Physical activity in the Fontan population: provider recommendations and patient actions.

BACKGROUND: Emerging evidence suggests that routine physical activity may improve exercise capacity, long-term outcomes, and quality of life in individuals with Fontan circulation. Despite this, it is unclear how active these individuals are and what guidance they receive from medical providers regarding physical activity. The aim of this study was to survey Fontan patients on personal physical activity behaviours and their cardiologist-directed physical activity recommendations to set a baseline for future targeted efforts to improve this. METHODS: An electronic survey assessing physical activity habits and cardiologist-directed guidance was developed in concert with content experts and patients/parents and shared via a social media campaign with Fontan patients and their families. RESULTS: A total of 168 individuals completed the survey. The median age of respondents was 10 years, 51% identifying as male. Overall, 21% of respondents spend > 5 hours per week engaged in low-exertion activity and only 7% spend > 5 hours per week engaged in high-exertion activity. In all domains questioned, pre-adolescents reported higher participation rates than adolescents. Nearly half (43%) of respondents reported that they do not discuss activity recommendations with their cardiologist. CONCLUSIONS: Despite increasing evidence over the last two decades demonstrating the benefit of exercise for individuals living with Fontan circulation, only a minority of patients report engaging in significant amounts of physical activity or discussing activity goals with their cardiologist. Specific, individualized, and actionable education needs to be provided to patients, families, and providers to promote and support regular physical activity in this patient population.

Identifying Frailty and Its Risk Factors in Pediatric Patients with Fontan Physiology.

Frailty is a clinical syndrome common in adults with chronic disease with resultant vulnerability to adverse health outcomes. Little is known about frailty in pediatric patients, including those with single-ventricle heart disease. This study aimed to examine the prevalence of frailty and its associated risk factors in patients with Fontan circulation. A single-center, prospective cohort study assessed frailty in patients (10-21 years old) after Fontan palliation. Slowness, weakness, exhaustion, shrinkage, and diminished physical activity were evaluated and scored using a modified Fried frailty assessment comprised of validated pediatric tests. Providers estimated subjects' degree of frailty. Patient-reported quality of life (QOL) was assessed. 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 had 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). One-third of pediatric patients after Fontan palliation were frail which was associated with lower physical functioning and higher healthcare utilization. Providers poorly recognized frailty. These findings highlight the need for improved screening and support for an at-risk population where frailty is not easily identified.

Insulin Resistance after Fontan Palliation.

Patients with a single ventricle heart who had Fontan palliation (S/P Fontan) are at increased risk for acquired morbidity. Insulin resistance (IR) is a predictor of cardiac morbidity and mortality. A single-center, cross-sectional study using S/P Fontan and controls was designed to assess IR S/P Fontan. Group comparisons were made in IR via the Quantitative Insulin Index (QUICKI) and the natural log-transformed homeostasis model assessment, ln (HOMA-IR), without/with adjusting for age. A total of 89 patients (59 Fontan and 30 controls) were included. Fontan patients showed a significant decrease in QUICKI (0.34 ± 0.03 vs 0.37 ± 0.02) and an elevation of ln (HOMA-IR) (0.82 ± 0.62 vs 0.24 ± 0.44) compared to controls (both p < 0.0001); this remained significant even adjusting for age. With older age, there was a significant, progressive decrease in QUICKI (p = 0.01) and an increase in ln (HOMA-IR) (p = 0.02) S/P Fontan. Analysis excluding Fontan patients with obesity still showed a significant reduction of QUICKI and an elevation of ln (HOMA-IR) in Fontan patients compared to controls when adjusting for age (both p < 0.05). Using QUICKI, IR was present in 41 (69.5%) Fontan patients vs. 3 (10%) controls (p < 0.0001) and using HOMA-IR, IR was present in 32 (54.2%) vs 5 (16.7%) controls (p = 0.001). Fontan patients had significantly more IR compared to controls and the prevalence of IR increases with age. Since IR is known to correlate with long-term morbidity and mortality and can be ameliorated by therapies, we believe it is critical that IR be identified as early as possible in Fontan patients.

Validity of Wrist-Worn Activity Tracker Heart Rate Detection in Fontan Patients During Exercise.

PURPOSE: Physical activity and a healthy lifestyle play an essential role in optimizing long-term health in patients with Fontan physiology. Wrist-worn activity trackers may be useful in medically directed exercise programs for patients with Fontan physiology. The objective of this study was to measure the validity of Garmin and Fitbit activity tracker heart rate detection in patients with Fontan circulation when compared to electrocardiogram (ECG) during cardiopulmonary exercise testing (CPET). METHODS: 47 Fontan patients undergoing CPET for clinical indications were included and wore activity trackers during CPET. Heart rate via the activity tracker was collected at baseline, maximal exercise, and recovery. Patient heart rates, peak VO2, and peak respiratory exchange ratio (RER) were collected using standard CPET protocols and equipment. Heart rate at each time point was compared between the activity trackers and CPET ECG. RESULTS: Median age of participants was 17.1 years, 15.1 years since Fontan completion. Mean percent of predicted peak VO2 was 56.8%, z-score -3.2 with 61.7% of participants completing a maximal CPET (RER ≥ 1.09). Baseline oxygen saturation mean was 92.9%, 90.0% at maximal exercise. Activity trackers demonstrated mean absolute percentage error < 10% at most time points, comparable with other studies. Demographics, Fontan-associated comorbidities, and echocardiogram findings did not impact the accuracy. CONCLUSIONS: Consumer-oriented wrist-worn activity trackers show promising accuracy for heart rate monitoring in medically directed exercise programs for adolescents and young adults with Fontan physiology. Further validation across different exercise modalities is needed.

Use of Hybrid Stage I to Stratify Between Single Ventricle Palliation and Biventricular Repair.

Background: Hybrid stage I palliation (HS1P) has been utilized for patients with single ventricle (SV) congenital heart disease (CHD). To date, reports on the use of HS1P for other indications including biventricular (BiV) CHD have been limited. Methods: We performed a single-center retrospective cohort study of patients who underwent HS1P with an anticipated physiologic outcome of BiV repair, or with an undetermined SV versus BiV outcome. Patient characteristics and outcomes from birth through definitive repair or palliation were collected and reported with descriptive statistics. Results: Nineteen patients underwent HS1P with anticipated BiV repair. Extracardiac and intracardiac risk factors (ICRF) were common. Ultimately, 13 (68%) patients underwent BiV repair, 1 (5%) underwent SV palliation, and 5 (26%) died prior to further palliation or repair. Resolution of ICRF tracked with BiV outcome (6/6, 100%), persistence of ICRF tracked with SV outcome or death (3/3, 100%). Twenty patients underwent HS1P with an undetermined outcome. Ultimately, 13 (65%) underwent BiV repair, 6 (30%) underwent SV palliation, and 1 (5%) underwent transplant. There were no deaths. Intracardiac risk factors were present in 15 of 20 patients (75%); BiV repair only occurred when all ICRF resolved (67%). Post-HS1P complications and reinterventions occurred frequently in both groups, through all phases of care. Conclusions: Hybrid stage 1 palliation can be used to defer BiV repair and to delay decision between SV palliation and BiV repair. Resolution of ICRF was associated with ultimate outcome. In this high-risk group, complications are common, and mortality especially in the marginal BiV patient is high.

Atrial septal interventions during and after hybrid stage I palliation of hypoplastic left heart syndrome.

BACKGROUND: Hybrid stage I palliation (HS1P) is an alternative approach for initial palliation in hypoplastic left heart syndrome (HLHS) patients. Unlike surgical stage I palliation where atrial septectomy is routinely performed, atrial septal intervention (ASI) during HS1P is variable. In this study, we described our experience with ASI in single ventricle (SV) patients who underwent HS1P and identified factors associated with need for ASI after HS1P. METHODS: Data were retrospectively collected for all HLHS patients who underwent HS1P at our center over the past 12 years. We evaluated ASIs performed during the HS1P (intra-HS1P ASI) and ASIs performed during the period from HS1P to the subsequent surgical stage, either interval Norwood stage I or comprehensive stage II (post-HS1P ASI). Patient factors and procedural data were compared to identify factors associated with undergoing post-HS1P ASI and the impact of ASI on patient outcomes was evaluated. RESULTS: Of 50 SV patients included, 23 (46%) underwent intra-HS1P ASI and 26 (52%) underwent post-HS1P ASI. Need for post-HS1P ASI was lower among patients who had an intra-HS1P ASI as compared to those who did not (30% vs. 70%; p = 0.005). There were no significant differences in short or Midterm outcomes between patients who underwent intra-HS1P ASI or post-HS1P ASI and their counterparts. CONCLUSIONS: ASI is common both during and after HS1P but is generally well tolerated and type of ASI does not significantly impact overall patient outcomes. Our findings suggest that the current approach of individualizing management of ASI in the HS1P population is effective and safe.

The Incidence and Relevance of Hematochezia in the Interstage Congenital Heart Population.

Necrotizing enterocolitis (NEC) increases morbidity and mortality for infants with single ventricle heart disease (SVHD). While hematochezia often proceeds NEC not all hematochezia progresses to NEC. We aimed to examine the incidence, risk-factors, and outcomes associated with hematochezia and NEC for infants with SVHD. A single-center cohort study including SVHD patients requiring Stage I palliation from 12/2010 to 12/2015 was performed. Demographic, clinical, and outcome measures during the interstage period were abstracted from medical records. We defined hematochezia as blood in the stool without alternative etiology and NEC as systemic or intestinal signs concurrent with hematochezia and/or the presence of radiographic pneumatosis. Clinical characteristics and outcome measures were compared between patients with/without hematochezia and with/without NEC. Of 135 patients, 59(44%) had hematochezia and 20(15%) developed NEC. Demographic and operative factors were similar between patients with and without hematochezia. Patients with NEC were more often premature (15% vs 0%, p = 0.04), have lower birth weight (3.0 ± 0.6 vs 3.3 ± 0.5 kg, p = 0.03), longer cardiopulmonary bypass time (median 131 vs. 90 min, p = 0.02) and more often underwent unplanned cardiac catheterization (20% vs 3%, p = 0.04). Patients with hematochezia had more line days (p < 0.0001) and longer post-Stage-I length of stay (p < 0.0001) than those without hematochezia, and those with NEC had more line days than those without NEC (p = 0.02). Hematochezia is frequent following Stage-I palliation, however only one third of these patients develop NEC. Non-NEC Hematochezia is associated with a similar increase in line and hospital days. Further research is needed to identify methods to avoid over treatment.

Morphology of the Second Mesiobuccal Canal in the Maxillary Second Molar / Morfología del Segundo Canal Mesiovestibular en el Segundo Molar Maxilar

SUMMARY: Failure to locate a complete canal system affects the prognosis of root canal treatment. A missed root canal is one of the most common reasons for failed root canal treatment. The prevalence of the second mesiobuccal canal in the maxillary second molar is relatively high and has a variety of configurations. Therefore, knowledge of its morphology is required in clinical endodontics. This review presented the canal in terms of its prevalence, classification, anatomical features, and the method for locating the second mesiobuccal canal in the maxillary second molar. Root canal treatment requires knowledge of tooth morphology, appropriate access preparation, and a thorough examination of the tooth's interior. Thus, clinicians should carefully employ various methods for assessing the anatomy of the entire root canal system to prevent failure in locating the second mesiobuccal canal. This canal can be located by modifying the access cavity design and utilizing specific instruments to improve the second mesiobuccal canal system visualization.

La falta de localización de un sistema completo de canal afecta el pronóstico del tratamiento de éste. La omisión de un tratamiento de canal es uno de los motivos más frecuentes por las que el tratamiento de canal fracasa. La prevalencia del segundo canal mesiovestibular en el segundo molar superior es relativamente alta y tiene una variedad de configuraciones. Por tanto, el conocimiento de su morfología es necesario en endodoncia clínica. Esta revisión presentó el canal en términos de su prevalencia, clasificación, características anatómicas y el método para localizar el segundo canal mesiovestibular en el segundo molar superior. El tratamiento de canal requiere conocimiento de la morfología del diente, una preparación adecuada del acceso y un examen exhaustivo del interior del diente. Por lo tanto, los dentistas deben emplear cuidadosamente varios métodos para evaluar la anatomía de todo el sistema de canales radiculares para evitar fallas en la localización del segundo canal mesiovestibular. Este canal se puede localizar modificando el diseño de la cavidad de acceso y utilizando instrumentos específicos para mejorar la visualización del sistema del segundo canal mesiovestibular.

Hypoplastic Left Heart Syndrome with Mitral Stenosis and Aortic Atresia-Echocardiographic Findings and Early Outcomes.

BACKGROUND: Mitral stenosis/aortic atresia (MS/AA) has been reported as a high-risk variant of hypoplastic left heart syndrome (HLHS), potentially related to ventriculocoronary connections (VCCs) or endocardial fibroelastosis (EFE) and myocardial hypoperfusion. We aimed to identify echocardiographic and clinical factors associated with early death or transplant in this group. METHODS: Patients with HLHS MS/AA treated at our center between 2000 and 2020 were included. Pre-stage I palliation echocardiograms were reviewed. Certain imaging factors, such as determination of VCC, EFE, and measurement of tricuspid annular plane systolic excursion were measured from retrospective review of preoperative images; others were derived from clinical reports. Groups were compared according to primary outcome of death or transplant prior to stage II palliation. RESULTS: Of 141 patients included, 39 (27.7%) experienced a primary outcome. Ventriculocoronary connections were identified in 103 (73.0%) patients and EFE in 95 (67.4%) patients. Among imaging variables, smaller ascending aorta size (median, 2.2 [interquartile range (IQR) 1.7-2.8] vs 2.6 [2.2-3.4] mm, P = .01) was associated with primary outcome. There was similar frequency of VCC (74.4% vs 72.5%, P = .83), EFE (59.0% vs 72.5%, P = .19), moderate or greater tricuspid regurgitation (5.1% vs 5.9%, P = 1.00), and similar right ventricular systolic function (indexed tricuspid annular plane systolic excursion 32.5 ± 7.3 vs 31.4 ± 7.2 mm/m2, P = .47) in the primary outcome group compared to other patients. Clinical factors associated with primary outcome included lower birth weight (mean, 2.8 ± SD 0.8 vs 3.3 ± 0.5 kg, P = .0003), gestational age <37 weeks (31.6% vs 4.9%, P < .0001), longer cardiopulmonary bypass time (median, 112 [IQR, 93-162] vs 82 [71-119] minutes, P = .001), longer intensive care unit length of stay (median, 19 [IQR, 10-30] vs 10 [7-15] days, P = .001), and extracorporeal membrane oxygenation following stage I palliation (43.6% vs 8.8%, P < .0001). Presence of VCCs and EFE was not associated with death or transplant after controlling for birth weight and era of stage I palliation. CONCLUSIONS: In one of the largest reported single-center cohorts of HLHS MS/AA, there were few pre-stage I palliation imaging characteristics associated with primary outcome. Imaging findings evaluated in this study, including the presence of VCC and/or EFE as determined using highly sensitive echocardiogram criteria, should not preclude intervention, although impact on long-term outcomes requires further evaluation.

Consensus-Based Development of a Pediatric Echocardiography Complexity Score: Design, Rationale, and Results of a Quality Improvement Collaborative.

BACKGROUND: The complexity of congenital heart disease has been primarily stratified on the basis of surgical technical difficulty, specific diagnoses, and associated outcomes. We report on the refinement and validation of a pediatric echocardiography complexity (PEC) score. METHODS AND RESULTS: The American College of Cardiology Quality Network assembled a panel from 12 centers to refine a previously published PEC score developed in a single institution. The panel refined complexity categories and included study modifiers to account for complexity related to performance of the echocardiogram. Each center submitted data using the PEC scoring tool on 15 consecutive inpatient and outpatient echocardiograms. Univariate and multivariate analyses were performed to assess for independent predictors of longer study duration. Among the 174 echocardiograms analyzed, 68.9% had underlying congenital heart disease; 44.8% were outpatient; 34.5% were performed in an intensive care setting; 61.5% were follow-up; 46.6% were initial or preoperative; and 9.8% were sedated. All studies had an assigned PEC score. In univariate analysis, longer study duration was associated with several patient and study variables (age <2 years, PEC 4 or 5, initial study, preoperative study, junior or trainee scanner, and need for additional imaging). In multivariable analysis, a higher PEC score of 4 or 5 was independently associated with longer study duration after controlling for study variables and center variation. CONCLUSIONS: The PEC scoring tool is feasible and applicable in a variety of clinical settings and can be used for correlation with diagnostic errors, allocation of resources, and assessment of physician and sonographer effort in performing, interpreting, and training in pediatric echocardiography.

Reducing Hyperoxia Exposure in Infants Requiring Veno-Arterial Extracorporeal Membrane Oxygenation after Cardiac Surgery.

Recent studies have suggested worse outcomes in patients exposed to hyperoxia while supported on veno-arterial extracorporeal membrane oxygenation (VA-ECMO). However, there are no data regarding the effect of reducing hyperoxia exposure in this population by adjusting the fraction of inspired oxygen (FiO2) of the sweep gas of the ECMO circuit. A retrospective review of 143 patients less than 1 year of age requiring VA-ECMO following cardiac surgery from 2007 to 2018 was completed. 64 patients had a FiO2 of the sweep gas < 100% with an average PaO2 of 210 mm Hg in the first 48 h of support [vs 405 mm Hg in the group with a FiO2 = 100% (p < 0.0001)]. There was no difference in mortality at 30 days after surgery or other markers of end-organ injury with respect to whether the FiO2 was adjusted. At least one PaO2 value < 200 mm Hg in the first 24 h on ECMO in patients with a FiO2 < 100% trended toward a significant association (OR = 0.45, 95% CI = 0.21-1.01) with decreased risk of 30-day mortality when compared to those patients with a FiO2 = 100% and all PaO2 values > 200 mm Hg. Only 47% of patients with a FiO2 < 100% had an average PaO2 less than 200 mm Hg which indicates that the intervention of reducing the FiO2 of the sweep gas was not entirely effective at reducing hyperoxia exposure. Future research is needed for developing clinical protocols to avoid hyperoxia and to identify mechanisms for hyperoxia-induced injury on VA-ECMO.

Experience with SGLT2 Inhibitors in Patients with Single Ventricle Congenital Heart Disease and Fontan Circulatory Failure.

Heart failure is the leading cause of morbidity and mortality in patients with Fontan circulation. Sodium-glucose-cotransporter 2 inhibitors (SGLT2i) have become a mainstay of heart failure therapy in adult patients, however, there remains a paucity of literature to describe its use in pediatric heart failure patients, especially those with single ventricle physiology. We describe our early experience using SGLT2i in patients with single ventricle congenital heart disease surgically palliated to the Fontan circulation. We conducted a single-center retrospective chart review of all patients with Fontan circulation who were initiated on an SGLT2i from January 1, 2022 to March 1, 2023. Patient demographics, diagnoses, clinical status, and other therapies were collected from the electronic medical record. During the study period, 14 patients (median age 14.5 years, range 2.0-26.4 years) with Fontan circulation were started on a SGLT2i. Mean weight was 54 kg (range 11.6-80.4 kg). Median follow-up since SGLT2i initiation was 4.1 months (range 13 days-7.7 months). Four patients had a systemic left ventricle and 10 had a systemic right ventricle. Half the patients had Fontan Circulatory Failure with reduced Ejection Fraction (FCFrEF) of the systemic ventricle and the other half had Fontan Circulatory Failure with preserved Ejection Fraction (FCFpEF) of the systemic ventricle. In addition, 3 patients experienced Protein Losing Enteropathy (PLE) and 2 patients had plastic bronchitis, one of whom also was diagnosed with chylothorax. There were no genitourinary infections, hypoglycemia, ketoacidosis, hypotension or other significant adverse effects noted in our patient population. One patient experienced significant diuresis and transient acute kidney injury. Patients with FCFrEF showed a decrease in natriuretic peptide levels. Given the lack of proven therapies, demonstrated benefits of SGLT2i in other populations, and some suggestion of efficacy in Fontan circulation, further study of SGTLT2i in patients with Fontan circulation is warranted.

Neurocognitive function and health-related quality of life in adolescents and young adults with CHD with pulmonary valve dysfunction.

BACKGROUND: Neurocognitive impairment and quality of life are two important long-term challenges for patients with complex CHD. The impact of re-interventions during adolescence and young adulthood on neurocognition and quality of life is not well understood. METHODS: In this prospective longitudinal multi-institutional study, patients 13-30 years old with severe CHD referred for surgical or transcatheter pulmonary valve replacement were enrolled. Clinical characteristics were collected, and executive function and quality of life were assessed prior to the planned pulmonary re-intervention. These results were compared to normative data and were compared between treatment strategies. RESULTS: Among 68 patients enrolled from 2016 to 2020, a nearly equal proportion were referred for surgical and transcatheter pulmonary valve replacement (53% versus 47%). Tetralogy of Fallot was the most common diagnosis (59%) and pulmonary re-intervention indications included stenosis (25%), insufficiency (40%), and mixed disease (35%). There were no substantial differences between patients referred for surgical and transcatheter therapy. Executive functioning deficits were evident in 19-31% of patients and quality of life was universally lower compared to normative sample data. However, measures of executive function and quality of life did not differ between the surgical and transcatheter patients. CONCLUSION: In this patient group, impairments in neurocognitive function and quality of life are common and can be significant. Given similar baseline characteristics, comparing changes in neurocognitive outcomes and quality of life after surgical versus transcatheter pulmonary valve replacement will offer unique insights into how treatment approaches impact these important long-term patient outcomes.

Augmented Reality Visualization of 3D Rotational Angiography in Congenital Heart Disease: A Comparative Study to Standard Computer Visualization.

Augmented reality (AR) visualization of 3D rotational angiography (3DRA) provides 3D representations of cardiac structures with full visualization of the procedural environment. The purpose of this study was to evaluate the feasibility of converting 3DRAs of congenital heart disease patients to AR models, highlight the workflow for 3DRA optimization for AR visualization, and assess physicians' perceptions of their use. This single-center study prospectively evaluated 30 retrospectively-acquired 3DRAs that were converted to AR, compared to Computer Models (CM). Median patient age 6.5 years (0.24-38.8) and weight 20.6 kg (3.4-107.0). AR and CM quality were graded highly. RV pacing was associated with higher quality of both model types (p = 0.02). Visualization and identification of structures were graded as "very easy" in 81.1% (n = 73) and 67.8% (n = 61) of AR and CM, respectively. Fifty-nine (66%) grades 'Agreed' or 'Strongly Agreed' that AR models provided superior appreciation of 3D relationships; AR was found to be least beneficial in visualization of aortic arch obstruction. AR models were thought to be helpful in identifying pathology and assisting in interventional planning in 85 assessments (94.4%). There was significant potential seen in the opportunity for patient/family counseling and trainee/staff education with AR models. It is feasible to convert 3D models of 3DRAs into AR models, which are of similar image quality as compared to CM. AR models provided additional benefits to visualization of 3D relationships in most anatomies. Future directions include integration of interventional simulation, peri-procedural counseling of patients and families, and education of trainees and staff with AR models.

Failed Extubation in Neonates After Cardiac Surgery: A Single-Center, Retrospective Study.

OBJECTIVES: To describe factors associated with failed extubation (FE) in neonates following cardiovascular surgery, and the relationship with clinical outcomes. DESIGN: Retrospective cohort study. SETTING: Twenty-bed pediatric cardiac ICU (PCICU) in an academic tertiary care children's hospital. PATIENTS: Neonates admitted to the PCICU following cardiac surgery between July 2015 and June 2018. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Patients who experienced FE were compared with patients who were successfully extubated. Variables associated with FE ( p < 0.05) from univariate analysis were considered for inclusion in multivariable logistic regression. Univariate associations of FE with clinical outcomes were also examined. Of 240 patients, 40 (17%) experienced FE. Univariate analyses revealed associations of FE with upper airway (UA) abnormality (25% vs 8%, p = 0.003) and delayed sternal closure (50% vs 24%, p = 0.001). There were weaker associations of FE with hypoplastic left heart syndrome (25% vs 13%, p = 0.04), postoperative ventilation greater than 7 days (33% vs 15%, p = 0.01), Society of Thoracic Surgeons-European Association for Cardio-Thoracic Surgery (STAT) category 5 operations (38% vs 21%, p = 0.02), and respiratory rate during spontaneous breathing trial (median 42 vs 37 breaths/min, p = 0.01). In multivariable analysis, UA abnormalities (adjusted odds ratio [AOR] 3.5; 95% CI, 1.4-9.0), postoperative ventilation greater than 7 days (AOR 2.3; 95% CI, 1.0-5.2), and STAT category 5 operations (AOR 2.4; 95% CI, 1.1-5.2) were independently associated with FE. FE was also associated with unplanned reoperation/reintervention during hospital course (38% vs 22%, p = 0.04), longer hospitalization (median 29 vs 16.5 d, p < 0.0001), and in-hospital mortality (13% vs 3%, p = 0.02). CONCLUSIONS: FE in neonates occurs relatively commonly following cardiac surgery and is associated with adverse clinical outcomes. Additional data are needed to further optimize periextubation decision-making in patients with multiple clinical factors associated with FE.

Medical and End-of-Life Decision-Making Preferences in Adolescents and Young Adults with Advanced Heart Disease and Their Parents.

Importance: Despite 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. Objective: To characterize decision-making preferences of AYAs with advanced heart disease and their parents and determine factors associated with these preferences. Design, Setting, and Participants: Cross-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 Measures: Single-item measure of medical decision-making preferences, MyCHATT, and Lyon Family-Centered Advance Care Planning Survey. Results: Fifty-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 Relevance: In 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.

Representation of Women and Minority Faculty and Fellows in Academic Pediatric Cardiology Training Programs.

BACKGROUND: Studies have shown that diverse care teams optimize patient outcomes. Describing the current representation of women and minorities has been a critical step in improving diversity across several fields. OBJECTIVES: To address the lack of data specific to pediatric cardiology, the authors conducted a national survey. METHODS: U.S. academic pediatric cardiology programs with fellowship training programs were surveyed. Division directors were invited (July 2021 to September 2021) to complete an e-survey of program composition. Underrepresented minorities in medicine (URMM) were characterized using standard definitions. Descriptive analyses at the hospital, faculty, and fellow level were performed. RESULTS: Altogether, 52 of 61 programs (85%) completed the survey, representing 1,570 total faculty and 438 fellows, with a wide range in program size (7-109 faculty, 1-32 fellows). Although women comprise approximately 60% of faculty in pediatrics overall, they made up 55% of fellows and 45% of faculty in pediatric cardiology. Representation of women in leadership roles was notably less, including 39% of clinical subspecialty directors, 25% of endowed chairs, and 16% of division directors. URMM comprise approximately 35% of the U.S. population; however, they made up only 14% of pediatric cardiology fellows and 10% of faculty, with very few in leadership roles. CONCLUSIONS: These national data suggest a "leaky pipeline" for women in pediatric cardiology and very limited presence of URRM overall. Our findings can inform efforts to elucidate underlying mechanisms for persistent disparity and reduce barriers to improving diversity in the field.

Contemporary Outcomes of Tracheostomy in Patients With Single Ventricle Heart Lesions.

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.

Paediatric cardiology training: burnout, fulfilment, and fears.

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.

Timing of Balloon Atrial Septostomy in Patients with d-TGA and Association with Birth Location and Patient Outcomes.

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 h for patients born at our institution vs. 14.1 h for those born elsewhere (p < .0001). A longer time from birth to BAS was associated with longer ICU (r = 0.21, p = 0.046) and hospital length of stay (r = 0.24, p = 0.02) and increased likelihood of elevated right ventricular pressure on post-operative discharge echocardiogram (p = 0.01). There were no differences in mortality between the groups. Therefore, prenatal planning for patients with known d-TGA should include a delivery plan with access to urgent BAS.