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Objectives Approximately 0.2% to 2.7% of children with congenital heart disease require a tracheostomy after cardiac surgery with the majority having single ventricle (SV) type heart lesions. Tracheostomy in SV patients is reported to be associated with high mortality. We hypothesized that short- and long-term survival of patients with SV heart disease would vary according to tracheostomy indication. Methods This is a single center, 20-year, retrospective review of all patients with SV heart disease who underwent tracheostomy. Demographic, cardiac anatomy, surgical, intensive care unit, and hospital course data were collected. The primary outcome was survival following tracheostomy. Secondary outcome was the completion of staged palliation to Fontan. Results In total, 25 patients with SV heart disease who underwent tracheostomy were included. Indications for tracheostomy included one or more of the following: tracheobronchomalacia (n = 8), vocal cord paralysis (n = 7), tracheal/subglottic stenosis (n = 6), primary respiratory insufficiency (n = 4), diaphragm paralysis (n = 3), suboptimal hemodynamics (n = 2), and other upper airway issues (n = 1). Survival at six months, one year, five years, and ten years was 76%, 68%, 63%, and 49%, respectively. Most patients completed Fontan palliation (64%). Patients who underwent tracheostomy for suboptimal hemodynamics and/or respiratory insufficiency had a higher mortality risk compared to those with indications of upper airway obstruction or diaphragm paralysis (hazard ratio 4.1, 95% confidence interval 1.2-13.7; P = .02). Conclusions Mortality risk varies according to tracheostomy indication in patients with SV heart disease. Tracheostomy may allow staged surgical palliation to proceed with acceptable risk if it was indicated for anatomic or functional airway dysfunction.

Background: Burnout is well characterised in physicians and residents but not in paediatric cardiology fellows, and few studies follow burnout longitudinally. Training-specific fears have been described in paediatric cardiology fellows but also have not been studied at multiple time points. This study aimed to measure burnout, training-specific fears, and professional fulfilment in paediatric cardiology fellows with the attention to time of year and year-of-training. Methods: This survey-based study included the Professional Fulfillment Index and the Impact of Events Scale as well as an investigator-designed Fellow Fears Questionnaire. Surveys were distributed at three-time points during the academic year to paediatric cardiology fellows at a large Midwestern training programme. Fellow self-reported gender and year-of-training were collected. Descriptive analyses were performed. Results: 10/17 (59%) of fellows completed all surveys; 60% were female, 40% in the first-year class, 40% in the second-year class, and 20% in the third-year class. At least half of the fellows reported burnout at each survey time point, with lower mean professional fulfilment scores. The second-year class, who rotate primarily in the cardiac ICU, had higher proportions of burnout than the other two classes. At least half of fellows reported that they “often” or “always” worried about not having enough clinical knowledge or skills and about work–life balance. Conclusions: Paediatric cardiology fellows exhibit high proportions of burnout and training-specific fears. Interventions to mitigate burnout should be targeted specifically to training needs, including during high-acuity rotations.

Kommerell diverticulum (KD) is a dilated proximal aberrant right or left subclavian artery associated with either right or left aortic arches (RAA-ARSA or LAA-ALSA). Although case series suggest that KD may be a liability for vascular complications, the risk, pattern of dilation throughout the life span, and differences between arch sides are not known.This study was a single-center retrospective review of patients of all ages with KD on cross-sectional imaging. Maximal short-axis diameter of KD (KDmax), absolute and indexed to descending aortic diameter (DAo), was correlated with age. Comparisons were made between arch sides. Patients with vascular complications were described.A total of 104 patients with KD were included: 68 (65%) with RAA-ALSA, 36 (35%) with LAA-ARSA, 43 (41%) asymptomatic. Although KDmax was correlated with age (RAA-ALSA r = 0.84; [P.0001]; LAA-ARSA r = 0.51 [P = .001]), KDmax indexed to DAo was not (RAA-ALSA r = 0.14 [P = .27]; LAA-ARSA r = -0.22 [P = .21]). Patients with RAA-ALSA had larger KDmax indexed to DAo (1.02 ± 0.20 mm/mm vs 0.89 ± 0.18 mm/mm; P = .002) and more symptoms (75% vs 28%; P.0001), and they were younger (median, 9.5 years vs 61.7 years; P.0001). Six patients (58 to 80 years of age) had vascular complications, and all 6 had LAA-ARSA and risk factors for acquired aneurysms.In older patients, KDmax indexed to DAo was not larger, thus arguing against isolated KD dilation with age. Diverticula from RAA-ALSA and LAA-ARSA demonstrated different phenotypes, a finding suggesting different disease processes and likely different risk. The incidence of vascular complications was lower than in previous reports, and these complications occurred exclusively in patients with LAA-ARSA and aneurysm risk factors. This finding suggests that conservative management of asymptomatic KD is often reasonable, especially in patients with RAA-ALSA.

The aim of this study was to evaluate the accuracy of cardiac magnetic resonance (CMR) in predicting coronary artery (CA) compression during transcatheter pulmonary valve implantation (TPVi).TPVi is a widely available option to treat dysfunctional right ventricle (RV)-to-pulmonary artery (PA) conduits, but CA compression is an absolute contraindication. CMR can evaluate coronary anatomy, but its utility in predicting CA compression is not well established.After Institutional Review Board approval was obtained, all patients at 9 centers with attempted TPVi in RV-PA conduits and recent CMR (≤12 months) were analyzed. A core laboratory reviewed all CMR studies for the shortest orthogonal distance from a CA to the conduit, the shortest distance from a CA to the most stenotic area of the conduit, and subjective assessment of CA compression risk.Among 231 patients, TPVi was successful in 198 (86%); in 24 (10%), balloon testing precluded implantation (documented CA compression or high risk). Distance to the RV-PA conduit ≤2.1 mm (area under the curve [AUC]: 0.70) and distance to most stenotic area ≤13.1 mm (AUC: 0.69) predicted CA compression. Subjective assessment had the highest AUC (0.78), with 96% negative predictive value. Both distances and qualitative assessment remained independently associated with CA compression when controlling for abnormal coronary anatomy or degree of conduit calcification.CMR can help predict the risk for CA compression during TPVi in RV-PA conduits but cannot completely exclude CA compression. CMR may assist in patient selection and counseling families prior to TPVi, although balloon testing remains essential.

We examined the status of women's representation on radiology journal editorial boards around the world and evaluated temporal trends in female board representation.Data on the gender of editorial board members, deputy editors, and editors-in-chief of top general radiology journals from the United States and from five continents outside North America was collected, based on listings in mastheads of each journal at 5-year intervals from 1998 to 2018. Female editorial board representation was compared to female membership in national professional radiology organizations and national physician registries.One of 42 editors-in-chief (2.4%), 24 of 135 deputy editors (17.8%), and 345 of 2545 (13.6%) editorial board members were female for the 20-year period. The overall percentage of female editorial board members increased from 9.7% (41 of 423) in 1998 to 17.0% (97 of 569) in 2018 (p0.0001). In 2018, women were less represented on the editorial boards than female professional society membership for all geographic regions except Australia/New Zealand; this difference reached statistical significance in Korea, Brazil, Europe, and China. Female editorial board representation ranged from 11.3% to 33.3%, with no significant difference between the United States (21.5%) and other countries.Although the proportion of female editorial board members of radiology journals worldwide has increased over the past two decades, female editorial board composition generally underrepresents the percentage of female radiologists. Editorial board female representation does not differ significantly between the United States and other countries.

Under-diagnosis of pediatric hypertension remains pervasive due to difficulty recognizing elevated systolic blood pressures (SBPs). We performed a retrospective review comparing recognition of and response to elevated SBPs ≥95th percentile before and after development of a clinical decision support tool (CDST) in an academic pediatric system. Of 44,351 encounters, 477 had elevated SBPs with documented recognition of an elevated SBP in 17.9% of encounters pre-CDST that increased to 33.7% post-CDST ( P = .001). Post-CDST, 75.5% of elevated SBPs had repeat measurement, with 90.8% of initially elevated SBPs normalizing to

In patients with congenitally corrected transposition of the great arteries (ccTGA) and hemodynamically significant concomitant lesions, physiologic repair may be undertaken, in which the circulation is septated but the morphologic right ventricle (RV) remains the systemic ventricle. Patients without significant concomitant lesions may be observed without surgery, with a similar physiologic result. We compared cardiovascular magnetic resonance measures of ventricular size and function in patients with physiologically repaired and unrepaired ccTGA. Patients with ccTGA who underwent cardiovascular magnetic resonance at our center between September 2007 and July 2019 were analyzed. In 38 patients identified (12, physiologically repaired; 26, unrepaired; mean age 34.5 [18.7 to 52.0] years), there was a higher proportion of RV ejection fraction ≤45% in physiologically repaired (75% vs unrepaired 35%, p = 0.02). Physiologically repaired patients had worse left ventricle global longitudinal strain (-14.9% ± 5.0% vs unrepaired patients -18.4% ± 2.7%, p = 0.04). The difference in tricuspid regurgitant fraction between groups did not achieve statistical significance (physiologically repaired 27.4 ± 11.1% vs unrepaired patients 19.2 ± 13.0%, p = 0.08). Evaluation for late gadolinium enhancement was more commonly undertaken in physiologically repaired patients (8 of 12 vs unrepaired 7 of 26, p = 0.03) and present more frequently in the left ventricle in physiologically repaired patients in patients evaluated (6 of 8 vs unrepaired 0 of 7, p = 0.01). In conclusion, ventricular function is decreased in patients with ccTGA undergoing physiologic repair compared with those without previous surgery. These cohorts should be considered separately when using ventricular function as an outcome. RV dysfunction is concerning for long-term outcomes following physiologic repair.

There are conflicting data on how delivery location impacts outcomes in neonates with ductal-dependent heart disease. Our goal was to evaluate the impact of delivery location on hospital length of stay and survival in infants with prenatally diagnosed hypoplastic left heart syndrome (HLHS) after stage 1 palliation (S1P). A multicenter cohort study was performed utilizing the National Pediatric Cardiology Quality Improvement Collaborative dataset for infants with prenatally diagnosed HLHS who underwent S1P from August 2016 to December 2018. Univariate comparisons of demographics, clinical, and outcome data were made and multivariable logistic regression was performed between groups stratified by distance from surgical center. A total of 790 patients from 33 centers were analyzed: 85% were born

Fontan palliation has improved survival for single ventricle patients, but long-term complications persist including cardiovascular dysfunction, neurohormonal abnormalities, and protein-losing enteropathy (PLE). Although chronic inflammation contributes to morbidity, an association between inflammation and vascular dysfunction has not been studied. We assessed inflammation and vascular function in 31 Fontan-palliated patients (52% male, median age 14.3 years), including 10 PLE+. Fontan circulation was associated with altered inflammatory cytokines (TNF-α: mean 2.5 ± 1.4 vs. 0.7 ± 0.2 pg/ml, p0.0001; sTNFR2: 371 ± 108 vs. 2694 ± 884 pg/ml, p0.0001) and vascular dysfunction [log-transformed reactive hyperemia index (lnRHI) 0.28 ± 0.19 vs. 0.47 ± 0.26, p0.01; augmentation index (AI) -2.9 ± 13.8 vs. -16.3 ± 12.0, p = 0.001; circulating endothelial progenitor cells (cEPCs) 5.0 ± 8.1 vs. 22.8 ± 15.9, p = 0.0002)]. Furthermore, PLE+ patients showed greater inflammation (IFN-γ 6.3 ± 2.2 vs. 11.5 ± 7.9 pg/ml, p = 0.01; sTNFR1: 1181 ± 420 vs. 771 ± 350 pg/ml, p = 0.01) and decreased arterial compliance (AI: 5.4 ± 17.1 vs. -6.8 ± 10.2, p = 0.02) than PLE- patients. Circulating EPCs, but not inflammatory cytokines, were inversely associated with arterial stiffness in Fontan patients. In conclusion, chronic inflammation and vascular dysfunction are observed after Fontan operation, with greater inflammation and arterial stiffness in Fontan patients with active PLE. However, there is no clear association between inflammatory cytokines and vascular dysfunction, suggesting these pathophysiologic processes are not mechanistically linked.

Introduction: Prolonged pleural effusions are common post Fontan operation and are associated with morbidity. Fontan pleural effusions have elevated proinflammatory cytokines. Little is known about the chest tube drainage after a superior cavopulmonary connection. We examined the chest tube drainage and the inflammatory profiles in post-operative superior cavopulmonary connection patients. Methods: This prospective cohort study enrolled 25 patients undergoing superior cavopulmonary connection and 10 age-similar controls. Data are also compared to 25 previously published Fontan patients and their 15 age-similar controls. Chest tube samples were analysed with a 17-cytokine BioPlex Assay. Descriptive statistics and univariate comparisons were made between groups. Results: Duration of chest tube drainage was significantly shorter in superior cavopulmonary connection patients (median 4 days, [interquartile range 3–5 days]) versus Fontan patients (10 days, [7–11 days], p < 0.0001). Cytokine concentrations were higher on post-operative day 1 in superior cavopulmonary connection patients versus Fontan patients (all p ≤ 0.01), however levels were comparable to age-similar controls. While proinflammatory IL 8, MIP-1β, and TNF-α concentrations increased in chest tube drainage of Fontan patients from post-operative day 1 to last chest tube day (all p < 0.0001), there was no change in these biomarkers in superior cavopulmonary connection patients, their controls, or Fontan controls. Conclusions: Our study demonstrates that after superior cavopulmonary connection, proinflammatory cytokines in the chest tube drainage remain similar to biventricular controls of both age groups, unlike the significant rise over time observed in Fontan patients. Inflammation within the chest tube drainage is likely not innate to single ventricle patients.

BACKGROUND In patients with repaired tetralogy of Fallot (TOF), key echocardiogram report elements have been identified, but poor adherence has been demonstrated, particularly for quantitative assessment. We report a quality improvement effort to improve adherence at our institution, with a focus on increasing quantitative assessment of right ventricular (RV) function. METHODS Baseline compliance was established by a 3-month retrospective review of outpatient echocardiogram reports. Intervention 1 included presenting baseline data and reviewing the guidelines with echocardiogram laboratory staff (physicians and sonographers). Intervention 2, chosen to focus on quantitative assessment of RV function, involved recommending measurement of tricuspid annular plane systolic excursion (TAPSE) for all echocardiograms. Reporting rates were prospectively analyzed for 1 month after each intervention. To evaluate sonographer versus physician compliance, both study images (acquisition of TAPSE images) and reports were reviewed. RESULTS At baseline, adherence was poor (median 65% of elements reported), with lower rates for measurements versus descriptive elements (median 40% vs 78%, p

ImportanceDespite high disease morbidity and mortality and complex treatment decisions, little is known about the medical and end-of-life decision-making preferences of adolescents and young adults (AYA) with advanced heart disease. AYA decision-making involvement is associated with important outcomes in other chronic illness groups.ObjectiveTo characterize decision-making preferences of AYAs with advanced heart disease and their parents and determine factors associated with these preferences.Design, Setting, and ParticipantsCross-sectional survey between July 2018 and April 2021 at a single-center heart failure/transplant service at a Midwestern US children’s hospital. Participants were AYAs aged 12 to 24 years with heart failure, listed for heart transplantation, or posttransplant with life-limiting complications and a parent/caregiver. Data were analyzed from May 2021 to June 2022.Main Outcomes and MeasuresSingle-item measure of medical decision-making preferences, MyCHATT, and Lyon Family-Centered Advance Care Planning Survey.ResultsFifty-six of 63 eligible patients enrolled in the study (88.9%) with 53 AYA-parent dyads. Median (IQR) patient age was 17.8 (15.8-19.0) years; 34 (64.2%) patients were male, and 40 patients (75.5%) identified as White and 13 patients (24.5%) identified as members of a racial or ethnic minority group or multiracial. The greatest proportion of AYA participants (24 of 53 participants [45.3%]) indicated a preference for active, patient-led decision-making specific to heart disease management, while the greatest proportion of parents (18 of 51 participants [35.3%]) preferred they and physician(s) make shared medical decisions on behalf of their AYA, representing AYA-parent decision-making discordance (χ2 = 11.7; P = .01). Most AYA participants stated a preference to discuss adverse effects or risks of treatment (46 of 53 participants [86.8%]), procedural and/or surgical details (45 of 53 participants [84.9%]), impact of condition on daily activities (48 of 53 participants [90.6%]), and their prognosis (42 of 53 participants [79.2%]). More than half of AYAs preferred to be involved in end-of-life decisions if very ill (30 of 53 participants [56.6%]). Longer time since cardiac diagnosis (r = 0.32; P = .02) and worse functional status (mean [SD] 4.3 [1.4] in New York Heart Association class III or IV vs 2.8 [1.8] in New York Heart Association class I or II; t-value = 2.7; P = .01) were associated with a preference for more active, patient-led decision-making.Conclusions and RelevanceIn this survey study, most AYAs with advanced heart disease favored active roles in medical decision-making. Interventions and educational efforts targeting clinicians, AYAs with heart disease, and their caregivers are needed to ensure they are meeting the decision-making and communication preferences of this patient population with complex disease and treatment courses.

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.

OBJECTIVES Neonatal cardiac surgery for congenital cardiac defects is associated with significant morbidity and mortality, and there is a need for early identification of patients at highest risk of adverse outcomes. Because vascular endothelial injury mediates damage across organ systems, we measured serum biomarkers of endothelial injury in neonates following cardiopulmonary bypass and examined their associations with short-term outcomes. DESIGN Prospective cohort study. SETTING Pediatric cardiac ICU. PATIENTS Thirty neonates less than 2 weeks old undergoing repair of congenital cardiac defects with cardiopulmonary bypass. INTERVENTIONS None. MEASUREMENTS AND MAIN RESULTS Biomarkers of endothelial integrity, angiopoietin-1 and angiopoietin-2, were measured preoperatively and at 24 hours postoperatively. A composite adverse outcome was defined as any of the following: stroke, need for renal replacement therapy, extracorporeal membrane oxygenation support, cardiac arrest, or death. Associations of biomarkers with adverse outcomes were examined using Wilcoxon rank-sum test. There was an increase in angiopoietin-2 from preoperatively to 24 hours postoperatively (p < 0.0001) and a decrease in angiopoietin-1 from preoperatively to 24 hours postoperatively (p < 0.0001). Patients with greater rise in angiopoietin-2 from preoperatively to 24 hours postoperatively had greater risk of composite adverse outcome (p = 0.04). They had a trend toward higher Vasoactive-Inotropic Score (p = 0.06) and a higher prevalence of low cardiac output syndrome (p = 0.06). Twenty-four hour postoperative angiopoietin-2 level was associated with the composite adverse outcome (p = 0.03). The rise in angiopoietin-2 level from preoperatively to 24 hours postoperatively directly correlated with cardiopulmonary bypass duration (r = 0.47; p = 0.01). CONCLUSIONS In neonatal cardiac surgery, longer duration of cardiopulmonary bypass is directly associated with greater endothelial injury as measured by increased serum levels of angiopoietin-2. Angiopoietin-2 levels 24 hours postoperatively were significantly associated with a composite adverse outcome. Postoperative angiopoietin-2 level may serve as an early indicator of patients in need of closer monitoring and protective intervention. Further research into endothelial protective strategies is warranted.

OBJECTIVE The objective was to evaluate and improve accuracy of anticipatory counseling regarding neonatal intervention for prenatally diagnosed tetralogy of Fallot (TOF) by assessing new and previously published predictors of neonatal intervention. METHODS This is a multi-center, retrospective study from three centers of 112 fetal TOF patients undergoing third trimester fetal echocardiograms from 2004 to 2017. Additional cardiac defects requiring neonatal intervention were excluded. Fetal echocardiographic, clinical, and consultation data were compared between neonatal and late intervention. Optimal echocardiographic values were determined. RESULTS Twenty-six infants (23%) required neonatal intervention. Those infants had significantly different pulmonary valve (PV) z-scores, PV:aortic valve (AoV) ratios, PV:AoV z-score differences (absolute difference between z-scores), and increased likelihood of abnormal ductal flow. Counseling during fetal echocardiogram regarding interventional timing was accurate for 50% needing neonatal intervention and 86% undergoing late intervention (P = .002). The best neonatal intervention predictors were PV:AoV ratio of

Patients with d-looped transposition of the great arteries (d-TGA), especially those without an adequate atrial septal defect, can experience severe hypoxemia and hemodynamic compromise in the neonatal period. This can be mitigated by urgent balloon atrial septostomy (BAS). However, some patients with d-TGA are born at centers without this capability. The aim of this retrospective study of d-TGA patients who had urgent or emergent BAS at our institution between 2010 and 2021 was to evaluate time from birth to BAS for infants born at a tertiary care center as compared to those requiring transport from other institutions and to examine correlation between time to BAS and patient outcomes. Our primary outcome was time from birth to BAS. Secondary outcomes included hospital and ICU length of stay, mortality, and evidence of pulmonary or neurologic abnormalities including pulmonary hypertension, abnormal neuroimaging, or seizures. Of 96 patients, 67 (70%) were born at our institution. The median time to BAS was 4 hours for patients born at our institution vs. 14.1 hours for those born elsewhere (p

We sought to describe QOL in children with VAD and to identify factors associated with impaired QOL.There were 82 children (6-19 years) in the Pediatric Interagency Registry for Mechanical Circulatory Support who completed the PedsQL +/- a VAD-specific QOL assessment pre-VAD implant (n = 18), 3 months post-VAD (n = 63), and/or 6 months post-VAD (n = 38). Significantly impaired QOL is a score1 SD below norms.Study patients were 59% male, 67% Caucasian, with cardiomyopathy diagnosis in 82%, and median age at implant of 14 y (IQR 11-17). PedsQL scores were lower than norms for physical (p .0001) and psychosocial (p .01) QOL in pre- and post-VAD groups. Compared to chronic health condition and complex or severe heart disease groups, PedsQL scores were lower for physical and psychosocial QOL in the pre-VAD group (p .0001); however, psychosocial QOL was not significantly different in post-VAD groups. Psychosocial QOL was impaired in 67%, 40%, and 24% in pre-VAD, 3-month, and 6-month post-VAD groups, respectively. Total and psychosocial QOL scores were significantly higher in the 3-month and 6-month post-VAD group than pre-VAD (all p ≤ .02). VAD patients were most bothered by their inability to participate in usual play activities. Impaired QOL 3 months post-VAD was associated with inotropic support2 weeks/ongoing post-VAD (p = .04).Physical QOL is significantly impaired in most children pre- and post-VAD. However, psychosocial QOL is not significantly impaired in most children post-VAD suggesting VAD implantation may improve psychosocial QOL in children.

Background:Patients with single ventricle heart disease are living into adulthood due to medical and surgical advancements but have significant physical comorbidities and an increased risk for psychological comorbidities compared to healthy subjects or those with other CHD diagnoses. This study aimed to systematically review psychological functioning in paediatric single ventricle heart disease.Methods:Literature was searched using PubMed, Embase, PsycInfo, CINAHL Complete and Scopus. Peer-reviewed articles that included patients ages 0–25 years with single ventricle heart disease, and quantitative measures of psychological outcomes were included. Meta-analysis using a fixed-effect model was conducted for internalising and externalising t-scores, utilised by the Achenbach Child Behavior Checklist.Results:Twenty-nine records met the criteria for inclusion. 13/24 studies demonstrated increased risk for internalising disorders, such as anxiety/depression; 16/22 studies demonstrated risk for externalising disorders, such as attention or behavioural problems. Meta-analysis of four studies revealed that paediatric single ventricle heart disease patients had no significant difference in internalising and externalising t-scores compared to normative values.Conclusions:The current review demonstrates the need for further studies to better understand psychological functioning in patients with single ventricle heart disease, with a majority of studies showing increased risk for psychological problems despite no difference seen in a small meta-analysis. This summary of the literature underscores the need for regular psychological screening, earlier intervention and integrated mental health therapies in paediatric single ventricle heart disease.

Neonates undergoing cardiac surgery are at risk for oral aversion (OA). OA is not well described outside of the index hospitalization and impacts patients and families. We evaluated the prevalence of OA at 1 year old after neonatal cardiopulmonary bypass (CPB) surgery.Retrospective cohort study.Single quaternary care hospital.Our cohort included 157 neonates who underwent CPB surgery from 2014 to 2017 and had follow-up data available at 1 year old.None.Three feeding experts reviewed the medical record to define children with OA; 30% of charts were evaluated in triplicate for validation. Neonates with and without OA were compared in univariate analysis, and risk factors for OA were explored in a limited multivariable analysis. OA was present at 1 year in 37 patients (23.6%) and other feeding difficulties were present in an additional 29 patients (18.5%). Thirty-eight patients (24.2%) had a feeding tube, including 12 (7.6%) with a gastrostomy tube. Factors associated with OA at 1 year included total ICU days, duration of mechanical ventilation, total number of nil per os days, and number of postoperative days (PODs) until oral feeding initiation (all p0.0001). Number of POD until oral feeding initiation remained independently associated with OA at 1 year in multivariable analysis (adjusted odds ratio, 1.08; 95% CI, 1.04-1.12; p0.0001). Infants with any oral intake at discharge had lower odds of OA at 1 year (0.21; 95% CI, 0.08-0.5; p = 0.0003). At hospital discharge, 132 patients (84.1%) were taking some oral feeds, and 128 patients (81.5%) received tube feeding.OA and other feeding difficulties are common at 1 year old in neonates undergoing CPB surgery. Delayed exposure to oral intake may be a modifiable risk factor for OA and efforts to improve early oral feeding could lead to better functional outcomes.

Although overall outcomes have improved for single-ventricle patients, substantial morbidity and mortality remain for certain high-risk groups. The hybrid stage I procedure is an alternative to the Norwood operation for stage I palliation, but it remains unclear whether it is associated with improved outcomes in high-risk patients.This single-center, nested, case-control study included high-risk patients with a systemic right ventricle who underwent hybrid stage I or Norwood palliation from January 2000 to December 2016. High-risk features included prematurity34 weeks, birth weight2.5 kg, restrictive/intact atrial septum, at least moderate atrioventricular valve regurgitation or right ventricular dysfunction, genetic or extracardiac anomalies, or left ventricular sinusoids. Patients were matched by presence of genetic anomaly, restrictive/intact atrial septum, and prematurity/weight2 kg. Early and midterm outcomes were compared in the matched hybrid vs Norwood groups.The study included 96 patients (35 hybrid, 61 Norwood). Despite improved 30-day survival in hybrid patients (91% vs 66%, P.01), 1-year survival was similar between the hybrid and Norwood groups (46% vs 48%, P = .9). No hybrid patients required dialysis or extracorporeal membrane oxygenation after stage I palliation as compared with 19% and 22% of Norwood patients, respectively (both P.01). Hybrid patients, however, required more unplanned reinterventions (43% vs 21%, P = .02).There remains significant morbidity and mortality among high-risk single-ventricle infants. Despite an early survival benefit, hybrid stage I palliation has not been associated with improved midterm outcomes at our center.

Background Although mortality has decreased considerably in pediatric heart transplantation, waitlist and post-transplant death rates remain notable. End-of-life focused research in this population, however, is very limited. This Pediatric Heart Transplant Society study aimed to describe the circumstances surrounding death of pediatric heart transplant patients. Methods A retrospective analysis of the multi-institutional, international, Pediatric Heart Transplant Society registry was conducted. Descriptive statistics and univariate analyses were performed to 1) describe end-of-life in pediatric pre- and post-heart transplant patients and 2) examine associations between location of death and technological interventions at end-of-life with demographic and disease factors. Results Of 9217 patients (0-18 years) enrolled in the registry between 1993 and 2018, 2804 (30%) deaths occurred; 1310 while awaiting heart transplant and 1494 post-heart transplant. The majority of waitlist deaths (89%) occurred in the hospital, primarily in ICU (74%) with most receiving mechanical ventilation (77%). Fewer post-transplant deaths occurred in the hospital (22%). Out-of-hospital death was associated with older patient age (p Conclusions ICU deaths with high use of technological interventions at end-of-life were common, particularly in patients awaiting heart transplant. In this high mortality population, findings raise challenging considerations for clinicians, families, and policy makers on how to balance quality of life amidst high risk for hospital-based death.

Introduction: Frailty is a clinical syndrome common in adults with chronic disease with resultant vulnerability to adverse health outcomes. There is little known about frailty in pediatric patients, including those with congenital heart disease. We hypothesized that the Fontan-palliated population have a high incidence of frailty. Methods: This single center, prospective cohort study assessed frailty in patients (10-21 years old) after Fontan palliation. The five domains of frailty including slowness, weakness, exhaustion, shrinkage, and diminished physical activity were evaluated using a modified Fried frailty assessment comprised of validated pediatric tests. Providers also estimated their patient’s frailty. Quality of life (QOL) was assessed using PedsQL generic and cardiac modules. Standard descriptive statistics and univariate analyses identified risk factors associated with frailty. Results: Of 54 participants (median age 15.3 years, 61% male), 18 (33%) were identified as frail while 26 (48%) were pre-frail. Patients frequently exhibited frailty in the domains of slowness (93%), weakness (41%), and diminished physical activity (39%). There was poor correlation between frailty scores and provider estimates of frailty (Kappa=0.11). Frail subjects were more likely to have lower PedsQL physical functioning scores (mean 62.8 ± SD 18.5 in Frail vs. 75.7 ± 16.0 in No/pre-Frail; p=0.01). Factors associated with frailty included protein losing enteropathy (p=0.03) and at least one hospitalization in the last year (p=0.047). Higher NYHA functional class (p=0.07) and > 4 clinic visits in the last year (p=0.08) trended toward an association with frailty. Conclusion: Frailty was identified in 1/3 of pediatric patients after Fontan palliation with providers inconsistently recognizing it. Frail patients were more likely to have lower physical functioning, and higher healthcare utilization. These findings highlight the need for improved screening and support for an at-risk population that may not be readily apparent to providers. Additional studies are needed to better understand pediatric frailty, its reversibility, and whether it predicts vulnerability to adverse health outcomes as seen in adults.

Background:Infants who require open heart surgery are at increased risk for developmental delays including gross motor impairments which may have implications for later adaptive skills and cognitive performance. We sought to evaluate the feasibility and efficacy of a tummy time intervention to improve motor skill development in infants after cardiac surgery.Methods:Infants Results:Parents of infants (n = 64) who had cardiac surgery at a median age of 5 days were randomly assigned to tummy time instruction (n = 20), tummy time + outpatient reinforcement (n = 21) or standard of care (n = 23). Forty-nine (77%) returned for follow-up. At follow-up, reported daily tummy time was not significantly different between groups (p = 0.17). Fifteen infants had 15 minutes of tummy time daily had a significantly greater improvement in motor scores than infants with Conclusion:In infants following cardiac surgery

BACKGROUND We aimed to describe the incidence, risk factors, and clinical outcomes of pericardial effusions within 6 months after pediatric heart transplantation (HT). METHODS A single-center retrospective cohort study was performed on all pediatric HT recipients from 2004 to 2018. Logistic regression was used to identify factors associated with pericardial effusions post-HT, and survival was compared using log-rank test. RESULTS During the study period, 97 HTs were performed in 93 patients. Fifty patients (52%) had a ≥small pericardial effusion within 6 months, 16 of which were, or became, ≥moderate in size. Pericardial drain was placed in 8 patients. In univariate analysis, larger recipient body surface area (p = .01) and non-congenital heart disease (p = .002) were associated with pericardial effusion development. Donor/recipient size ratios, post-HT hemodynamics, and rejection did not correlate with pericardial effusion development. In multivariable analysis, non-congenital heart disease (adjusted odds ratio 3.3, p = .01) remained independently associated with development of pericardial effusion. There were no significant differences in post-HT survival between patients with and without ≥small (p = .68) or ≥moderate pericardial effusions (p = .40). CONCLUSIONS Pericardial effusions are common after pediatric HT. Patients with cardiomyopathy, or non-congenital heart disease, were at higher risk for post-HT pericardial effusions. Pericardial effusions increased morbidity but had no effect on mortality in our cohort. The risk factors identified may be used for anticipatory guidance in pediatric HT.

In single-ventricle patients with native-to-neoaortic anastomoses, the native aortic root serves as a conduit to the coronary arteries. Thrombosis of the native aortic root has been described only in small, limited reports. We described our center's experience with this rare adverse event. All single-ventricle patients who underwent native-to-neoaortic anastomosis from 2002 to 2017 were compiled from institutional databases. Chart review identified cases of native aortic root thrombosis. Of 467 patients, there were 9 (2%) cases of native aortic root thrombosis; all had hypoplastic left heart syndrome. Timing of thrombosis varied and occurred following each stage of single-ventricle palliation. For treatment, 8 patients received systemic anticoagulation and one patient did not receive any treatment. One patient also underwent percutaneous thrombectomy. Three patients (33.3%) died during the follow-up period. Among survivors, 4/6 had right ventricular systolic function that was mildly depressed or worse at a median follow-up of 8.3 years (interquartile range 1.3-10.8). Native aortic root thrombosis is a rare complication occurring at various stages of single-ventricle palliation. In this series, 6 of 9 patients (66.6%) survived; however, ventricular function was often compromised. No risk factors were identified to focus preventative therapies.

Objective To determine the impact of damaging genetic variation in proangiogenic pathways on placental function, complications of pregnancy, fetal growth, and clinical outcomes in pregnancies with fetal congenital heart defect. Study design Families delivering a baby with a congenital heart defect requiring surgical repair in infancy were recruited. The placenta and neonate were weighed and measured. Hemodynamic variables were recorded from a third trimester (36.4 ± 1.7 weeks) fetal echocardiogram. Exome sequencing was performed on the probands (N = 133) and consented parents (114 parent-child trios, and 15 parent-child duos) and the GeneVetter analysis tool used to identify damaging coding sequence variants in 163 genes associated with the positive regulation of angiogenesis (PRA) (GO:0045766). Results In total, 117 damaging variants were identified in PRA genes in 133 congenital heart defect probands with 73 subjects having at least 1 variant. Presence of a damaging PRA variant was associated with increased umbilical artery pulsatility index (mean 1.11 with variant vs 1.00 without; P = .01). The presence of a damaging PRA variant was also associated with lower neonatal length and head circumference for age z score at birth (mean −0.44 and −0.47 with variant vs 0.23 and −0.05 without; P = .01 and .04, respectively). During median 3.1 years (IQR 2.0-4.1 years) of follow-up, deaths occurred in 2 of 60 (3.3%) subjects with no PRA variant and in 9 of 73 (12.3%) subjects with 1 or more PRA variants (P = .06). Conclusions Damaging variants in proangiogenic genes may impact placental function and are associated with impaired fetal growth in pregnancies involving a fetus with congenital heart defect.

We sought to assess acute hemodynamic changes after implementation of negative extrathoracic pressure (NEP) in spontaneously breathing ambulatory Fontan patients with symptomatic heart failure. We hypothesized that application of NEP would result in an acute decrease in pulmonary artery pressure. Ten patients with clinical evidence of Fontan failure underwent baseline hemodynamic catheterization while breathing spontaneously. Hemodynamic measurements were then repeated after 30 min of continuous NEP. After 30 min of continuous NEP, 4/10 patients had a decrease in their Fontan pressure by 2 mmHg and one patient had a decrease by 1 mmHg. There were three patients that had an increase in Fontan pressure by 2 mmHg. In 7/10 patients, indexed pulmonary vascular resistance decreased by an average of 31%. In symptomatic Fontan patients with a favorable hemodynamic response to NEP during catheterization, potential benefit of longer-term NEP to improve clinical status should be explored.

Elevated left ventricular end diastolic pressure is a risk factor for ventricular arrhythmias in patients with tetralogy of Fallot. The objective of this retrospective study was to identify echocardiographic measures associated with left ventricular end diastolic pressure >12 mmHg in this population. Repaired tetralogy of Fallot patients age ≥13 years, who underwent a left heart catheterisation within 7 days of having an echocardiogram were evaluated. Univariate comparison was made in echocardiographic and clinical variables between patients with left ventricular end diastolic pressure >12 versus ≤12 mmHg. Ninety-four patients (54% male) with a median age of 24.6 years were included. Thirty-four (36%) had left ventricular end diastolic pressure >12 mmHg. Patients with left ventricular end diastolic pressure >12mmHg were older (median 32.9 versus 24.0 years, p = 0.02), more likely to have a history of an aortopulmonary shunt (62% versus 38%, p = 0.03), and have a diagnosis of hypertension (24% versus 7%, p = 0.03) compared to those with left ventricular end diastolic pressure ≤12 mmHg. There were no significant differences in mitral valve E/A ratio, annular e’ velocity, or E/e’ ratio between patients with left ventricular end diastolic pressure >12 versus ≤12 mmHg. Patients with left ventricular end diastolic pressure >12mmHg had larger left atrial area (mean 17.7 versus 14.0 cm2, p = 0.03) and larger left atrium anterior–posterior diameter (mean 36.0 versus 30.6 mm, p = 0.004). In conclusion, typical echocardiographic measures of left ventricular diastolic dysfunction may not be reliable in tetralogy of Fallot patients. Prospective studies with the use of novel echocardiographic measures are needed.

BACKGROUND The influence of Fontan-associated protein-losing enteropathy's (PLE) severity, duration, and treatment on heart transplant (HTx) outcomes is unknown. We hypothesized that long-standing PLE and PLE requiring more intensive therapy are associated with increased post-HTx mortality. METHODS This 12-center, retrospective cohort study of post-Fontan patients with PLE referred for HTx from 2003 to 2015 involved collection of demographic, medical, surgical, and catheterization data, as well as PLE-specific data, including duration of disease, intensity/details of treatment, hospitalizations, and complications. Factors associated with waitlist and post-HTx outcomes and PLE resolution were sought. RESULTS Eighty patients (median of 5 per center) were referred for HTx evaluation. Of 68 patients listed for HTx, 8 were removed due to deterioration, 4 died waiting, and 4 remain listed. In 52 patients undergoing HTx, post-HTx 1-month survival was 92% and 1-year survival was 83%. PLE-specific factors, including duration of PLE pre-HTx, pre-HTx hospitalizations, need for/frequency of albumin replacement, PLE therapies, and growth parameters had no association with post-HTx mortality. Immunosuppressant regimen was associated with mortality; standard mycophenolate mofetil immunotherapy was used in 95% of survivors compared with only 44% of non-survivors (p = 0.03). Rejection (53%) and infection (42%) post-HTx were common, but not associated with PLE-specific factors. PLE resolved completely in all but 1 HTx survivor at a median of 1 month (interquartile range 1 to 3 months); resolution was not affected by PLE-specific factors. CONCLUSIONS PLE severity, duration, and treatment do not influence post-HTx outcome, but immunosuppressive regimen may have an impact on survival. PLE resolves in nearly all survivors.

Objectives:Assess the acute and short-term haemodynamic impact of transcatheter pulmonary valve implantation on left ventricular systolic and diastolic function stratified by pre-transcatheter pulmonary valve implantation physiology.Background:Transcatheter pulmonary valve implantation is a widely available option to treat residual or recurrent pulmonary stenosis and pulmonary insufficiency. Transcatheter pulmonary valve implantation acutely increases pulmonary artery size and diastolic pressure in patients with pulmonary insufficiency and acute pulmonary edema has been reported after transcatheter pulmonary valve implantation, possibly related to acute left ventricular volume loading. However, the impact of transcatheter pulmonary valve implantation on left ventricular diastolic function has not been established.Methods:Patients who underwent transcatheter pulmonary valve implantation from 2010 to 2017 at our centre were grouped by indication for transcatheter pulmonary valve implantation as pulmonary stenosis, pulmonary insufficiency, or mixed disease. Separate analysis was performed on those who underwent transcatheter pulmonary valve implantation for pulmonary stenosis versus pulmonary insufficiency or mixed disease. Intracardiac haemodynamics immediately before and after transcatheter pulmonary valve implantation and echocardiographic assessment of left ventricular systolic and diastolic function at baseline, 1-day post transcatheter pulmonary valve implantation, and 1-year post transcatheter pulmonary valve implantation were compared between groups.Results:In 102 patients who underwent transcatheter pulmonary valve implantation, the indication was pulmonary stenosis in 29 (28%), pulmonary insufficiency in 28 (29%), and mixed disease in 44 (43%). There were no significant differences in left ventricular systolic or diastolic function between groups at baseline, immediately after transcatheter pulmonary valve implantation, or 1-year post implantation. The mean pulmonary artery wedge pressure increased equally across groups.Conclusions:While patients with pulmonary insufficiency likely have acute left ventricular volume loading following transcatheter pulmonary valve implantation, this does not appear to be haemodynamically significant as transcatheter pulmonary valve implantation was not associated with measurable changes in left ventricular systolic or diastolic function acutely or 1-year post implantation.

Background/Purpose: There is conflicting data on how geographic distance from delivery location to the cardiac surgical center impacts outcomes in neonates with ductal-dependent heart disease. Our goal was to describe national current practices in delivery location of neonates with a prenatal diagnosis of hypoplastic left heart syndrome (HLHS) and evaluate the impact of delivery location and age at admission to the surgical center ICU on post-operative length of hospital stay and transplant-free survival after the Norwood operation. Methods: A multicenter cohort study was performed utilizing the National Pediatric Cardiology Quality Improvement …

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: 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: Transcatheter pulmonary valve implantation (TPVi) is a widely available option to treat right ventricular outflow tract (RVOT) conduits, but coronary artery compression is an absolute contraindication. Cardiac magnetic resonance (CMR) may evaluate coronary anatomy, but its utility in predicting coronary compression is not well established. Hypothesis: CMR prior to TPVi can accurately predict coronary compression risk. Methods: We analyzed all patients with a recent CMR (≤ 12 months) and attempted TPVi in an RVOT conduit at 9 centers from 2007-2016. A core lab reviewed all CMRs for the shortest orthogonal distance from a coronary artery to the conduit, the shortest distance from a coronary artery to the most stenotic area of the conduit, and subjective assessment of coronary compression risk. Receiver operating characteristic curve was used to determine optimal predictive distances. Univariate and independent associations of the distances and qualitative assessment with coronary compression were examined using logistic regression. Results: Of 231 patients (62% male, median age 19.0 years), TPVi was successful in 198 (86%); in 24 (10%) balloon testing (documented coronary compression or high risk) precluded implantation. Distance to the RV to PA conduit ≤ 2.1 mm (area under the curve [AUC] 0.70) and distance to most stenotic area ≤ 13.1 mm (AUC 0.69) predicted coronary compression (Table). Subjective assessment had the highest AUC (0.78), with 96% negative predictive value. Both distances and qualitative assessment remained independently associated with coronary compression when controlling for abnormal coronary anatomy. Conclusions: CMR can predict the risk of coronary compression during TPVi in RVOT conduits but cannot completely exclude the risk of coronary compression. CMR may assist in patient selection and counselling families prior to TPVi, although balloon testing prior to TPVi remains essential.

Introduction: The Institute of Medicine called for scientific investigation and the development of guidelines to improve end of life care for pediatric patients with serious illnesses. Despite high morbidity and mortality in pediatric heart transplantation (HTx), research on the end of life care needs of this population is extremely limited. Aims: This study aimed to describe the circumstances surrounding death of pediatric HTx patients and examine associations between location of death and technological interventions at end-of-life with demographic, disease, and HTx-related factors. Methods: This retrospective analysis of the Pediatric Heart Transplant Society registry utilized descriptive statistics and standard univariate analysis to examine associations between location of death and patient factors. Results: Of 9,217 registry entries, 2,804 (30%) deaths occurred; 1,310 while awaiting HTx; 1,494 post-HTx. Location of death was only recorded for 1,113 patients which included 804 waitlist deaths; 89% occurred in the hospital, primarily in ICU settings (74%) with most requiring mechanical ventilation (77%). A subset (39%) were supported by ECMO/VAD at time of death with 69% receiving inotrope support. Location of death was captured for 309 post-HTx patients with only 22% occurring in hospital; primarily in the ICU (74%) with half receiving mechanical ventilation (52%) and a smaller proportion supported by ECMO/VAD (18%) or inotropes (21%) at time of death. Overall, location of death was not associated with patient sex, race, ethnicity, insurance type, or primary etiology. Out of hospital death was associated with older patient age (p Conclusions: Death occurred in ~1/3 of patients captured in this pediatric HTx registry. ICU deaths with high use of technological interventions were common, particularly in waitlisted patients, but family decision to discontinue interventions was infrequently cited. Findings underscore the need for palliative care interventions and improved research strategies to better understand end of life in pre- and post-HTx pediatric populations.

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: 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.