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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.

Objectives:Virtual reality has emerged as a unique educational modality for medical trainees. However, incorporation of virtual reality curricula into formal training programmes has been limited. We describe a multi-centre effort to develop, implement, and evaluate the efficacy of a virtual reality curriculum for residents participating in paediatric cardiology rotations.Methods:A virtual reality software program (“The Stanford Virtual Heart”) was utilised. Users are placed “inside the heart” and explore non-traditional views of cardiac anatomy. Modules for six common congenital heart lesions were developed, including narrative scripts. A prospective case–control study was performed involving three large paediatric residency programmes. From July 2018 to June 2019, trainees participating in an outpatient cardiology rotation completed a 27-question, validated assessment tool. From July 2019 to February 2020, trainees completed the virtual reality curriculum and assessment tool during their cardiology rotation. Qualitative feedback on the virtual reality experience was also gathered. Intervention and control group performances were compared using univariate analyses.Results:There were 80 trainees in the control group and 52 in the intervention group. Trainees in the intervention group achieved higher scores on the assessment (20.4 ± 2.9 versus 18.8 ± 3.8 out of 27 questions answered correctly, p = 0.01). Further analysis showed significant improvement in the intervention group for questions specifically testing visuospatial concepts. In total, 100% of users recommended integration of the programme into the residency curriculum.Conclusions:Virtual reality is an effective and well-received adjunct to clinical curricula for residents participating in paediatric cardiology rotations. Our results support continued virtual reality use and expansion to include other trainees.

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.

Introduction: Anatomic repair of congenitally corrected transposition of the great arteries (ccTGA) generally poses short-term risk for presumed long-term functional benefit by restoring the left ventricle to the systemic circulation. Understanding early and late surgical outcomes is crucial for counseling patients and families. Methods: All patients with anatomic repair at a single institution from 1993-2018 were included. Follow-up data was obtained by contact with patients’ primary cardiologists. The primary outcome was long-term survival and the secondary outcome was freedom from reintervention. Univariate Cox proportional hazard model was used to identify risk factors for mortality. In-hospital mortality and morbidity were compared between the most recent (2010-2018) and past surgical eras. Results: Anatomic repair was performed in 118 patients: 64 Senning/arterial switch operations (ASO) and 54 Senning/Rastelli operations. Survival was 85%, 82%, and 70% at 1, 10, and 20 years after repair, respectively, and was significantly lower in the Senning/Rastelli group compared to Senning/ASO (Hazard ratio 3.0, p=0.01; Figure 1). Preoperative factors associated with mortality were heterotaxy syndrome (p=0.03) and right ventricular systolic dysfunction (p=0.01). During a median follow up of 9.4 years, 47 of 98 patients (48%) required surgical or catheter-based reintervention; median freedom from reintervention was 11.7 years. For the most recent era, 30 of 32 patients (94%) with Senning/ASO and 19 of 22 patients (86%) with Senning/Rastelli operations survived to hospital discharge. In-hospital morbidity was similar between eras (30% in 1993-2009 and 28% in 2010-2018, p=0.82). Conclusions: Short- and long-term outcomes are favorable following Senning/ASO. Mortality following Senning/Rastelli operations remains high, highlighting the need for careful patient selection and family counseling.

Introduction: After anatomic repair (AR) of congenitally corrected transposition of the great arteries (ccTGA), the left ventricle (LV) is re-established as the systemic ventricle. As such, LV dysfunction is an important factor in long-term patient morbidity. While predictors exist for successful AR, little is known regarding measures of LV function before and after AR, and no known predictors exist of worsening global longitudinal strain (GLS). Methods: All patients with ccTGA who survived to discharge after AR at a single institution from 2000-2018 were included. Pre-operative and discharge echocardiograms were reviewed and GLS analysis was performed. LV sphericity index was measured to determine the degree of ventricular septal shift; a lower index denotes more septal bowing into the LV. Logistic regression was used to identify factors associated with worsened GLS after AR. Results: Of 91 survivors (65% male), 51 underwent Senning/arterial switch (ASO) and 40 had Senning/Rastelli. Mean LV ejection fraction (EF) decreased from 66% to 61% after AR (p = 0.0003) with 26% having LVEF less than or equal to 55% (increased from 4% pre-op, p = 0.0003). Mean LV GLS was normal pre-op (-20.0% ± SD 4.1%) but worsened following AR (-16.5% ± 4.6%; p -18%) increased to 62% (vs 32% pre-op, p Conclusions: Following AR of ccTGA, LVEF decreases but remains in the normal range, while LV GLS detects abnormalities. Risk factors for worsening GLS include abnormal pre-op LV GLS, lower LV sphericity index, and a Senning/ASO repair. Lower LV sphericity index, or more septal shift into the LV, may represent a “less trained” LV, highlighting the need for careful patient selection for AR.

Introduction: Children with complex congenital heart disease (CHD) are at increased risk for neurodevelopmental impairments and reduced quality of life (QOL). Patients (pts) with congenitally corrected transposition of the great arteries (ccTGA) often undergo anatomic repair to make the left ventricle the systemic ventricle, with the goal of long term cardiac benefits. Understanding long-term outcomes and QOL may inform preoperative counseling and treatment strategies for ccTGA. Methods: We measured QOL using PedsQL generic and cardiac modules for pts following anatomic repair of ccTGA with Senning/Arterial Switch or Senning/Rastelli operations. Impaired QOL by the PedsQL is defined by total scores Results: Of 88 eligible pts, 56 (64%) returned surveys (29% female, median age 11.8 years). Respondents were more likely to have pre-operative arrhythmias or pacemaker implantation compared to non-respondents. Mean total PedsQL score was 72 + 19 for pt report and 75 + 18 for parent report; 22 of 56 (39%) pts had impaired QOL scores, compared to 20% impaired scores in pts with any type of CHD, previously described. Mean physical functioning score was 72 + 22 for pt report and 76 + 21 for parent report. Mean psychosocial summary score was 73 + 18 and 75 + 18 for pt and parent report, respectively. Communication was the lowest scored subscale of the Cardiac module by pt (63 + 26) and parent (66 + 31) report. The treatment II subscale addressing medication compliance was the highest scored subscale with scores of 90 + 8 for pts and 91 + 15 for parent, respectively. Longer cardiopulmonary bypass time (p = 0.01), surgical or catheter-based reintervention following hospital discharge (p = 0.02) and heart failure symptoms (p = 0.04) were associated with impaired QOL. Conclusions: After anatomic repair of ccTGA, pts are at risk of impaired QOL compared to healthy controls and to pts with a broad range of CHD. Indicators of more complex surgical and post-operative courses were associated with impaired QOL.

Introduction: Limited data exist on the electrophysiologic outcomes of patients undergoing anatomic repair (AR) for congenitally corrected transposition of the great arteries (ccTGA). AR was defined as an atrial switch procedure plus either arterial switch (ASO) or Rastelli operation. Aims: To report mid and late electrophysiologic outcomes after AR and identify risk factors for those outcomes. Methods: Single center retrospective cohort study of patients undergoing AR between 1993-2017. Data were collected from available records. Transplant-free survival to 1 year post repair was required for inclusion. Standard descriptive statistical analysis and Cox proportional hazards were used. Results: Of 85 patients included, 95% had lesions in addition to ccTGA: most commonly VSD (84%) and pulmonary stenosis or atresia (58%). Median age at AR was 1.5y (IQR 0.9-2.8) with Senning/ASO in 56%, Senning/Rastelli in 38%, and hemi-Senning/Glenn/Rastelli in 6%. During a median follow-up of 10.6y, 45 (53%) patients developed an arrhythmia requiring intervention. Atrial tachycardia (AT) in 27 (32%) or ventricular tachycardia (VT) in 11 (13%) patients required intervention at a median of 7.4y (IQR 1.6-15.3y) and 15.9y (IQR 4.5-17.9) post-AR, respectively. Treatments included chronic medications in 29 (64%), cardioversion in 15 (33%) and catheter ablation in 10 (22%). Median freedom from AT and VT was 17.3y and 25y post-AR, respectively. D-looped ventricles (p=0.03) and multiple operations prior to AR (p=0.02) were associated with increased AT risk; and native pulmonary stenosis with increased VT risk (p=0.01). Those needing heart failure/transplant referral had increased risk of both AT and VT (both p=0.04). Pacemaker was implanted for heart block and/or SND prior to or during AR in 14 (16%), immediately post-op in 9 (11%), and late (median 6y post-AR) in 24 (28%). ICDs were implanted in 5 (6% of cohort), 4 for primary prevention. No patient had an appropriate shock. Conclusions: Anatomic ccTGA repair is associated with significant electrophysiologic morbidity. AT, VT, and SND develop at a similar incidence to that reported for d-TGA patients after atrial switch. The incidence of AV block follows a similar trajectory to that of physiologically palliated ccTGA.

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.

Objective: Transition is defined as “the process by which adolescents and young adults with chronic childhood illnesses are prepared to take charge of their lives and their health in adulthood”. We previously reported common knowledge deficits and lack of transition readiness (TR) in 13-25 year olds with congenital or acquired heart disease. The aims of this study were to re-evaluate TR in these patients at follow-up (F/U) and to examine the relationship between changes in TR and quality of life (QOL). Methods: Patients (n=106) completed the TR Assessment and Pediatric Quality of Life Inventory (PedsQL) utilizing an e-tablet, web-based format at a routine F/U clinic visit. Changes from initial to F/U scores were evaluated. Results: Median patient age was 18.7 yrs at a median F/U time of 1.02 yrs. Average perceived knowledge deficit score (% of items with no knowledge) at F/U was 18.0 ± 15.2%, decreased from 24.7 ± 16.5%, p Conclusion: While deficits in knowledge and self-management skills persist, TR assessment and recognition of deficits can improve transition readiness with improved psychosocial QOL. Routine TR assessment is important to identify transition needs. Further studies are needed to examine the relationship between TR and outcomes in young adults with heart disease.