Educational DVD for parents of children with congenital heart disease - a pilot study.

Parental knowledge regarding their child's congenital heart defect (CHD) is limited. This study developed and evaluated an interactive DVD that helped parents to understand their child's CHD. A DVD describing four cardiac abnormalities was created using videos and Adobe Flash. The parents' knowledge was assessed via a questionnaire before and after receiving the DVD. A control group did not receive the DVD. The DVDs were successfully created and improved the parental knowledge of their child's cardiac anomaly. The pilot study suggests the need for further development of DVDs to describe additional abnormalities, whilst incorporating local management and outcomes.

ß-adrenergic signaling inhibits Gq-dependent protein kinase D activation by preventing protein kinase D translocation.

RATIONALE: Both ß-adrenergic receptor (ß-AR) and Gq-coupled receptor (GqR) agonist-driven signaling play key roles in the events, leading up to and during cardiac dysfunction. How these stimuli interact at the level of protein kinase D (PKD), a nodal point in cardiac hypertrophic signaling, remains unclear. OBJECTIVE: To assess the spatiotemporal dynamics of PKD activation in response to ß-AR signaling alone and on coactivation with GqR-agonists. This will test our hypothesis that compartmentalized PKD signaling reconciles disparate findings of PKA facilitation and inhibition of PKD activation. METHODS AND RESULTS: We report on the spatial and temporal profiles of PKD activation using green fluorescent protein-tagged PKD (wildtype or mutant S427E) and targeted fluorescence resonance energy transfer-based biosensors (D-kinase activity reporters) in adult cardiomyocytes. We find that ß-AR/PKA signaling drives local nuclear activation of PKD, without preceding sarcolemmal translocation. We also discover pronounced interference of ß-AR/cAMP/PKA signaling on GqR-induced translocation and activation of PKD throughout the cardiomyocyte. We attribute these effects to direct, PKA-dependent phosphorylation of PKD-S427. We also show that phosphomimetic substitution of S427 likewise impedes GqR-induced PKD translocation and activation. In neonatal myocytes, S427E inhibits GqR-evoked cell growth and expression of hypertrophic markers. Finally, we show altered S427 phosphorylation in transverse aortic constriction-induced hypertrophy. CONCLUSIONS: ß-AR signaling triggers local nuclear signaling and inhibits GqR-mediated PKD activation by preventing its intracellular translocation. PKA-dependent phosphorylation of PKD-S427 fine-tunes the PKD responsiveness to GqR-agonists, serving as a key integration point for ß-adrenergic and Gq-coupled stimuli.

The development of a CD-ROM: an aid to fetal cardiac diagnosis and counseling.

OBJECTIVE: Obstetric ultrasound allows for the fetal diagnosis of complex congenital heart disease, which once diagnosed is especially stressful for the affected parents. This study aimed to develop and pilot an educational CD-ROM for parents who had a fetal diagnosis of a congenital heart defect (CHD). MATERIAL AND METHODS: A CD-ROM was developed which included a brief description of a normal heart in text and cartoons, followed by one of five congenital heart abnormalities (again described in text and illustrated by cartoons highlighting the anatomy), the likely interventions (surgical or by catheter), and the local outcomes. A pilot study was conducted whereby parents were provided with the CD-ROM and asked to complete a brief evaluation following the fetal diagnosis of a suspected CHD and subsequent to counseling by the pediatric cardiologist, but prior to the birth of the infant. The CD-ROM was to be viewed at the parents' convenience. The questionnaire was returned via postal mail. RESULTS: A CD-ROM covering five common congenital heart abnormalities was successfully developed and distributed to 20 parents. Fourteen responses were received. The feedback was favorable. The parents found the CD-ROM to be informative and easily understood and supplemented the information previously provided by the pediatric cardiologist. The diagrams were particularly helpful. CONCLUSION: An educational CD-ROM describing a cardiac abnormality of a fetus was successfully created and favorably received, complementing the earlier counseling given. Further development of the CD-ROM with modifications highlighting parental suggestions and to include other abnormalities will allow for a wider audience.

The incidence of venous thromboembolism in children following colorectal resection for inflammatory bowel disease: A multi-center study.

BACKGROUND/PURPOSE: Children with inflammatory bowel disease (IBD) have increased risk for venous thromboembolism (VTE). We sought to determine incidence and risk factors for postoperative VTE in a multicenter cohort of pediatric patients undergoing colorectal resection for IBD. METHODS: Retrospective review of children ≤18 years who underwent colorectal resection for IBD from 2010 to 2016 was performed at four children's hospitals. Primary outcome was VTE that occurred between surgery and last follow-up. Factors associated with VTE were determined using univariable and multivariable analyses. RESULTS: Two hundred seventy-six patients were included with median age 15 years [13,17]. Forty-two children (15%) received perioperative VTE chemoprophylaxis, and 88 (32%) received mechanical prophylaxis. DVT occurred in 12 patients (4.3%) at a median of 14 days postoperatively [8,147]. Most were portomesenteric (n = 9, 75%) with the remaining catheter-associated DVTs in extremities (n = 3, 25%). There was no association with chemoprophylaxis (p > 0.99). On Cox regression, emergent procedure [HR 18.8, 95%CI: 3.18-111], perioperative plasma transfusion [HR 25.1, 95%CI: 2.4-259], and postoperative infectious complication [HR 10.5, 95%CI: 2.63-41.8] remained predictive of DVT. CONCLUSION: Less than 5% of pediatric IBD patients developed postoperative VTE. Chemoprophylaxis was not protective but rarely used. Patients with risk factors identified in this study should be monitored or given prophylaxis for VTE. LEVEL OF EVIDENCE: Treatment Study, Level III.

The Laparoscopic Versus Open Approach for Reduction of Intussusception in Infants and Children: An Updated Institutional Experience.

PURPOSE: We have previously demonstrated successful laparoscopic management after failed enema reduction of children with intussusception. The purpose of this study is to assess the effectiveness of our mature experience with laparoscopic reduction by evaluating operative success, duration of hospital stay, postoperative complications, and hospital readmission rates. MATERIALS AND METHODS: After IRB approval, a retrospective review was conducted on children (age 0-18 years) who failed enema reduction of intussusception between 2008 and 2017. Cases were classified as either open or laparoscopic. Demographic data, incidence of bowel resection, postoperative length of stay, complications, and hospital readmission rates were abstracted from patient medical records. Comparative analysis was performed in STATA with a P value <.05 determined as significant. RESULTS: A total of 81 children were included in our study with 63 patients (78%) undergoing a laparoscopic reduction and 18 patients (22%) undergoing an open operation. Laparoscopic reduction carried similar complication rates (11%) when compared with children undergoing an open reduction (11%, P = 1.00). Furthermore, both hospital readmission rates and returns to the operating room were similar between the two groups (P = .345 and P = .672, respectively). The median postoperative length of stay was shorter for patients undergoing a laparoscopic reduction (4 days, interquartile range [IQR], 2-5 days) than for patients undergoing an open reduction (5 days, IQR, 4-6 days, P = .001). Children undergoing a laparoscopic reduction had a decreased rate of bowel resection (43% versus 50%, P = .591) despite similar rate of pathological lead points (21% versus 22%, P = .884). CONCLUSION: Laparoscopic management of intussusception after failed radiographic reduction yields a reduced hospital length of stay with no increase in hospital readmission rates and reoperations.