Maude Abbott: "A Feminine Misfit in an Exclusive Male Environment" and Her Strategies for Success.

Maude Abbott was a pioneering female Canadian physician who became a world authority on medical museums and congenital heart disease. Abbott spent almost all her career in highly sexist, discriminatory work environments. This paper reviews Abbott's life and accomplishments, but, more importantly, analyzes her pathway to success in the masculine world of early 20th-century academic pathology. Abbott, though well-trained as a pathologist, never provided clinical service, but instead worked as museum curator at McGill University. She established the International Association of Medical Museums (predecessor to the International Academy of Pathology), edited its journal, and essentially ran the organization. Abbott, surrounded by influential males, dealt differently with each. In general, she recognized that male doctors believed women lacked the gravitas to lead major initiatives but that she could circumnavigate this supposed impediment by co-leading projects with male counterparts, preferably ones too busy to get in her way. She repeatedly used this approach, and by doing most of the work but sharing credit, succeeded in gaining reputation, accomplishment, and advancement. Abbott's pioneering work on congenital heart disease established her as one of the founders of pediatric pathology, and, overall, her career promoted the entry of women physicians into the pathology profession.

The Effect of Stenting on Post-Stenotic Dilatation in Coarctation of The Aorta.

The effect of stenting of native aortic coarctation (CoA) on post-stenotic dilatation (PSD) has not been previously described. We hypothesized that CoA stenting may lead to positive remodeling of PSD. Retrospective analysis of patients who underwent stent implantation for native CoA from 1999 to 2021 was performed. Primary outcome was incremental change PSD diameter and the PSD/DescAo (Descending Aorta) following stent implantation and comparison between covered and bare-metal stents. 90 consecutive patients, (26 female, average age at first intervention 12.0 years) were included. 35 patients (38.9%) underwent dilatation with bare-metal stents and 55 patients (61.1%) with covered stents. The covered stent subgroup was older (14.0 vs. 9.2 years old, p < 0.001) and PSD was larger (17.0 vs 14.0 mm, p < 0.001). Over a mean of 3.2 years, mean inter-catheterization growth of the PSD was blunted [- 0.05 mm, 95% CI (- 1.5 to 1.4)]. The covered stent subgroup demonstrated a negative inter-catheterization growth compared to the bare-metal stent subgroup (- 0.7 vs 1.6 mm, p < 0.001). When controlled for somatic growth, the PDS/DescAo decreased more significantly among those with covered stent vs bare metal (- 0.12 vs - 0.058, p = 0.004). Stenting of native CoA blunts the growth of PSD; covered stents were significantly associated with regression of the diameter of the PSD over time compared to bare-metal stents.

Team dynamics after implementation of guidelines for anaesthesia for children with CHD.

Guidelines were created at our single centrer institution for which anesthesiology team should care for pediatric cardiac patients for noncardiac surgery. The goal of the survey was to assess inter-team dynamics after the implementation of guidelines and revealed that practice behaviour can quickly change but a sustained change in team dynamics and workplace culture takes time.

Assessment of accuracy of two pulse oximeters in infants with cyanotic and acyanotic congenital heart diseases.

BACKGROUND: Peripherally measured oxygen saturation (SpO2) may often differ from arterial oxygen saturation (SaO2), measured by co-oximetry, especially within the lower range of oxygen saturations. This can potentially impact clinical decisions and therapy in children with congenital heart disease, as critical hypoxemia might remain unnoticed. AIMS: Our aim was to investigate the accuracy of two different pulse oximeters compared to SaO2 in infants with congenital heart diseases. METHODS: Simultaneous recordings of SpO2, measured by two different pulse oximeters (Philips IntelliVue X3 Monitor and Nellcor™ OxiMax™), were compared to SaO2 obtained by arterial blood gas analysis. RESULTS: A total of 153 measurements were performed in 44 infants with arterial oxygen saturation between 70 and 100%. We divided the measurements into 3 subgroups: group 1-SaO2 70.0%-85.0%, group 2-SaO2 85.1%-94.0%, group 3-SaO2 >94.1%. For Philipps, the median bias was 5.3 (IQR: 2.6-8.7) %, 2.3 (IQR: 0.9-6.0) % and 1.1 (IQR: -0.8-2.4) % in group 1, 2 and 3, respectively. For OxiMax™, the median bias was 2.7 (IQR: 0.5-5.1) %, 0.2 (IQR: -0.9-2.6) % and -0.5 (IQR: -1.3-0.6) % in group 1, 2 and 3, respectively. Regarding the accuracy of these oximeters, as evaluated with the Accuracy root mean squared index (Arms), it was 9.8 versus 4.5% in group 1, 4.5 versus 2.9% in group 2 and 2.4 versus 1.9% in group 3 for Philipps and OxiMax™, respectively. CONCLUSIONS: In lower range saturations between 70% and 85% the accuracy of both pulse oximeters exceeded the threshold of ≤3% recommended by the Food and Drug Administration (FDA). Therefore, peripheral pulse oximetry within the lower range of oxygen saturations should be interpreted with caution in infants with congenital heart diseases, taking into consideration its limitations. Direct co-oximetry should be the preferred method to support clinical decisions in children with cyanotic congenital heart diseases.

Hemodynamic Assessment of Infants With Congenital Heart Disease Using Ferumoxytol-Enhanced 4D Flow Cardiac Magnetic Resonance.

In complex congenital heart disease, characterization of the circulation is necessary to anticipate the clinical course. Four-dimensional cardiac magnetic resonance imaging enhanced by superparamagnetic iron oxide contrast agents (ferumoxytol) enables detailed and efficient assessment of both anatomy and physiology in neonates. We demonstrate this impact in 3 cases of neonates with congenital heart disease.

Longitudinal changes in systemic right ventricular remodeling in adult patients with transposition of the great vessels as assessed by cardiovascular magnetic resonance imaging.

BACKGROUND: Systemic right ventricular (sRV) physiology occurs in patients with congenitally corrected transposition of the great arteries (ccTGA) and D-TGA post atrial switch repair, and the natural history is of progressive sRV dysfunction. No study has assessed longitudinal changes in sRV remodeling by serial CMR. METHODS: Patients evaluated at two adult congenital heart disease centers and who underwent ≥2 CMR exams were studied. Indexed sRV end-diastolic volume (sRVEDVi), end-systolic volume (sRVESVi), and ejection fraction (sRVEF) were determined by a core laboratory. Concurrent echocardiograms were assessed for degree of systemic TR (sTR). Tricuspid valve events were defined as ≥moderate sTR, or interval tricuspid replacement (TVR). Generally, the earliest and most recent studies were compared. A subset of patients were followed with ≥moderate sTR, and then subsequently underwent interval TVR. For these patients, two study time-intervals were defined to analyze the impact of each event independently. RESULTS: 67 patients were studied (33±11 years, 47% male, 33% ccTGA), with 72 total time intervals studied (median interval 9.0 years [IQR 4.6-13.3]). There was a small increase in sRVEDVi over time (ΔsRVEDVi 5.5±15.8ml/m2, p<0.001), but mean change in sRVEF was not significant (ΔsRVEF 0.1±6.9%, p=0.86); notably, confidence intervals were wide for both. ccTGA patients had a trend towards greater decrement in sRVEF (ΔsRVEF -1.7±6.8 vs 1.3±6.7%, p=0.06). For each 25ml/m2 increase in baseline sRVEDVi, there was a 1.8% decrease in sRVEF (95% CI -3.2% to -0.5%, p=0.01). Patients without significant sTR had lesser deterioration in sRVEF compared to those with ≥moderate sTR or with interval TR intervention (ΔsRVEF 1.8±6.9% vs -2.1±6.6% and -2.6±4.5, p<0.05). Interval sRV conduction delay was associated with a trend towards greater decrements in sRVEF (ΔsRVEF -3.9±6.3 vs. 0.9±6.8%, p=0.07). Overall, underlying congenital anatomy, baseline sRVEDVi, advanced sTR or interval TVR, and sRV conduction delay explained only 16% of the variability in ΔsRVEF over time. CONCLUSIONS: Longitudinal changes in sRV remodeling were small, with great heterogeneity. Apparent risk factors in our study, namely underlying congenital anatomy, baseline sRVEDVi, TR events, and sRV conduction disease accounted for only 16% of the variability seen in the longitudinal change of sRVEF.

Heart transplantation for pediatric patients with malignant arrhythmias: Indications and Outcomes.

BACKGROUND: Arrhythmias can lead to cardiac arrest (CA) and heart failure. When intractable, heart transplant (HTX) can become the only viable treatment. This rare, high-risk cohort has not been reported as a distinct group. OBJECTIVE: Characterize the outcomes of pediatric patients listed for HTX with the primary indication being malignant arrhythmia (MA). METHODS: Using the Pediatric Heart Transplant Society prospective registry, we identified all patients <18 years-old listed between 2014-2022. MA as the listing indication was categorized into primary tachy-arrhythmia (PT), inherited arrhythmia (IA), congenital heart disease (CHD) and cardiomyopathy (CM) with secondary arrhythmia. Demographic, listing and transplant data were analyzed. RESULTS: Among 4630 patients listed and 3317 transplanted, MA was the indication in 63 (1.4%) and 49 (1.5%), respectively. MA patients were categorized as PT in 11, IA in 4, CHD in 6 and CM in 42. When compared to the non-MA cohort, patients listed for MA were older (mean (SD) age 10.6 (6.2) vs. 6.1 (6.2) years, p<0.01), more likely to present with a CA (43% vs. 11%, p<0.01), and less likely to be in the intensive care unit (40% vs. 58%, p<0.01) or on inotropes (30% vs. 60%, p<0.01) at listing. Outcomes including waitlist mortality, transplantation, post-transplant survival and freedom from rejection were comparable to the non-MA cohort. CONCLUSION: Patients with MA constitute a small proportion of those listed for HTX in childhood. CM was the most common category, while IA and PT were rare. Their waitlist mortality and post-transplant outcomes were comparable to the non-MA cohort.

Single-cell RNA sequencing profiling of mouse cardiac cells in response to retinoic acid.

Congenital heart disease (CHD) is the leading cause of birth defect-related mortality. CHD is a multifactorial, complex disease involving environmental factors playing important roles. To elucidate the cardiac cellular and molecular mechanisms of cardiac malformation, we administered pregnant mice with a single dose of all-trans retinoic acid (RA) at E8.5, as the CHD model. We performed single-cell RNA sequencing on cardiac cells from developing mouse hearts spanning from E8.5 to E17.5 after RA administration. A total of 69,447 cells were obtained from seven developmental stages ranging from E8.5 to E17.5. RA significantly impacted various CM subpopulations, particularly the outflow tract CMs at E9.0 by reduction of Tdgf1 expression. RA also influences the transition of endocardial-to-mesenchymal cells by decreasing the Stmn2 levels, which may contribute to abnormal valve development. In addition, RA altered the metabolic pattern of epicardial cells at E11.5 and promoted its differentiation potential. Taken together, these results are valuable for the development of preventive and therapeutic strategies for CHDs.

Correlation of Transthoracic Echocardiographic Estimates of Right Ventricular Pressure with Right Ventricular Pressure Measurements on Cardiac Catheterization in Children with Pulmonary Hypertension.

OBJECTIVES: Evaluate the correlation of non-invasive echocardiographic estimates of right ventricular systolic pressure with measurements on cardiac catheterization in children with pulmonary hypertension. DESIGN: Retrospective chart review. SETTING: Quaternary academic children's hospital. PARTICIPANTS: Patients younger than 18 years with a diagnosis of pulmonary hypertension and confirmatory cardiac catheterization from 2015 to 2018. MEASUREMENTS AND MAIN RESULTS: We analyzed the correlation between measures of right ventricular systolic pressure using nonparametric Spearman rho (ρ) with statistical significance set at p < 0.05. RESULTS: Children (N = 111) with biventricular circulation, strictly defined pulmonary hypertension, and adequate tricuspid regurgitation on echocardiogram to estimate right ventricular systolic pressure using the modified Bernoulli equation. Median age and weight were 4.3 years and 14.4 kg. Median right ventricular systolic pressure estimated by tricuspid regurgitant velocity on echocardiography was 55 mmHg (IQR 45-75 mmHg) plus right atrial pressure. On cardiac catheterization, median right ventricular systolic pressure was 57 mmHg (IQR 46-75 mmHg). Echocardiographic estimates of right ventricular systolic pressure were moderately well correlated with right ventricular systolic pressure directly measured on catheterization (ρ = 0.44, 95% CI 0.27-0.6, p < 0.001) with a median difference of 4 mmHg (IQR -10 to 17). Subgroup analysis revealed that echocardiography and catheterization measurements correlated well in children with suprasystemic right ventricular pressure on cardiac catheterization (ρ = 0.75, 95% CI 0.51-0.99, p < 0.001) although catheterization measurements were a median of 26 mmHg (IQR 12-31) higher than echocardiographic estimates in this subgroup. CONCLUSIONS: In children with pulmonary hypertension, echocardiographic estimates of right ventricular pressure correlated moderately well with gold standard measurements by cardiac catheterization with stronger correlation in children with suprasystemic right ventricular pressures. This is reassuring for clinicians who must rely on echocardiography for risk stratification before anesthetizing children with pulmonary hypertension.

Outcomes of the Modified Warden Procedure for Partial Anomalous Pulmonary Venous Connection Drainage to a High Location in the Superior Vena Cava.

AIM: To report the safety and effectiveness of a modified Warden procedure. METHOD: Twenty-six (26) patients underwent modified Warden surgery at our centre from September 2017 to September 2021. In all patients, the superior vena cava (SVC) was reconstructed by turning the atrial wall in the main body of the right atrium upwards and applying fresh autologous pericardial patches. There were 13 male patients and 13 female patients, and the median age of the patients was 7.0 (range, 0.3-47.0) years. The median follow-up time after surgery was 47.0 (range, 32.0-80.0) months. RESULTS: The aortic cross-clamp time was 108.50±34.72 minutes, the cardiopulmonary bypass time was 154.81±41.65 minutes, the median postoperative mechanical ventilation time was 8.5 (range, 0.0-91.0) hours, the median intensive care unit stay was 43.5 (range, 15.0-352.0) hours, and the median length of postoperative hospital stay was 7.0 (range, 6.0-19.0) days. All patients were safely and successfully discharged from the hospital. No deaths or sinus node dysfunction occurred during the follow-up period. The mean gradient of the reconstructed SVC and/or the right pulmonary vein (PV) increased in eight patients and decreased to less than 1 mmHg in six patients. One (1) adult patient had already undergone two vascular interventions, and one child had persistent stenosis but did not undergo reoperation at the time of the study. In addition, two patients were subjected to pericardial windowing. CONCLUSIONS: A modified Warden technique using a right atrial flap and autologous pericardium effectively corrected the high-plane connection between the PVs and SVC. This technique effectively avoids sinus node dysfunction; however, a short-term increase in the differential pressure of the re-established SVC or PV after the procedure is acceptable, and reintervention is required only in rare cases.

A comparative study of fetal cardiovascular assessment: utilizing Doppler ultrasound gated MRI and echocardiography with detailed analysis using five axial views.

Objectives: To investigate the diagnostic performance of fetal cardiovascular magnetic resonance imaging (MRI) using Doppler ultrasound (DUS) gating for the evaluation of the standardized five axial views in comparison with fetal echocardiography. Methods: In this prospective study 29 pregnant women (median: 34.4 weeks of gestation) underwent fetal cardiovascular MRI using DUS gating at 3 Tesla. The standardized five axial views in prenatal screening (fetal abdomen, four-chamber view, left ventricular outflow tract, right ventricular outflow tract, and three-vessel view) were independently assessed and analysed by both fetal MRI and fetal echocardiography on the same day. Image analysis included qualitative assessment and quantitative measurements of cardiovascular structures. MR image quality was assessed using a 4-point scale (from 1 = low to 4 = excellent). Postnatal echocardiography was performed for validation. Results: 17/28 fetuses (60.7%) had pathological findings [16 congenital heart defect (CHD), one diaphragmatic hernia] in prenatal echocardiography. One fetus was excluded due to severe motion. Overall sensitivity and specificity in detecting fetal cardiac abnormalities was 88% and 100%, respectively, for fetal MRI and 100% and 100% for fetal echocardiography. MR image quality for evaluation of cardiac structures was high with a mean score of 2.8 (±0.8) (score 4: 15.9%, score 3: 53.8%, score 2: 19.3%, score 1: 11%). Quantitative measurements did not differ between fetal cardiovascular MRI and fetal echocardiography (all p > 0.05). Conclusion: Diagnostic performance of fetal cardiovascular MRI using DUS gating was comparable to fetal echocardiography. Fetal cardiovascular MRI using DUS gating might be a valuable diagnostic adjunct for the prenatal evaluation of CHD.

Twenty-Seven-Year Institutional Experience With Surgery for Adults With Congenital Heart Disease.

BACKGROUND: Given improved contemporary survival of adults with congenital heart disease (ACHD), we aimed to evaluate trends in ACHD surgery and outcomes at a single center over a 27-year period. METHODS: Surgical databases were retrospectively queried for patients >18 years old who underwent ACHD surgery between January 1, 1994, and December 31, 2020. A total of 2,195 included patients underwent 2,425 cardiac surgical procedures within the specified time frame. Patients were grouped by era: I, 1994-2000; 2, 2001-2010; and 3, 2011-2020. Trends in primary cardiac diagnosis and surgical management were evaluated. RESULTS: The median age increased across the eras. The most common primary cardiac diagnoses (n = 2,425) overall were left ventricular outflow tract anomalies (n = 2,019, 83%), atrial septal defect (n = 407, 17%), right ventricular outflow tract anomalies (n = 360, 15%), and ventricular septal defect (n = 110, 4.5%). The most commonly observed procedures overall were operations on the left ventricular outflow tract (n = 1,633, 67%), aorta (n = 675, 28%), coronary arteries (n = 449, 19%), right ventricular outflow tract (n = 323, 13%), and atrial septal defect (n = 264, 11%). Major complications occurred in 10% of cases, and 58 patients died within 30 days of their operation yielding an operative mortality of 2.4%. CONCLUSION: To our knowledge, this is the largest single center report on surgery for adults with congenital heart disease. Surgery for ACHD has been performed at our center with relatively low morbidity and mortality over the last few decades.

Complete Transposition of the Great Arteries: 5-Year Experience From a Single Center.

Complete transposition of the great arteries (TGA) is the most common cause of cyanosis in the first week of life. Prenatal diagnosis helps with counseling, planning delivery, and postnatal management for resource-rich health services. In a resource-limited setting, postnatal diagnosis is the norm. This work examines cases of complete TGA in one center in Cambodia without prenatal testing. Twenty-four cases were studied over 5 years. Complex TGA was frequently seen. The majority had normal coronary anatomy and arterial switch operation (ASO) was performed in most cases with a favorable outcome.

Enhancement of the lymphatic system following intravenous administration of ferumoxytol.

BACKGROUND: Lymphatic imaging is becoming increasingly important in the management of patients with congenital heart disease. However, the influence of the intravenous contrast agent ferumoxytol on lymphatic imaging is not well understood. OBJECTIVE: To evaluate the impact of intravenous ferumoxytol on T1-weighted and T2-weighted lymphatic imaging in patients with congenital heart disease. MATERIALS AND METHODS: We included consecutive patients receiving ferumoxytol-enhanced 3D angiography for congenital heart disease evaluation. The visibility of the thoracic duct was reviewed on the T1-weighted 3D inversion recovery balanced-steady-state free precession (SSFP) with respiratory navigator gating sequence which is routinely used for angiography and the heavily T2-weighted turbo spin echo sequence which is employed for lymphatic imaging. Data on demographics and time interval between contrast administration and imaging were collected. Statistical analyses were performed using t-tests for continuous variables and chi-squared tests for categorical variables. RESULTS: One hundred nineteen consecutive patients with a mean age of 12.46 years±7.7 years were included. Of these, 45 cases underwent both T1-weighted and T2-weighted imaging; the other 74 underwent only T1-weighted imaging. Of the 45 patients, 20 had thoracic duct enhancement on T1-weighted imaging; among the 26 sedated, only 2 showed enhancement, while 18 of 19 non-sedated patients showed enhancement (P<0.001), indicating a strong association between sedation and reduced thoracic duct visibility. If T2-weighted imaging was performed after contrast administration, the thoracic duct was not visible on those images. For all 45 cases of visible thoracic duct in the entire cohort, the time from contrast administration to imaging ranged from 8 min up to 75 min. CONCLUSION: The enhancement of the thoracic lymphatic duct on T1-weighted imaging, coupled with degradation observed on T2-weighted imaging, suggests that intravenously administered ferumoxytol rapidly enters the lymphatic fluid. To prevent T2 shortening from degrading the imaging results, T2-weighted imaging for lymphatic evaluation should be performed prior to the administration of ferumoxytol. Sedation and, by inference, fasting may influence this property and warrant further investigation in future studies.

Congenital Heart Disease among Children Undergoing Echocardiography in the Department of Paediatrics of Tertiary Care Centre: A Descriptive Cross-sectional Study.

INTRODUCTION: Congenital heart disease in children are a major cause of infant mortality and morbidity. It is important to detect and manage these disorders timely as they are preventable. The objective of this study was to find out proportion of congenital heart disease in children in paediatric department in a tertiary hospital. METHODS: This is a descriptive cross-sectional study carried out in the Department of Paediatric at Nepal Medical College and Teaching Hospital where all children (0-18 years) suspected to have congenital heart disease who underwent echocardiography were studied over a period of 1 year (2020-2021). The presence or absence of congenital heart disease were confirmed by echocardiography performed by paediatric cardiologist. The socioeconomic variables,clinical features and echocardiography findings were noted. RESULTS: Out of total 249 patients,the proportion of patients diagnosed to have cardiac disorders was 73 with male predominance of 165 (66.26%). The most common age group was found to be neonates 111 (44%).The notable clinical features were murmur 47 (18.87%), tachypnoea 27 (10.84%) ,tachycardia 27 (10.84%) and cyanosis 9 (3.61%), clubbing 2 (0.80%), oedema 1 (0.40%), hypertension 9 (3.65%), murmur 47 (18.87%).Out of the total, there were 49 (19.67%) cases of acyanotic congenital heart disease, and 27 (10.84%) cases of cyanotic congenital heart disease. CONCLUSIONS: Our study focuses on early recognition of cardiac diseases which is crucial for preventing morbidity and mortality.

Comprehensive Review of the Psychosocial Impact on Parents of Newborns With Congenital Heart Disease: A Significant Problem in Low- and Middle-Income Countries.

Congenital heart disease (CHD) is a common and significant congenital disorder. Despite advancements in neonatal care, Congenital heart disease remains a leading cause of death among infants with congenital malformations. Congenital heart disease is responsible for a significant portion of stillbirths and includes several forms with various anatomical variations. A CHD diagnosis leads to significant emotional distress for parents, affecting family dynamics and quality of life. Parents often experience psychological impacts, such as anxiety, depression, and guilt, particularly when diagnoses occur after birth. This literature review explores the psychosocial impact of CHD on families and examines how the timing of diagnosis influences outcomes. Parents face numerous challenges, including managing complex medical care and addressing financial and emotional burdens. Effective communication between healthcare providers and families is crucial, as is providing continuous emotional support and counseling. Early psychological interventions can alleviate depression and anxiety, and pediatric psychologists play a significant role in reducing the negative long-term effects on neurodevelopment. Further research is needed to develop strategies to improve mental health services and enhance the quality of life for families affected by CHD.

Stepped-Care Web-Based Parent Support Following Congenital Heart Disease: Protocol for a Randomized Controlled Trial.

BACKGROUND: Early neurodevelopmental risks, compounded with traumatic medical experiences, contribute to emotional and behavioral challenges in as many as 1 in 2 children with congenital heart disease (CHD). Parents report a strong need for supports; yet, there remains a lack of accessible, evidence-based behavioral interventions available for children with CHD and their families. I-InTERACT-North is a web-based stepped-care mental health program designed to support family well-being and reduce behavioral concerns through positive parenting for children with early medical complexity. In previous pilot studies, the program was effective in increasing positive parenting skills and decreasing child behavior problems, with high parent-reported acceptability. This paper presents the protocol for the first randomized study of stepped-care parent support for families of children with CHD. OBJECTIVE: This study will involve a single-site, 2-arm, single-blind randomized controlled trial to evaluate (1) the feasibility and acceptability of a web-based stepped-care parent support program (I-InTERACT-North) and (2) the effectiveness of the program in enhancing positive parenting skills and reducing behavioral concerns among families of children with CHD. METHODS: Families will be randomized (1:1) to either receive treatment or continue with care as usual for 12 months. Randomization will be stratified by child's sex assigned at birth and baseline parent-reported child behavior intensity. Primary outcomes include positive parenting skills and child behavior at baseline, 3 months, 6 months, and 12 months. Secondary outcomes include parental mental health, quality of life, service usage, and feasibility including program reach and adherence. A sample size of 244 families will provide >95% power to detect an effect size of d=0.64. Based on attrition data from pilot studies, a target of 382 families will be enrolled. Parent reports of acceptability, adoption, and suggested adaptability of the program will be examined using cross-case thematic analyses. Primary efficacy analysis will be conducted using an intent-to-treat approach. Generalized estimating equations will be used to examine changes in positive parenting. Child behavior, quality of life, and parent mental health will be tested with repeated-measures analyses. Additional sensitivity and replication analyses will also be carried out. RESULTS: Recruitment began in February 2024, and recruitment and follow-up will continue until January 2029. We anticipate results in late 2029. CONCLUSIONS: This study aims to test the effectiveness of I-InTERACT-North web-based stepped-care parent support in improving positive parenting skills and reducing child behavior problems in families of children with CHD compared with a care as usual control group. Results will inform future clinical implementation and expansion of this program among families of children with early medical conditions. TRIAL REGISTRATION: ClinicalTrials.gov NCT06075251; https://clinicaltrials.gov/study/NCT06075251. INTERNATIONAL REGISTERED REPORT IDENTIFIER (IRRID): DERR1-10.2196/64216.

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.

Surgical repair of "Swiss Cheese" ventricular septal defects with two-patch and right ventricular apex-exclusion technique: mid-term follow-up results.

BACKGROUND: "Swiss Cheese" ventricular septal defects represent a serious congenital heart disease with suboptimal clinical outcomes and a lack of consensus regarding its management. This study presents mid-term follow-up results of surgical repairs for "Swiss Cheese" ventricular septal defects, utilizing the two-patch and right ventricle apex-exclusion technique. METHODS: A retrospective review was conducted on 13 patients who underwent surgical repair utilizing the two-patch and right ventricle apex-exclusion technique at our institution between May 2014 and October 2021. The procedure involved the closure of defects in the outflow tract ventricular septal and the apex trabecular ventricular septal regions using two patches, with concurrent exclusion of the right ventricular apex from the right ventricular inflow tract. RESULTS: Median follow-up was 4.9 ± 2.1 years (range: 2-9 years). All cases were successful without mortality or major complications. Two years post-surgery, cardiac magnetic resonance revealed median values for left ventricular ejection fraction, right ventricular ejection fraction, left ventricular end-diastolic volume and right ventricular end-diastolic volume of 63.9% ± 1.8% (range: 61-67%), 49.2% ± 2.6% (range: 46-55%), 39.15 ± 2.11 ml (range: 36.2-42.7 ml), 44.55 ± 3.33 ml (range: 38.7-48.6 ml), respectively. No thrombosis occurred. The latest echocardiography results confirmed normal cardiac function in all cases. CONCLUSIONS: The surgical repair of "Swiss Cheese" ventricular septal defects utilizing the two-patch and right ventricle apex-exclusion technique is a viable approach with favorable mid-term outcomes. More cases and long-term follow-up results are needed to validate the feasibility and safety of this technique.

Tetralogy of Fallot With Absent Pulmonary Valve Syndrome: The Experience of a Tertiary Care Center in a Developing Country.

BACKGROUND: Tetralogy of Fallot with an absent pulmonary valve is a very rare variant of tetralogy. It is characterized by absent valve tissue, severe pulmonary regurgitation, and secondary aneurysmal dilatation of the pulmonary arteries. AIM: In this study, we aim to investigate the clinical presentations, management strategies, and outcomes of patients with tetralogy of Fallot and absent pulmonary valve. METHODOLOGY: We retrospectively reviewed the charts of all patients who presented to the American University of Beirut Medical Center between January 2010 and December 2020 and who were diagnosed with this anomaly. RESULTS: A total of 300 cases of tetralogy of Fallot were identified, of which 18 patients had absent pulmonary valves. They were followed up for an average of 8.2 years. Prenatal diagnoses were made in four patients, while 13 patients were identified in the neonatal period, with an average age of 4.5 days. Genetic testing confirmed DiGeorge syndrome in one patient. Five patients underwent surgical intervention in the neonatal period, while the remaining patients were operated on during their early childhood. While overall there were no surgical mortalities nor any need for reinterventions, a variety of morbidities were encountered. CONCLUSION: This study provides an overview of this rare anomaly and its management in a developing country.