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Adolescents with congenital heart disease transition from childhood to adulthood and transfer from pediatric-oriented to adult-oriented care. High-level empirical evidence on the effectiveness of transitional care is scarce. This study investigated the empowering effect (primary outcome) of a structured person-centered transition program for adolescents with congenital heart disease and studied its effectiveness on transition readiness, patient-reported health, quality of life, health behaviors, disease-related knowledge, and parental outcomes e.g., parental uncertainty, readiness for transition as perceived by the parents (secondary outcomes). The STEPSTONES-trial comprised a hybrid experimental design whereby a randomized controlled trial was embedded in a longitudinal observational study. The trial was conducted in seven centers in Sweden. Two centers were allocated to the randomized controlled trial-arm, randomizing participants to intervention or control group. The other five centers were intervention-naïve centers and served as contamination check control group. Outcomes were measured at the age of 16 years (baseline), 17 years, and 18.5 years. The change in empowerment from 16 years to 18.5 years differed significantly between the intervention group and control group (mean difference = 3.44; 95% confidence interval = 0.27–6.65; p =.036) in favor of intervention group. For the secondary outcomes, significant differences in change over time were found in parental involvement (p =.008), disease-related knowledge (p =.0002), and satisfaction with physical appearance (p =.039). No differences in primary or secondary outcomes were detected between the control group and contamination check control group, indicating that there was no contamination in the control group. The STEPSTONES transition program was effective in increasing patient empowerment, reducing parental involvement, improving satisfaction with physical appearance, and increasing disease-related knowledge. [Display omitted] [ABSTRACT FROM AUTHOR]

Transposition of the great arteries (TGA) is a complex congenital heart disease that requires early diagnosis as well as advanced surgical repair and postoperative support. This study sought to investigate the impact of surgical timing on early postoperative morbidity. This study reviewed all patients with TGA repaired at a single institution (Skåne University Hospital, Lund, Sweden) by arterial switch operation (ASO) between June 2001 and June 2017. Major postoperative morbidity (MPM) and death within 30 days after ASOs were documented. Patients with double-outlet right ventricle, chromosomal abnormalities, and noncardiac diseases were excluded. MPM was defined as the presence of at least 1 of the following: delayed sternum closure, reoperation, prolonged mechanical ventilation, noninvasive ventilation after extubation, peritoneal dialysis, extracorporeal membrane oxygenation, and readmission. A total of 241 patients were included, with medians for birth weight, gestational week, and age at surgery of 3.5 kg, 39 weeks, and 5 days, respectively. MPM was encountered in 32.3% of patients. Prematurity (P =.001) and need for aortic arch repair at the time of ASO (P =.04) were associated with a significant increase in MPM. Non-A coronary anatomy, associated ventricular septal defect requiring surgical closure, and fetal diagnosis of TGA had no significant impact on MPM (P =.35,.08, and.21, respectively). There was no significant difference in MPM among the surgical groups (P =.49). Early complications after ASO do occur and are mostly associated with prematurity and the need for aortic arch repair. Timing of surgical repair does not seem to influence the rate of these complications. [ABSTRACT FROM AUTHOR]

Objective: Pulmonary venous obstruction (PVO) is an important cause of late mortality in total anomalous pulmonary venous connection (TAPVC). We aimed to describe current practices for the management of postoperative PVO and the efficacy of the different interventional procedures. Methods: We conducted a retrospective international collaborative population-based study involving 19 pediatric cardiac centers in the United Kingdom, Ireland, and Sweden. Patients with TAPVC born between January 1, 1998, and December 31, 2004, were identified. Patients with functionally univentricular circulation or atrial isomerism were excluded. All available data and images were reviewed. Results: Of 406 patients undergoing repair of TAPVC, 71 (17.5%) had postoperative PVO. The diagnosis was made within 6 months of surgery in 59 (83%) of the 71 patients. In 12, serial imaging documented change in appearance of the pulmonary veins. Good-sized pulmonary veins can progress to diffusely small veins and rarely atresia. Patients presenting after 6 months had less severe disease; all are alive at most recent follow-up. Fifty-six (13.8%) of 406 patients underwent intervention for postoperative PVO: 44 had surgical treatment and 12 had an initial catheter intervention. One half underwent 1 or more reinterventions. Three-year survival for patients with postoperative PVO was 58.7% (95% confidence intervals, 46.2%-69.2%) with a trend that those having a surgical strategy did better (P = .083). Risk factors for death included earlier presentation after TAPVC repair, diffusely small pulmonary veins at presentation of postoperative PVO, and an increased number of lung segments affected by obstruction. Conclusions: Postoperative PVO tends to appear in the first 6 months after TAPVC repair and can be progressive. Early intervention for PVO may be indicated before irreversible secondary changes occur. [Copyright &y& Elsevier]