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Copyright of Automobile Technology is the property of Automobile Technology Editorial Office and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

There is currently no clear consensus on screening techniques to evaluate the presence or severity of Fontan-associated liver disease (FALD). Cardiac MRI (CMR) is used routinely for post-Fontan surveillance, but CMR-derived measures that relate to the severity of FALD are not yet defined. This was a cross-sectional single-center study of post-Fontan patients who underwent a CMR. CMR exams were re-analyzed by a single pediatric cardiologist. Surrogates of FALD included Gamma-Glutamyl Transferase (GGT), Fibrosis-4 laboratory score (FIB-4), and imaging findings. Findings consistent with cirrhosis on liver ultrasound included increased liver echogenicity and/or nodularity. Statistical analyses were performed to investigate potential relationships between CMR parameters and markers of FALD. Sixty-one patients were included. A larger inferior vena cava cross-sectional area (IVC-CSA) indexed to height was significantly associated with a higher FIB-4 score (Spearman's ρ = 0.28, p = 0.04), a higher GGT level (Spearman's ρ = 0.40, p = 0.02), and findings consistent with cirrhosis on liver ultrasound (OR 1.17, 95% CI: (1.01, 1.35), p = 0.04). None of the other CMR parameters were associated with markers of FALD. A larger indexed IVC-CSA was associated with higher systemic ventricle end-diastolic pressure (EDP) on cardiac catheterization (Spearman's ρ = 0.39, p = 0.018) as well as older age (Spearman's ρ = 0.46, p = < 0.001). Indexed IVC-CSA was the only CMR parameter that was associated with markers of FALD. This measure has the potential to serve as an additional non-invasive tool to improve screening strategies for FALD. Visual abstract summarizing the primary findings of this paper [ABSTRACT FROM AUTHOR]

D-transposition of the great arteries (d-TGA) is surgically repaired with the arterial switch operation (ASO) with excellent results, however short and long-term morbidities still develop including neurocognitive delay. Clinically significant central sleep apnea is uncommon in non-premature infants, but when present indicates immature autonomic control of respiration likely due to a neurologic disorder. We report the unanticipated finding of central sleep apnea in four-term neonates with d-TGA after uncomplicated ASO, with the short-term complication of delayed hospital discharge and long-term concerns regarding this early marker of brain immaturity and its hindrance to normal development. Within this report, we will review each patient's clinical course and then examine the literature on pediatric central sleep apnea, neurodevelopmental outcomes after ASO, and the important overlap of these entities in the care of patients going forward. [ABSTRACT FROM AUTHOR]

Copyright of Bagcilar Medical Bulletin / Bağcılar Tıp Bülteni is the property of Galenos Yayinevi Tic. LTD. STI and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Individuals with Fontan circulation (FC) often have diminished exercise capacity and are at risk for psychological problems. The current study examines associations between exercise capacity and psychological functioning in children and adolescents with FC. A multidisciplinary team evaluated participants in a clinic for pediatric patients with FC. Participants completed cardiopulmonary exercise testing (CPET). Parent and child were administered a psychological questionnaire (BASC-3) to measure child depression, anxiety, and inattention symptoms. Individuals who completed CPET with adequate effort and a psychological measure were eligible for inclusion. Clinical sample (n = 51) was 55% male with a mean age of 13.6 years (SD = 2.5). A majority had hypoplastic left heart syndrome (51%). Parent-report of inattention was negatively correlated with peak VO2 (R =  - .307, 95% CI - .549/ - .018, P = 0.038). Self-report of anxiety was positively correlated with HR recovery at 3 (R = .438, 95% CI .155/.655, P = 0.004) and 8 (R = .432, 95% CI .147/.651, P = 0.004) minutes post exercise. Depression was positively correlated with HR recovery at 3 min for parent-report (R = .294, 95% CI .004/.538, P = 0.047) and 8 min for self-report (R = .410, 95% CI .122/.635, P = 0.007). Greater inattention may have negatively impacted CPET engagement, reflected in lower peak VO2. The more rapid decline from max HR to recovery for those with greater depression and anxiety symptoms was unexpected, perhaps explained by a reduction in anxiety state after exercise or possibly an age effect, as a blunted HR decline has been found in adult cardiac patients with depression/anxiety., (© 2024. The Author(s).)

Background: An increasing number of centers are undertaking combined heart and liver transplantation in adult and pediatric patients with congenital heart disease., Aim: The primary aim of this study was to describe the perioperative management of a single center cohort, identifying challenges and potential solutions., Methods: We conducted a retrospective review of all patients undergoing combined heart and liver transplantation at Stanford Children's Hospital from 2006 to 2022. Preoperative information included cardiac diagnosis, hemodynamics, and severity of liver disease. Intraoperative data included length of surgery, cardiopulmonary bypass time, and blood products transfused. Postoperative data included blood products transfused in the intensive care unit, time to extubation, length of intensive care unit stay, survival outcomes and 30-day adverse events., Results: Eighteen patients underwent en bloc combined heart and liver transplantation at Stanford Children's Hospital from 2006 to 2022, and the majority 15 (83%) were transplanted for failing Fontan circulation with Fontan Associated Liver Disease. Median surgical procedure time was 13.4 [11.5, 14.5] h with a cardiopulmonary bypass time of 4.3 [3.9, 5.8] h. Median total blood products transfused in the operating room post cardiopulmonary bypass was 89.4 [63.9, 127.0] mLs/kg. Nine patients (50%) had vasoplegia during cardiopulmonary bypass. Activated prothrombin complex concentrates were used post cardiopulmonary bypass in 15 (83%) patients with a 30-day thromboembolism rate of 22%. Median time to extubation was 4.0 [2.8, 6.5] days, median intensive care unit length of stay 20.0 [7.8, 48.3] days and median hospital length of stay 54.0 [30.5, 68.3] days. Incidence of renal replacement therapy was 11%; however, none required renal replacement therapy by the time of hospital discharge. Neurological events within 30 days were 17% and the 30 day and 1 year survival was 89%., Conclusions: Perioperative challenges include major perioperative bleeding, unstable hemodynamics, and end organ injury including acute kidney injury and neurological events. Successful outcomes for en bloc combined heart and liver transplantation are possible with careful multidisciplinary planning, communication, patient selection, and integrated peri-operative management., (© 2024 John Wiley & Sons Ltd.)

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., (© 2024. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Backgroud. The results of the Fontan operation, depending on the anatomy of the pulmonary arteries, have been studied quite well. Various indices have been proposed to assess the degree of hypoplasia of the pulmonary arterial bed (Nakata, Reddy, and McGoon indexes). At the same time, an obstruction of pulmonary venous blood return may be considered as a contraindication to Fontan operation. Aim of the Study. To present an optimal method for pulmonary venous index (PVI) calculation based on computed tomography angiography (CTA) enhancement of the heart data in patients with a functional single ventricle. Materials and Methods. 63 patients with a functional single ventricle (SV) underwent CTА (Philips, Brilliance iCT) before the Fontan operation. Axial sections were reconstructed to a thickness of 0.75–3 mm using soft tissue and lung filters, followed by postprocessing of the data (Horos and OsiriX software) and construction of multiplanar and 3D images. The diagnoses were presented by various types of SV of the heart. The age of the patients ranged from 3 to 30 years (median 7 years). Comparison of PVI was carried out in patients of two groups: those who survived the intervention (n = 55 patients) and those who died (n = 8). The evaluation of the pulmonary veins (PV) and the calculation of the pulmonary venous index (PVI) were carried out based on the measurement of each pulmonary vein at 2 levels (at the level of the orifices and bifurcation). The calculation of the PVI was carried out according to the formula: the sum of the cross-sectional area of the main pulmonary veins, related to the body surface area. 3 variants of PVI calculation were compared: taking into account the values of the PV areas at the level of the orifices, the bifurcation, and the sum of the minimum areas of each of the PVs. Results. In the group of survived patients, the median PVI at the level of the PV orifices was 292 mm/m2, and in the group of deceased, it was 242 mm/m2 p = 0.0326 ; at the level of PV bifurcation in the group of survivors, it was 299 mm/m2, and in the group of dead patients, it was 281 mm/m2 p = 0.0776 ; the minimum PVI was 257 mm/m2 in the survivor group and 218 mm/m2 in the deceased group p = 0.006 . An ROC analysis performed to determine the critical value of the minimum PVI affecting survival after Fontan operation revealed that PVI measured taking into account the minimum dimensions of the areas of the PV is a significant risk factor for death after Fontan operation p = 0.00015 , with its value (cutoff) <233.5 mm2/m2. Conclusion. The value of the minimum PVI can be an important morphological indicator of the state of PV blood return and serve as an additional criterion in determining indications for the Fontan operation. [ABSTRACT FROM AUTHOR]

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., (© 2024. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Late preterm infants have lower breastfeeding rates than term infants, yet few published interventions or guidelines exist to guide hospitals in managing them in the non-intensive nursery setting. Here we describe the development and implementation of an interdisciplinary, hospital-based, breastfeeding support program among late preterm infants not requiring intensive care. The Early Bloomer Program includes an order set applied at birth, immediate lactation consultation, availability of donor milk, parent education on late preterm infants, hand expression teaching and kit including spoon and video link, and daily interdisciplinary team huddles. The program was developed with staff input using Diffusion of Innovation Theory, and implemented first among early adopters before expanding to all postpartum nurses. Nursing staff received education on late preterm infant physiology and feeding, and trainings on the Early Bloomers program through various learning modalities during the year leading up to implementation. To further understand the strengths and weakness of the program, we surveyed nurses (n = 43). Nurses reported that they were more attentive to the needs of late preterm infants, more confident in their care, and more aware of possible complications and feeding needs. Areas noted in need of improvement included lack of overnight lactation consults and little involvement of labor and delivery staff in the program. The Early Bloomers program increased confidence in care and was well received by nurses. Well-designed clinical studies are needed to identify effective breastfeeding support practices for late preterm infants. [ABSTRACT FROM AUTHOR]

The gold standard for determining the severity of liver disease in Fontan patients is now liver biopsy. Since it is an invasive procedure, this study determined the possibility of applying mitochondrial function from isolated peripheral blood mononuclear cells (PBMCs) as a non‐invasive indicator of liver fibrosis. Fontan patients (n = 37) without known liver disease were analysed cross‐sectionally. Patients were classified according to their histology using the METAVIR score as follows; F0/F1—no/mild fibrosis; F2—moderate fibrosis; and F3/F4—cirrhosis. Peripheral blood mononuclear cells were assessed for mitochondrial activity and apoptosis. This study did not find any significant differences in cardiac function among the groups according to liver histology. Interestingly, our findings indicated a significant decrease in maximal respiration and spare respiratory capacity, in both the moderate (F2) and cirrhosis (F3/F4) groups compared with the group without significant fibrosis (F0/F1). Moreover, the cirrhosis group exhibited higher levels of apoptosis and lower levels of live cells, compared with both the moderate and no significant fibrosis groups. In conclusion, the degree of liver fibrosis in Fontan patients is strongly correlated with mitochondrial dysfunction in PBMCs. Mitochondrial function and apoptosis could potentially serve as novel markers for tracking the progression of liver fibrosis in these patients. [ABSTRACT FROM AUTHOR]

Background. Quality education is inseparable from how leaders can empower all elements in schools by school conditions. Improving the quality of school-based education, starting from the state of schools that are believed to have weaknesses, is time for improvements. Purpose. Therefore educational leaders must be observant in seeing which conditions are most important for improvement or change so that the goal of improving the quality of school-based education achieves its goals. Making changes in the context of improving the quality of education cannot be done alone but requires the full support of all elements of the school, starting from educators, education staff, students and the school community. Method. This article uses the literature review method by analyzing several research results and some literature related to educational leadership and school-based quality. The literature review method collects data using various literature, such as books, journals, and other references that are considered appropriate to the research topic. Results. Effective educational leadership can cover all aspects of schools' need to improve quality, especially the quality of teaching staff as the frontline. The qualified teaching staff is a benchmark for school progress which will eventually become the quality of education. Therefore, it needs to be empowered according to the expertise possessed so that the quality of teaching staff can be improved sustainably. Educational leadership in carrying out their duties must be distinct from their leadership style, which also affects the quality of education. Conclusion. In addition, educational leadership also has competencies that are part of the leader's self to realize the achievement of educational goals. [ABSTRACT FROM AUTHOR]

Fontan circulation failure is a term used for cardiogenic non-cardiac and/or cardiac complications after Fontan surgery, such as persistent edema, reduced cardiac output with limited physical capacity, protein-losing enteropathy, plastic bronchitis, Fontan-associated liver disease, and arrhythmias. It is a serious late problem in patients after Fontan surgery. Its pathogenesis is complex, multifactorial and still a serious challenge in terms of its prevention and therapeutic behaviour. In recent years, there is a significant progress in the understanding of this problem, giving hope for achieving better results in the treatment of patients. The review presents comprehensive information concerning the risk factors, pathogenesis, diagnosis and management of these complications. [ABSTRACT FROM AUTHOR]

Sinus node dysfunction with concomitant junctional rhythm (JR) is frequently observed among Fontan patients and has been recognized as a contributor to heart failure. The impact and management of JR is unclear. A survey was mailed to all members of the Pediatric and Congenital Electrophysiology society (PACES) and members were asked to forward the questionnaire to their non-electrophysiology colleagues. Responses were received from 154 physicians (88 electrophysiologists (EP's) and 66 non-EP's (46 pediatric cardiologists and 20 adult congenital cardiologists). There were few differences in the response between EP's and non-EP's. Overall, 57% recommended an annual ambulatory ECG (AECG). A significant majority (80%) opted to continue to follow patients with significant periods of JR on AECG as long as the patients were asymptomatic, and showed no echocardiographic signs of cardiac decompensation. However, 84% would place a pacemaker in a patient with JR who was having open chest surgery for other reasons. Finally, pacemaker placement would be performed by 91% if a patient with JR showed signs of heart failure. Most congenital cardiologists would not recommend pacemaker placement in asymptomatic Fontan patients with JR. Further studies are needed on the Fontan population to determine the impact of SND and JR on longer term outcomes and to determine the role and optimal timing of pacemaker placement in these patients. [ABSTRACT FROM AUTHOR]

Copyright of Coal Science & Technology (0253-2336) is the property of Coal Science & Technology and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

End-stage Fontan patients with single-ventricle (SV) circulation are often bridged-to-heart transplantation via mechanical circulatory support (MCS). Donor shortage and complexity of the SV physiology demand innovative MCS. In this paper, an out-of-the-box circulation concept, in which the left and right ventricles are switched with each other is introduced as a novel bi-ventricle MCS configuration for the "failing" Fontan patients. In the proposed configuration, the systemic circulation is maintained through a conventional mechanical ventricle assist device (VAD) while the venous circulation is delegated to the native SV. This approach spares the SV and puts it to a new use at the right-side providing the most-needed venous flow pulsatility to the failed Fontan circulation. To analyze its feasibility and performance, eight SV failure modes have been studied via an established multi-compartmental lumped parameter cardiovascular model (LPM). Here the LPM model is experimentally validated against the corresponding pulsatile mock-up flow loop measurements of a representative 15-year-old Fontan patient employing a clinically-approved VAD (Medtronic-HeartWare). The proposed surgical configuration maintained the healthy cardiac index (3–3.5 l/min/m2) and the normal mean systemic arterial pressure levels. For a failed SV with low ejection fraction (EF = 26%), representing a typical systemic Fontan failure, the proposed configuration enabled a ~ 28 mmHg amplitude in the venous/pulmonary waveforms and a 2 mmHg decrease in the central venous pressure (CVP) together with acceptable mean pulmonary artery pressures (17.5 mmHg). The pulmonary vascular resistance (PVR)—SV failure case provided a ~ 5 mmHg drop in the CVP, with venous/pulmonary pulsatility reaching to ~ 22 mmHg. For the high PVR failure case with a healthy SV (EF = 44%) pulmonary hypertension is likely to occur as expected. While this condition is routinely encountered during the heart transplantation and managed through pulmonary vasodilators a need for precise functional assessment of the spared failed-ventricle is recommended if utilized in the PVR failure mode. Comprehensive in vitro and in silico results encourage this novel concept as a low-cost, more physiological alternative to the conventional bi-ventricle MCS pending animal experiments. [ABSTRACT FROM AUTHOR]

Background: The assessment of Fontan circuit’s flow is traditionally evaluated by multiple through-plane phase-contrast MRI acquisitions (2D flow), while recently, a single volumetric 4D-flow MRI acquisition is emerging as a comprehensive tool for the hemodynamic evaluation in congenital heart diseases. Purpose: To compare 2D and 4D-flow MRI measurements in patients after Fontan palliation and to evaluate parameters affecting potential disagreement. Methods: 39 patients after Fontan palliation (23 males, age 22 ± 11 years) who underwent cardiac MRI with 2D and 4D-flow MRI acquisition were included in the study. In all patients, blood flow quantification in the Fontan circuit and aorta by 2D flow and by 4D flow MRI acquisition blinding to the 2D results was performed. The agreement between 2D and 4D-flow MRI was calculated as the intraclass correlation coefficient (ICC). The mean absolute differences between 4D and 2D flows were analyzed using linear regression models. Results: 4D-flow MRI acquisition time was slightly lower than 2D (7.6 ± 1.8 min vs. 9.4 ± 3.3 min, p = 0.03). Flow was slightly predominant in the right pulmonary artery (58% of total pulmonary flow). Conduit/tunnel-pulmonary arteries flow accounted for 60% of the Fontan circuit. Agreement between 2D and 4D was overall good-to-excellent from ICC: 0.817 95% CI: 0.637–0.907 to 0.932 95% CI: 0.866–0.965. There was no significant influence of evaluated parameters on the agreement on 4D and 2D flow. Conclusions: 4D-flow MRI represents a valid tool in Fontan’s flow quantification. Further larger studies are needed to confirm our results and to evaluate the impact of advanced 4D-flow MRI parameters on the prognostic stratification in patients after Fontan palliation. [ABSTRACT FROM AUTHOR]

Technological advancements have greatly impacted the healthcare industry, including the integration of e-health in pediatric cardiology. The use of telemedicine, mobile health applications, and electronic health records have demonstrated a significant potential to improve patient outcomes, reduce healthcare costs, and enhance the quality of care. Telemedicine provides a useful tool for remote clinics, follow-up visits, and monitoring for infants with congenital heart disease, while mobile health applications enhance patient and parents' education, medication compliance, and in some instances, remote monitoring of vital signs. Despite the benefits of e-health, there are potential limitations and challenges, such as issues related to availability, cost-effectiveness, data privacy and security, and the potential ethical, legal, and social implications of e-health interventions. In this review, we aim to highlight the current application and perspectives of e-health in the field of fetal and neonatal cardiology, including expert parents' opinions. [ABSTRACT FROM AUTHOR]

Purpose of Review: Heart failure is the leading cause of morbidity and mortality in adults with congenital heart disease and is characterized by a variety of underlying mechanisms. Here, we aim to elaborate on the medical and technological advancements in the management of heart failure in adult patients with congenital heart disease and highlight the use of imaging modalities to guide therapy. Recent Findings: There have been several advances over the past decade with angiotensin receptor neprilysin and sodium-glucose cotransporter-2 inhibitors, atrioventricular valve clips, transcatheter pulmonary valves, catheter ablation, and cardiac resynchronization therapy, as well as the introduction of lymphatic interventions. Expanded use of echocardiography, cardiac magnetic resonance imaging, and cardiac computed tomography has guided many of these therapies. Summary: Significant innovations in the management of heart failure in adults with congenital heart disease have evolved with advancements in imaging modalities playing a critical role in guiding treatment therapies. [ABSTRACT FROM AUTHOR]

Background and Objectives: Patients with congenital heart disease (CHD), especially as a concomitant syndromal disease of trisomy 21 (T21), are at risk for impaired neurodevelopment. This can also affect these patients' education. However, there continues to be a research gap in the educational development of CHD patients and T21 CHD patients. Materials and Methods: In total, data from 2873 patients from the German National Register for Congenital Heart Defects were analyzed. The data are based on two online education surveys conducted among patients registered in the National Register for Congenital Heart Defects (2017, 2020). Results: Of 2873 patients included (mean age: 14.1 ± 4.7 years, 50.5% female), 109 (3.8%) were identified with T21 (mean age: 12.9 ± 4.4 years, 49.5% female). T21 CHD participants had a high demand for early specific interventions (overall cohort 49.1%; T21 cohort 100%). T21 CHD children more frequently attended special schools and, compared to non-trisomy 21 (nT21) CHD patients, the probability of attending a grammar school was reduced. In total, 87.1% of nT21 CHD patients but 11% of T21 CHD patients were enrolled in a regular elementary school, and 12.8% of T21 CHD patients could transfer to a secondary school in contrast to 35.5% of nT21 CHD patients. Most of the T21 CHD patients were diagnosed with psychiatric disorders, e.g., learning, emotional, or behavioral disorders (T21 CHD patients: 82.6%; nT21 CHD patients: 31.4%; p < 0.001). Conclusions: CHD patients are at risk for impaired academic development, and the presence of T21 is an aggravating factor. Routine follow-up examinations should be established to identify developmental deficits and to provide targeted interventions. [ABSTRACT FROM AUTHOR]

Cardiopulmonary bypass (CPB) provides cerebral oxygenation and blood flow (CBF) during neonatal congenital heart surgery, but the impacts of CPB on brain oxygen supply and metabolic demands are generally unknown. To elucidate this physiology, we used diffuse correlation spectroscopy and frequency-domain diffuse optical spectroscopy to continuously measure CBF, oxygen extraction fraction (OEF), and oxygen metabolism (CMRO2) in 27 neonatal swine before, during, and up to 24 h after CPB. Concurrently, we sampled cerebral microdialysis biomarkers of metabolic distress (lactate–pyruvate ratio) and injury (glycerol). We applied a novel theoretical approach to correct for hematocrit variation during optical quantification of CBF in vivo. Without correction, a mean (95% CI) +53% (42, 63) increase in hematocrit resulted in a physiologically improbable +58% (27, 90) increase in CMRO2 relative to baseline at CPB initiation; following correction, CMRO2 did not differ from baseline at this timepoint. After CPB initiation, OEF increased but CBF and CMRO2 decreased with CPB time; these temporal trends persisted for 0–8 h following CPB and coincided with a 48% (7, 90) elevation of glycerol. The temporal trends and glycerol elevation resolved by 8–24 h. The hematocrit correction improved quantification of cerebral physiologic trends that precede and coincide with neurological injury following CPB. [ABSTRACT FROM AUTHOR]

Lymphatic flow disorders are rare but devastating complications in children with congenital heart disease. T2-weighted magnetic resonance lymphography and intranodal dynamic contrast magnetic resonance lymphangiography are imaging modalities that can depict central lymphatic anatomy and flow pattern. Our objective was to describe the technical aspects and our imaging findings of central lymphatic abnormalities and their impact on patient management and outcomes: We conducted a retrospective review of 26 children with congenital heart disease who presented for lymphatic imaging between 2015 and 2020 at our institution. Eleven had postoperative chylothorax, six had plastic bronchitis, seven had protein-losing enteropathy and three had Noonan syndrome. Our lymphatic imaging demonstrated severely abnormal lymphatic flow in all of the children, but only minor abnormalities in protein-losing enteropathy. No major procedure-related complication occurred. Lymphatic interventions were performed in six patients, thoracic duct decompression in two patients and chylothorax revision in three patients. This led to symptomatic improvements in all of the patients: Lymphatic imaging is safe and essential for the diagnosis of lymphatic flow disorders and therapy planning. Our intranodal lymphangiography depicts an abnormal lymphatic flow pattern from the central lymphatics but failed to demonstrate an abnormal lymphatic flow in protein-losing enteropathy. These imaging techniques are the basis for selective lymphatic interventions, which are promising to treat lymphatic flow disorders. [ABSTRACT FROM AUTHOR]

Background and Aims: Fontan‐associated liver disease (FALD) is a long‐term complication of the Fontan procedure. Guidelines recommend elastography, but the utility of transient elastography (TE) and two‐dimensional shear wave elastography (2D SWE) is unknown. We aimed to evaluate the relationship between TE and 2D SWE in FALD. Methods: This prospective cohort study included 25 patients managed in a specialist clinic between January 2018 and August 2021. Trained clinicians performed 2D SWE (GE Logiq‐E9) and TE (FibroScan 503 Touch) on the same day under the same conditions. Laboratory, echocardiography, and imaging data were collected. The atrioventricular systolic‐to‐diastolic duration (AVV S/D ratio) was calculated as a measure of cardiac diastolic function. Results: We analyzed 40 paired measurements. Median age was 22 years. Median liver stiffness measurement (LSM) was 15.4 kPa (12.1–19.6) by TE and 8.0 kPa (7.0–10.3) (P = 0.001) by 2D SWE. There was weak correlation between the modalities (r = 0.41, P = 0.004). There was no correlation between time since Fontan and LSM by TE (r = 0.15, P = 0.19) or 2D SWE (r = 0.19, P = 0.13). There was no difference in LSM irrespective of whether sonographic cirrhosis was present or absent by TE (17.4 kPa [15.9–23.6] vs. 14.9 kPa [12.0–19.4], respectively, P = 0.6) or 2D SWE (9.0 kPa [2.8–10.5] vs. 8.0 kPa [6.7–10.1], P = 0.46). There was no correlation between AVV S/D ratio and LSM by TE (r = 0.16, P = 0.18) or 2D SWE (r = 0.02, P = 0.45). Conclusions: In FALD, TE and 2D SWE are poorly correlated. LSM by either modality was not associated with known risk factors for liver fibrosis or Fontan function. Based on these data, the role of elastography in FALD is uncertain. [ABSTRACT FROM AUTHOR]

Herein we respond to the controversial United States Food and Drug Administration (FDA) recommendation to perform thyroid function testing in children up to 3 years of age within 3 weeks of exposure to iodinated contrast media (ICM). Considering the many effects of thyroid hormone on the cardiovascular system, the increased risk of thyroid disease in patients with congenital heart disease, the hemodynamic consequences of the Fontan circulation on the thyroid gland, and the potential clinical significance of subclinical hypothyroidism, we share our perspective that the cardiovascular effects of thyroid hormone may carry more influence for patients with single ventricle heart disease. In our opinion, the FDA statement prompts us to consider routine thyroid hormone surveillance testing in the long-term management of patients with single ventricle heart disease. [ABSTRACT FROM AUTHOR]

Children with congenital heart disease (CHD) are at increased risk for neurodevelopmental challenges across the lifespan. These are associated with neurological changes and potential acquired brain injury, which occur across a developmental trajectory and which are influenced by an array of medical, sociodemographic, environmental, and personal factors. These alterations to brain development lead to an array of adverse neurodevelopmental outcomes, which impact a characteristic set of skills over the course of development. The current paper reviews existing knowledge of aberrant brain development and brain injury alongside associated neurodevelopmental challenges across the lifespan. These provide a framework for discussion of emerging and potential interventions to improve neurodevelopmental outcomes at each developmental stage. [ABSTRACT FROM AUTHOR]

Congenital heart disease (CHD) patients are at risk for alterations in the cerebral white matter microstructure (WMM) throughout development. It is unclear whether the extent of WMM alterations changes with age, especially during adolescence when the WMM undergoes rapid maturation. We investigated differences in WMM between patients with CHD and healthy controls from childhood until early adulthood in a pooled sample of children, adolescents, and young adults. The association between WMM and EF was assessed. Patients with CHD (N=78) and controls (N=137) between 9 and 32 years of age underwent diffusion tensor imaging and an executive function test-battery. Mean fractional anisotropy (FA) was calculated for each white matter tract. Linear regression tested age and group effects (CHD vs control) and their interaction on FA. Relative Variable Importance (RI) estimated the independent contribution of tract FA, presence of CHD, CHD complexity, and parental education to the variability in EF. Mean FA was lower in patients compared to controls in almost all tracts (p between 0.057 and <0.001). WMM alterations in patients were not different depending on age (all interaction effects p>0.074). Predictors of EF were CHD group (RI=43%), parental education (RI=23%), CHD complexity (RI=10%), FA of the hippocampal cingulum (RI=6%) and FA of the corticospinal tract (RI=6%). The lack of group-FA-interactions indicates that the extent of altered FA remains similar across age. Altered FA is associated with EF impairments. CHD is a chronic disease with cerebral and neurocognitive impairments persisting into adulthood and, thus, long-term follow-up programs may improve overall outcome for this population. [ABSTRACT FROM AUTHOR]

Objective: Sudden cardiac death (SCD) and malignant ventricular arrhythmia (VA) are increasingly recognized as important issues for people living with a Fontan circulation, but data are lacking. We sought to characterize the cohort who had sudden cardiac death, most likely related to VA and/or documented VA in the Australia and New Zealand Fontan Registry including risk factors and clinical outcomes. Methods: A retrospective cohort study was performed. Inclusion criteria were documented non-sustained ventricular tachycardia, sustained ventricular tachycardia, ventricular fibrillation, resuscitated cardiac arrest or SCD > 30 days post-Fontan completion. Results: Of 1611 patients, 20 (1.2%) had VA; 14 (1.0%) had VA without SCD and 6 (<1%) had SCD (6% of all deaths recorded in Registry; 5 of those had documented VA at the time of arrest and 1 was presumed to be VA-associated). The median age at first VA was 20.5 (14–32) years, 10 (50%) were females, and the median age at Fontan operation was 8 (4–17) years. On univariable analysis, hypoplastic left heart syndrome (p = 0.03) and older age Fontan operation (p < 0.001) were associated with VA. Earlier Fontan era (p < 0.003), atriopulmonary Fontan (p < 0.001), pre-Fontan atrioventricular valve repair (p = 0.013) pre- or post-Fontan atrial arrhythmia (p = 0.010) were associated with SCD. Patients with VA had a 3 times higher risk of death or heart transplant (HR 3.27(1.19, 8.98), p = 0.02). Conclusions: A proportion of people living with a Fontan circulation have malignant VA. Routine VA screening in this cohort is essential. More data are needed to aid risk stratification. [ABSTRACT FROM AUTHOR]

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To determine whether children who underwent resection of a congenital lung abnormality (CLA) are at higher risk for neurodevelopmental impairments than peers in the general population. The study population consisted of children born between 1999–2018 who underwent resection of a symptomatic CLA. Neurocognitive development (intelligence, memory, attention, visuospatial processing, executive functioning) and motor function of this population are monitored through our structured, prospective longitudinal follow-up program at the ages of 30 months, 5, 8, and 12 years. We compared study population scores with Dutch norm values using one-sample t-tests and one-sample binominal proportion tests. Forty-seven children were analyzed. The 8-year-olds showed significant impairments in sustained attention through the Dot Cancellation Test (mean z-scores -2.4; [-4.1; -0.8], p = 0.006 and -7.1; [-12.8; -1.4], p = 0.02 for execution speed and fluctuations respectively). Visuospatial memory was impaired at 8 years, though only in 1 out of 3 assessment tools (Rey Complex Figure Test z-scores (-1.0; [-1.5; -0.5], p < 0.001). Further neurocognitive outcomes were unimpaired at all tested ages. Regarding motor function outcomes, mean z-scores of total motor functioning were unimpaired across assessed ages. However, at 8 years, significantly more children than expected had definite motor problems (18% vs 5%, 95% CI [0.052; 0.403], p = 0.022). Conclusion: This evaluation reveals impairment in some subtests of sustained attention, visuospatial memory and motor development. However, globally, normal neurodevelopmental outcomes were found throughout childhood. We recommend testing for neurodevelopmental impairments in children who underwent surgery for CLA only if associated morbidities are present or if caregivers express doubts about their daily functioning. What is Known: • In general, surgically managed CLA cases seldom suffer from long-term surgery-related morbidity and show favorable lung function. What is New: • Long-term neurocognitive and motor function outcome appear unimpaired within surgically managed CLA cases. We recommend testing for neurodevelopmental impairments in children who underwent surgery for CLA only if associated morbidities are present or if caregivers express doubts about their daily functioning. [ABSTRACT FROM AUTHOR]

Background Patients with single ventricle congenital heart disease who undergo total cavo-pulmonary anastomosis (Fontan surgery) suffer from elevated pulmonary artery pressure (PAP), which leads to multiple adverse sequelae. Traditionally, the Fontan pressures are assessed via invasive haemodynamic catheterization that exposes these medically fragile patients to the risks of vascular injury and anaesthesia. While the CardioMEM remote PAP monitor has been extensively used in adults with heart failure, the safety of this device has not been established in children. Case summary We report safety and utility of this device in eight paediatric Fontan patients. Our patients ranged from 9 to 18 years of age. There were no reported complications related to the implantation of the CardioMEMS device in our population. Discussion This is the first case series of safety of CardioMEMS device in paediatric Fontan Powered by Editorial Manager® and ProduXion Manager® from Aries Systems Corporation patients. Our experience indicates that the device may be safely utilized for the management of Fontan-related complications in this vulnerable population. [ABSTRACT FROM AUTHOR]