Hepatic and Renal Consequences of Single-Ventricle Physiology Palliated With the Fontan Operation.

Over time, long-term survival has dramatically increased for patients with complex congenital heart disease who undergo the Fontan operation. With this increased survival, it has become apparent that such a circulation has important consequences for other organ systems, particularly the liver and kidney. The adverse milieu created by chronic venous hypertension, low cardiac output, and an inflammatory state contribute to the pathologic changes observed in the liver and kidneys over the long term in Fontan patients.

A text messaging intervention and quality of life in adolescents with solid organ transplants.

Background: Facilitating communication between adolescents and HCP outside of appointments may enhance patient experience and outcomes. The purpose of this study was to determine whether SMS enhances the healthcare experience, QoL, and medication adherence in adolescent SOT patients.

Methods: This was a prospective observational study of an SMS platform (WelTel Inc) for SOT patients aged 12-19 years.

Association of Preoperative Diuretic Use With Early Acute Kidney Injury in Infants With Biventricular Hearts Following Cardiac Surgery.

Background Diuretics are used to manage congestive heart failure in infants with congenital heart disease. Adult data indicate that preoperative diuretic use increases the risk of cardiac surgery associated acute kidney injury (CS-AKI). We have sought to understand if preoperative diuretics in infants increases the risk of CS-AKI.

Adolescents with solid organ transplant: Using the BRIEF2 parent-report and self-report to measure parent-child agreement and everyday executive function.

Agreement between parent and adolescent ratings of executive function (EF) is not known in adolescents with solid organ transplant (SOT), even though pressing concerns about EF deficits are being raised in this population. The current study investigated EF in adolescents with SOT using parent and self report. Twenty-five adolescents ( = 15.

Fontan-Associated Liver Disease: Spectrum of Disease in Children and Adolescents.

Background Information is evolving on liver disease in pediatric patients with Fontan physiology. The purpose of this investigation is to evaluate the spectrum of liver disease in a pediatric population of patients with Fontan physiology and evaluate transient elastography (TE) as a noninvasive marker of liver disease. Methods and Results We prospectively enrolled all children with Fontan physiology.

Fontan-Associated Liver Disease: Evidence for Early Surveillance of Liver Health in Pediatric Fontan Patients.

The growing awareness of Fontan-associated liver disease (FALD) in adults with Fontan physiology has provided the impetus to better understand the natural history of FALD and develop a reliable noninvasive method to diagnose and monitor liver health in this population. Biochemical and imaging tests have been investigated to determine their association with liver pathology. The congestive hepatopathy that develops after the Fontan procedure has made interpreting these tests challenging.

Hybrid stenting for left ventricular outflow tract obstruction in congenitally corrected transposition of the great arteries.

Congenitally corrected transposition of the great arteries is commonly associated with left ventricular outflow tract obstruction. We describe a case of congenitally corrected transposition of the great arteries and previous surgical ventricular septal defect repair with recurrent left ventricular outflow tract obstruction. The patient underwent a hybrid procedure to stent the left ventricular outflow tract, which was successful with no re-intervention through 3 years of follow-up.

Outcomes of Radiofrequency Perforation for Pulmonary Atresia and Intact Ventricular Septum: A Single-Centre Experience.

Percutaneous radiofrequency perforation (RFP) of the pulmonary valve is used as a primary therapy in neonates with pulmonary atresia and intact ventricular septum (PAIVS). We sought to determine the safety and efficacy of RFP for PAIVS in a single center and assess the pre-intervention anatomical parameters associated with a biventricular outcome. We retrospectively reviewed all cases of PAIVS treated with RFP at a single center from 1999 through 2012.

Effect of different single-session educational modalities on improving medical students' ability to demonstrate proper pressurized metered dose inhaler technique.

Objective: Pressurized metered dose inhalers (pMDIs) remain important therapeutic options for obstructive lung diseases. The ability to instruct and evaluate inhaler technique is a crucial skill that all medical professionals should possess; unfortunately, many professionals lack proficiency with pMDIs. We aimed to determine if brief education interventions of differing modalities can positively affect medical students' skills over the long term.

Internal return of carbon dioxide in decarboxylation: catalysis of separation and 12C/13C kinetic isotope effects.

It has been proposed that the decarboxylation of mandelylthiamin, the adduct of benzoylformate and thiamin, is uniquely catalyzed by protonated pyridines through a preassociation mechanism in which proton transfer competes with the internal return of carbon dioxide. Application of this mechanism suggests that the observed primary (12)C/(13)C kinetic isotope effect in the absence of catalyst is reduced in magnitude because diffusion of carbon dioxide is partially rate-determining. Where proton transfer blocks the internal return of carbon dioxide, the separation of carbon dioxide is facilitated, and the observed isotope effect increases toward the intrinsic value for carbon-carbon bond breaking.

Catalyzing separation of carbon dioxide in thiamin diphosphate-promoted decarboxylation.

Thiamin diphosphate-dependent decarboxylases form addition intermediates between thiamin diphosphate (ThDP) and 2-ketoacids. Although it appears that the intermediate should react without the intervention of catalysts, evidence has clearly shown that Brønsted acid catalysis occurs through a pre-associated system. This can promote separation of carbon dioxide from the residual carbanion by protonation of the carbanion.