ultimate Treatment Option for Failing Fontan Physiology.

Purpose of the Review: This is a comprehensive update on failing Fontan physiology and the role of heart and combined heart and liver transplantation in the current era. Recent Findings: Single ventricle physiology encompasses a series of rare congenital cardiac abnormalities that are characterized by absence of or hypoplasia of one ventricle. This effectively results in a single ventricular pumping chamber. These abnormalities are rarely compatible with long-term survival if left without surgical palliation in the first few years of life. Surgical treatment of single ventricle physiology has evolved over the past 60 years and is characterized by numerous creative innovations. These include the development of arteriopulmonary shunts, the evolution of partial cavopulmonary connections, and the eventual development of the "Fontan" operation. Regardless of the type of Fontan modification, the long-term consequences of the Fontan operation are predominantly related to chronic central venous hypertension and the multi-organ consequences thereof. Atrial arrhythmias can further compromise this circulation.Patients with single ventricle physiology represent a special sub-segment of congenital cardiac transplants and are arguably the most challenging patients considered for transplantation. Summary: This review describes in detail the challenges and opportunities of heart and liver transplantation in Fontan patients, as viewed and managed by the experienced team at the Ahmanson/UCLA Adult Congenital Heart Center.

School-supervised use of a once-daily inhaled corticosteroid regimen: A cluster randomized trial.

BACKGROUND: School-supervised use of a once-daily inhaled corticosteroid regimen (supervised therapy) can improve medication adherence and asthma control. OBJECTIVE: We sought to evaluate the effectiveness of supervised therapy in a unique setting and population. METHODS: We conducted a cluster randomized trial of supervised therapy in 20 elementary schools with a disproportionate enrollment of low-income Latino students. Schools were purposively selected, matched, and randomized to receive 9 months of supervised therapy with mometasone furoate or usual care. All English- or Spanish-speaking students with self-reported asthma were eligible. The Asthma Control Questionnaire (ACQ) was interviewer administered quarterly at school. Students in supervised therapy schools were hypothesized to have lower ACQ scores than students in usual-care schools. RESULTS: Of 393 enrolled students, 189 students receiving immediate intervention and 143 students receiving delayed intervention provided 1 or more ACQ data points, were between 6 and 10 years of age, and were included in the primary analysis. At baseline, 39% of students reported taking a controller medication, and 24% had well-controlled asthma. Eighty percent of students receiving immediate intervention were prescribed mometasone. Schools administered 98% of prescribed doses when students attended school. Absences, weekends, and holidays reduced calendar adherence to 53%. During the first year, the mean ACQ score for students receiving immediate and delayed intervention was 1.55 (95% CI, 1.41-1.70) and 1.64 (95% CI, 1.47-1.80), respectively. The estimated treatment effect was -0.08 (95% CI, -0.31 to 0.14). DISCUSSION: Compared with usual care, supervised therapy did not improve asthma control among this population of Latino students. Additional research is warranted to confirm these results.

Global Health Education in US Pediatric Residency Programs.

BACKGROUND AND OBJECTIVE: Despite the growing importance of global health (GH) training for pediatric residents, few mechanisms have cataloged GH educational opportunities offered by US pediatric residency programs. We sought to characterize GH education opportunities across pediatric residency programs and identify program characteristics associated with key GH education elements. METHODS: Data on program and GH training characteristics were sought from program directors or their delegates of all US pediatric residency programs during 2013 to 2014. These data were used to compare programs with and without a GH track as well as across small, medium, and large programs. Program characteristics associated with the presence of key educational elements were identified by using bivariate logistic regression. RESULTS: Data were collected from 198 of 199 active US pediatric residency programs (99.5%). Seven percent of pediatric trainees went abroad during 2013 to 2014. Forty-nine programs (24.7%) reported having a GH track, 66.1% had a faculty lead, 58.1% offered international field experiences, and 48.5% offered domestic field experiences. Forty-two percent of programs reported international partnerships across 153 countries. Larger programs, those with lead faculty, GH tracks, or partnerships had significantly increased odds of having each GH educational element, including pretravel preparation. CONCLUSIONS: The number of pediatric residency programs offering GH training opportunities continues to rise. However, smaller programs and those without tracks, lead faculty, or formal partnerships lag behind with organized GH curricula. As GH becomes an integral component of pediatric training, a heightened commitment is needed to ensure consistency of training experiences that encompass best practices in all programs.

Qualitative variation in proanthocyanidin composition of Populus species and hybrids: genetics is the key.

The literature on proanthocyanidins (tannins) in ecological systems is dominated by quantitative studies. Despite evidence that the qualitative characteristics (subunit type, polymer chain length) of these complex polyphenolics are important determinants of biological activity, little is known about genetic and environmental controls on the type of proanthocyanidins produced by plants. We tested the hypothesis that genetics, season, developmental stage, and environment determine proanthocyanidin qualitative characteristics by using four Populus "cross types" (narrowleaf [P. angustifolia], Fremont [P. fremontii], F1 hybrids, and backcrosses to narrowleaf). We used thiolysis and HPLC analysis to characterize the proanthocyanidins, and found that genetics strongly control composition. The narrowleaf plants accumulate mixed procyanidin/prodelphinidins with average composition epicatechin(11)-epigallocatechin(8)-catechin(2)-catechin((terminal)). Backcross genotypes produce mixed procyanidin/prodelphinidins similar to narrowleaf, while Fremont makes procyanidin dimers, and the F1 plants contain procyanidin heptamers. Less striking effects were noted for genotype × environment, while season and developmental zone had little effect on proanthocyanidin composition or chain length. We discuss the metabolic and ecological consequences of differences in condensed tannin qualitative traits.