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The Single Ventricle Reconstruction (SVR) Trial was the first randomized clinical trial of a surgical approach for treatment of congenital heart disease. Infants with hypoplastic left heart syndrome (HLHS) and other single right ventricle (RV) anomalies were randomized to a modified Blalock Taussig Thomas shunt (mBTTS) or a right-ventricular-to-pulmonary-artery shunt (RVPAS) at the time of the Norwood procedure. The aim of the Long-term Outcomes of Children with HLHS and the Impact of Norwood Shunt Type (SVR III) study is to compare early adolescent outcomes including measures of cardiac function, transplant-free survival, and neurodevelopment, between those who received a mBTTS and those who received an RVPAS.Transplant-free survivors of the SVR cohort were enrolled at 10 to 15 years of age for multifaceted in-person evaluation of cardiac function (cardiac magnetic resonance [CMR], echocardiogram and exercise test) and neurodevelopmental evaluation. Right ventricular ejection fraction measured by CMR served as the primary outcome. Development of arrhythmias, protein losing enteropathy, and other comorbidities were assessed through annual medical history interview. Through the course of SVR III, protocol modifications to engage SVR trial participants were designed to enhance recruitment and retention.Evaluation of long-term outcomes will provide important data to inform decisions about the shunt type placed at the Norwood operation and will improve the understanding of cardiovascular and neurodevelopmental outcomes for early adolescents with HLHS.

Background:Amongst patients with CHD, the time of transition to adulthood is associated with lapses in care leading to significant morbidity. The purpose of this study was to identify differences in perceptions between parents and teens in regard to transition readiness.Methods:Responses were collected from 175 teen–parent pairs via the validated CHD Transition Readiness survey and an information request checklist. The survey was distributed via an electronic tablet at a routine clinic visit.Results:Parents reported a perceived knowledge gap of 29.2% (the percentage of survey items in which a parent believes their teen does not know), compared to teens self-reporting an average of 25.9% of survey items in which they feel deficient (p = 0.01). Agreement was lowest for long-term medical needs, physical activities allowed, insurance, and education. In regard to self-management behaviours, agreement between parent and teen was slight to moderate (weighted κ statistic = 0.18 to 0.51). For self-efficacy, agreement ranged from slight to fair (weighted κ = 0.16 to 0.28). Teens were more likely to request information than their parents (79% versus 65% requesting at least one item) particularly in regard to pregnancy/contraception and insurance.Conclusion:Parents and teens differ in several key perceptions regarding knowledge, behaviours, and feelings related to the management of heart disease. Specifically, parents perceive a higher knowledge deficit, teens perceive higher self-efficacy, and parents and teens agree that self-management is low.

We previously reported common knowledge deficits and lack of transition readiness in 13- 25-year-olds with congenital or acquired heart disease. The aims of this study were to re-evaluate transition readiness in this cohort at follow-up and to examine the relationship between changes in transition readiness and quality of life (QOL).In this prospective cohort study, patients completed the Transition Readiness Assessment and the Pediatric Quality of Life Inventory using an e-tablet, web-based format at a routine follow-up visit. Changes from initial to follow-up scores were evaluated.Sixty-five percent of patients (106 of 164) completed follow-up assessments at a median age of 18.7 years (IQR, 16.5-21.2 years) at a median follow-up of 1 year. The average perceived knowledge deficit score (percent of items with no knowledge) at follow-up was 18.0 ± 15.2%, which decreased from 24.7 ± 16.5% (P .0001). On a 100-point scale, the mean score for self-efficacy increased from 71.4 ± 17.0 to 76.7 ± 18.2 (P = .0004) and for self-management increased from 47.9 ± 18.4 to 52.0 ± 20.7 (P = .004). Although physical QOL did not change, the mean psychosocial QOL score increased significantly (P = .02). A decrease in the knowledge deficit score at follow-up was significantly associated with an increased psychosocial QOL score (P = .03). An increase in the self-efficacy score was associated with an increase in psychosocial QOL score (P = .04), especially social QOL (P = .02).Although deficits in knowledge and self-management skills persist, transition readiness assessment and recognition of deficits can improve transition readiness with improved psychosocial QOL.

To examine changes in transition readiness (knowledge, self-efficacy, self-management) over time and explore factors associated with transition readiness, including psychosocial quality of life (QOL) and health service utilization in teens/young adults with congenital heart disease.In a multicenter prospective cohort study, 356 patients, age 14-27 years, completed transition readiness and QOL assessments at routine cardiology visits at baseline and 1-year follow-up.Median patient age was 19.8 years at 1.03 years (IQR 0.98-1.24) following baseline transition readiness assessment. Average knowledge deficit scores decreased at follow-up (P .0001) and self-efficacy scores increased (P .0001). Self-management scores increased (P .0001), but remained low (mean 57.7, 100-point scale). Information was requested by 73% of patients at baseline and was associated with greater increase in knowledge at follow-up (P = .005). Increased knowledge (P = .003) and perceived self-efficacy (P = .01) were associated with improved psychosocial QOL, but not health service utilization at follow-up. Patients who preferred face-to-face information from healthcare providers (47%) vs other information sources were more likely to request information (P .0001). In patients18 years old, greater agreement between teen and parental perception of teen's knowledge was associated with greater increase in patient knowledge (P = .02) and self-efficacy (P = .003).Transition readiness assessment demonstrated improved knowledge, self-efficacy, and self-management at 1-year follow-up in teens/young adults with congenital heart disease. Improved knowledge and self-efficacy were associated with improved psychosocial QOL. Self-management remained low. Supplemental media for conveying information and greater involvement of parents may be needed to optimize transition readiness.

To evaluate transition readiness, including perceived knowledge deficits, self-efficacy, and self-management behaviors, in 13- to 25-year-olds with congenital heart disease or heart transplant and to examine the relationships between transition readiness assessment, information seeking behavior, and quality of life (QOL).In this cross-sectional study, patients (n = 164) completed the Transition Readiness Assessment and the Pediatric Quality of Life Inventory using an e-tablet, web-based format at a routine clinic visit.Median patient age was 18.1 years (range 13.0-25.5). Average perceived knowledge deficit score (% of items with no knowledge) was 25.7% (range 0%-75%). On a 100-point scale, the mean score was 72.0 ± 17.2 for self-efficacy and 49.7 ± 17.5 for self-management. Knowledge deficits were negatively correlated with self-efficacy (r = -0.45, P.0001) and self-management (r = -0.36, P.0001). Overall, 66% of patients requested information (73% ≥ 18 years old). Higher psychosocial QOL scores were correlated with lower knowledge deficit scores (r = -0.21, P = .01) and higher perceived self-efficacy scores (r = 0.41, P.0001).Transition knowledge deficits are common and associated with decreased self-efficacy and self-management skills in adolescents and young adults with heart disease. Greater transition knowledge and perceived self-efficacy are associated with better psychosocial QOL. Routine assessment of transition readiness is recommended to facilitate recognition of deficits and interventions to promote successful transition outcomes.