Processing Speed Partially Mediates Executive Function Impairments in Adolescents with Congenital Heart Disease: Results from a Prospective Cohort Study.

OBJECTIVE: To assess processing speed, fine motor function, attention, and executive function (EF) impairments in adolescents with complex congenital heart disease (CHD) who underwent open-heart surgery during infancy. STUDY DESIGN: We administered a comprehensive neuropsychological test battery evaluating 5 EF domains: working memory, inhibition, cognitive flexibility, fluency, and planning and primary neurodevelopmental processes (PNPs): processing speed, fine motor function, and attention. The sample included 100 adolescents with complex CHD from a previous University Children's Hospital Zurich study, with 104 healthy controls for comparison. We generated scores for each EF domain and computed an EF summary score. Group comparisons and associations were analyzed with multiple regression, accounting for parental education. Mediation analysis explored how PNPs mediate the effect between a CHD diagnosis and EF. RESULTS: In adolescents with complex CHD all EF domains and the EF summary score were impaired (ß=0.20 to 0.37, all P<0.05). Furthermore, they exhibited slower processing speed (ß=0.27, P<0.01) than healthy controls, with no differences in attention (ß=-0.07, P=0.34) and fine motor function (ß=0.08, P=0.34). Processing speed showed a strong association with the EF summary score (ß=0.60, P<0.001) and partially mediated the relationship between CHD diagnosis and the EF summary score (ß=0.37, 95% CI [0.24, 0.50], P<0.001). CONCLUSION: Adolescents with complex CHD show difficulties in EFs and processing speed. Notably, processing speed is strongly associated with EFs and partly accounts for EFs disparities between patients and healthy controls. Early detection and interventions for processing speed difficulties may improve EF outcomes in these patients.

Stress Markers, Executive Functioning, and Resilience Among Early Adolescents With Complex Congenital Heart Disease.

Importance: Infants with complex congenital heart disease (cCHD) may experience prolonged and severe stress when undergoing open heart surgery. However, little is known about long-term stress and its role in neurodevelopmental impairments in this population. Objective: To investigate potential differences between early adolescents aged 10 to 15 years with cCHD and healthy controls in physiological stress markers by hair analysis, executive function (EF) performance, and resilience. Design, Setting, and Participants: This single-center, population-based case-control study was conducted at the University Children's Hospital Zurich, Switzerland. Patients with different types of cCHD who underwent cardiopulmonary bypass surgery during the first year of life and who did not have a genetic disorder were included in a prospective cohort study between 2004 and 2012. A total of 178 patients were eligible for assessment at ages 10 to 15 years. A control group of healthy term-born individuals was cross-sectionally recruited. Data assessment was between 2019 and 2021. Statistical analysis was performed from January to April 2023. Exposure: Patients with cCHD who underwent infant open heart surgery. Main Outcomes and Measures: Physiological stress markers were quantified by summing cortisol and cortisone concentrations measured with liquid chromatography with tandem mass spectrometry in a 3-centimeter hair strand. EFs were assessed with a neuropsychological test battery to produce an age-adjusted EF summary score. Resilience was assessed with a standardized self-report questionnaire. Results: The study included 100 patients with cCHD and 104 controls between 10 and 15 years of age (mean [SD] age, 13.3 [1.3] years); 110 (53.9%) were male and 94 (46.1%) were female. When adjusting for age, sex, and parental education, patients had significantly higher sums of hair cortisol and cortisone concentrations (ß, 0.28 [95% CI, 0.12 to 0.43]; P < .001) and lower EF scores (ß, -0.36 [95% CI, -0.49 to -0.23]; P < .001) than controls. There was no group difference in self-reported resilience (ß, -0.04 [95% CI, -0.23 to 0.12]; P = .63). A significant interaction effect between stress markers and EFs was found, indicating a stronger negative association in patients than controls (ß, -0.65 [95% CI, -1.15 to -0.15]; P = .01). The contrast effects were not significant in patients (ß, -0.21 [95% CI, -0.43 to -0.00]; P = .06) and controls (ß, 0.09 [95% CI, -0.11 to 0.30]; P = .38). Conclusions and Relevance: This case-control study provides evidence for altered physiological stress levels in adolescents with cCHD and an association with poorer EF. These results suggest that future studies are needed to better understand the neurobiological mechanisms and timing of alterations in the stress system and its role in neurodevelopment.

Stenting of native right ventricular outflow tract obstructions in symptomatic infants: histological work-up of explanted specimen.

BACKGROUND: Stenting of stenotic right ventricular outflow tract is a palliative measure for severely impaired small babies with Tetralogy of Fallot or similar pathologies. Little is known about the histopathological fate of the stents in the right ventricular outflow tract. METHODS: Eight samples of surgically removed right ventricular outflow tract stents were histologically analysed according to a predefined protocol. RESULTS: The most frequent diagnosis was Tetralogy of Fallot in four patients, pulmonary atresia with ventricular septal defect in two patients, double outlet right ventricle with pulmonary obstruction in one patient, and muscular obstruction of the right ventricular outflow tract in one patient with a syndromic disease with hypertrophic cardiomyopathy. Stents mean implantation duration was 444 days ranging from 105 to 1117 days (median 305.5 days). Histology revealed a variable degree of pseudointima formation consisting of fibromuscular cells surrounded by extracellular matrix. Four of the specimen contained adjacent myocardial tissue fragments, which showed regressive changes. Neither myocardium nor pseudointima tissue or tissue parts locally related to stent struts were infiltrated by inflammatory cells. CONCLUSIONS: Histological analysis after explantation of early-in-life implanted right ventricular outflow tract stents revealed predominantly pronounced neo-intimal proliferation with a visible endothelial layer, no signs of inflammation, and no prolapse of muscular tissue through the stent struts. Thus, implantation of stents in early life seems to interfere little with the hosts' immune system and might help to open up the right ventricular outflow tract by mechanical forces and regressive changes in adjacent muscular tissue.

Pulmonary hemodynamics before and after pediatric heart transplantation.

BACKGROUND: Pulmonary hypertension (PH) may limit the outcome of pediatric heart transplantation (pHTx). We evaluated pulmonary hemodynamics in children undergoing pHTx. METHODS: Cross-sectional, single-center, observational study analyzing pulmonary hemodynamics in children undergoing pHTx. RESULTS: Twenty-three children (female 15) underwent pHTx at median (IQR) age of 3.9 (.9-8.2) years with a time interval between first clinical signs and pHTx of 1.1 (.4-3.2) years. Indications for pHTx included cardiomyopathy (CMP) (n = 17, 74%), congenital heart disease (CHD) (n = 5, 22%), and intracardiac tumor (n = 1, 4%). Before pHTx, pulmonary hemodynamics included elevated pulmonary artery pressure (PAP) 26 (18.5-30) mmHg, pulmonary capillary wedge pressure (PCWP) 19 (14-21) mmHg, left ventricular enddiastolic pressure (LVEDP) 17 (13-22) mmHg. Transpulmonary pressure gradient (TPG) was 6.5 (3.5-10) mmHg and pulmonary vascular resistance (Rp) 2.65 WU*m2 (1.87-3.19). After pHTx, at immediate evaluation 2 weeks after pHTx PAP decreased to 20.5 (17-24) mmHg, PCWP 14.5 (10.5-18) mmHg (p < .05), LVEDP 16 (12.5-18) mmHg, TPG 6.5 (4-12) mmHg, Rp 1.49 (1.08-2.74) WU*m2 resp.at last invasive follow up 4.0 (1.4-6) years after pHTx, to PAP 19.5 (17-21) mmHg (p < .05), PCWP 13 (10.5-14.5) mmHg (p < .05), LVEDP 13 (10.5-14) mmHg, TPG 7 (5-9.5) mmHg, Rp 1.58 (1.38-2.19) WU*m2 (p < .05). In CHD patients PAP increased (p < .05) after pHTx at immediate evaluation and decreased until last follow-up (p < .05), while in CMP patients there was a continuous decline of mean PAP values immediately after HTx (p < .05). CONCLUSIONS: While PH before pHTx is frequent, after pHTx the normalization of PH starts immediately in CMP patients but is delayed in CHD patients.

Multimodal personalised executive function intervention (E-Fit) for school-aged children with complex congenital heart disease: protocol for a randomised controlled feasibility study.

INTRODUCTION: Children with congenital heart disease (CHD) are at risk for executive functions (EF) impairments. To date, interventions have limited effects on EF in children and adolescents with complex CHD. Therefore, we developed a new multimodal and personalised EF intervention (E-Fit). This study aims to test the feasibility of this intervention called 'E-Fit' for children with complex CHD and EF impairments. METHODS AND ANALYSIS: This is a single-centre, single-blinded, randomised controlled feasibility study exploring the E-Fit intervention. We aim to enrol 40 children with CHD aged 10-12 years who underwent infant cardiopulmonary bypass surgery and show clinically relevant EF impairments (T-score ≥60 on any Behaviour Rating Inventory for Executive Function questionnaire summary scale). The multimodal intervention was developed with focus groups and the Delphi method involving children and adolescents with CHD, their parents and teachers, and health professionals. The intervention is composed of three elements: computer-based EF training using CogniFit Inc 2022, performed three times a week at home; weekly EF remote strategy coaching and analogue games. The content of the computer and strategy training is personalised to the child's EF difficulties. The control group follows their daily routines as before and completes a diary about their everyday activities four times a week. Participants will be randomised in a 1:1 ratio. Feasibility is measured by the participants' and providers' ratings of the participants' adherence and exposure to the intervention, recruitment rates and the evaluation of the intended effects of the programme. ETHICS AND DISSEMINATION: Local ethics committee approval was obtained for the study (BASEC-Nr: 2021-02413). Parents provide written informed consent. Key outputs from the trial will be disseminated through presentations at conferences, peer-reviewed publications and directly to participating families. Furthermore, these results will inform the decision whether to proceed to a randomised controlled trial to investigate effectiveness. TRIAL REGISTRATION NUMBER: NCT05198583.

Risk Factors and Outcome of Pulmonary Artery Stenting After Bidirectional Cavopulmonary Connection (BDCPC) in Single Ventricle Circulation.

After bidirectional cavopulmonary connection (BDCPC) central pulmonary arteries (PAs) of single ventricle (SV) patients can be affected by stenosis or even closure. Aim of this study is to compare SV patients with and without PA-stent implantation post-BDCPC regarding risk factors for stent implantation and outcome. Single center, retrospective (2006-2021) study of 136 SV consecutive patients with and without PA-stent implantation post-BDCPC. Patient characteristics, risk factors for PA-stent implantation and PA growth were assessed comparing angiographic data pre-BDCPC and pre-TCPC. A total of 40/136 (29%) patients underwent PA-stent implantation at median (IQR) 14 (1.1-39.0) days post-BDCPC. 37/40 (92.5%) underwent LPA-stenting. Multiple regression analysis showed single LV patients to receive less likely PA-stents than single RV patients (OR 0.41; p = 0.05). Reduced LPA/BSA (mm/m2) and larger diameter of neo-ascending aorta pre-BDCPC were associated with an increased likelihood of PA-stent implantation post-BDCPC (OR 0.89, p = 0.03; OR 1.05, p = 0.001). Stent re-dilatation was performed in 36/40 (89%) after 1 (0.8-1.5) year. Pulmonary artery diameters pre-BDCPC were lower in the PA-stent group: McGoon (p < 0.001), Nakata (p < 0.001). Indexed pulmonary artery diameters increased equally in both groups but remained lower pre-TCPC in the PA-stent group: McGoon (p < 0.001), Nakata (p = 0.009), and Lower Lobe Index (p = 0.003). LPA and RPA grew symmetrically in both groups. Single RV, larger neo-ascending aorta, and small LPA pre- BDCPC are independent risk factors for PA-stent implantation post-BDCPC. Pulmonary artery diameters after PA-stent implantation and stent re-dilatation showed significant growth together with the contralateral side, but the PA-system remained symmetrically smaller in the stent group.

Risk Factors and Outcomes of Children with Congenital Heart Disease on Extracorporeal Membrane Oxygenation-A Ten-Year Single-Center Report.

For children born with congenital heart defects (CHDs), extracorporeal life support may be necessary. This retrospective single-center study aimed to investigate the outcomes of children with CHDs on extracorporeal membrane oxygenation (ECMO), focusing on various risk factors. Among the 88 patients, 36 (41%) had a single-ventricle heart defect, while 52 (59%) had a biventricular defect. In total, 25 (28%) survived, with 7 (8%) in the first group and 18 (20%) in the latter. A p-value of 0.19 indicated no significant difference in survival rates. Children with biventricular hearts had shorter ECMO durations but longer stays in the intensive care unit. The overall rate of complications on ECMO was higher in children with a single ventricle (odds ratio [OR] 1.57, 95% confidence interval [CI] 0.67-3.7); bleeding was the most common complication in both groups. The occurrence of a second ECMO run was more frequent in patients with a single ventricle (22% vs. 9.6%). ECMO can be effective for children with congenital heart defects, including single-ventricle patients. Bleeding remains a serious complication associated with worse outcomes. Patients requiring a second ECMO run within 30 days have lower survival rates.

Exceptional case of aortic atresia associated with atypical double aortic arch.

We present the unique case of atretic aortic valve associated with hypoplastic ascending aorta and double aortic arch of unusual configuration supplying retrograde blood flow to the coronary arteries.

White matter microstructure and executive functions in congenital heart disease from childhood to adulthood: A pooled case-control study.

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.

Mental sequelae of the COVID-19 pandemic in children with and without complex medical histories and their parents: well-being prior to the outbreak and at four time-points throughout 2020 and 2021.

The objective of this study is to understand the long-term mental sequelae for families over the course of the COVID-19 pandemic by longitudinally investigating the well-being of children with and without complex medical histories and their parents. Well-being of 200 children (between 7 and 18 years of age; 73 typically developing, 46 born very preterm, 73 with complex congenital heart disease) and 175 of their parents was assessed prior to and during the first (April-May 2020), second (October-November 2020), third (April-May 2021), and fourth wave (October-November 2021) of the pandemic with standardized questionnaires. Linear mixed models were used to investigate longitudinal changes in child and parent well-being compared to before the pandemic. Social and COVID-19-specific determinants were investigated as predictors of impaired well-being. To illustrate clinical relevance, the proportion of children and parents scoring > 1 SD below normative mean/median was reported. Compared to before the pandemic, child proxy-reported well-being was lower during the first but not the second, third, and fourth waves. Child self-reported well-being was not lower during the pandemic compared to before. Parent well-being dropped during the first wave and remained low throughout the subsequent waves. Proxy-reported child and self-reported parent well-being was lower in families with sparse social support and poor family functioning. Parents of typically developing children reported lower well-being than parents of children born very preterm or with a complex congenital heart disease. In November 2021, 20% of children (both self- and proxy-report) and 24% of parents scored below the normal range compared to 11% (child self-report), 10% (child proxy-report), and 16% (parent self-report), respectively, before the pandemic. The pandemic continues to impact the well-being of parents of school-aged children with and without complex medical histories more than 1 year after its outbreak. Children's well-being was specifically affected during the first wave of the pandemic and has recovered thereafter. Families with sparse social support and poor family functioning are particularly at risk for compromised well-being and support should be provided to them.

Congenital heart disease in school-aged children: Cognition, education, and participation in leisure activities.

BACKGROUND: Children with congenital heart disease (CHD) are at risk for neurodevelopmental deficits. This study aimed to investigate the impact of cognitive deficits on educational outcome and participation in leisure activities. METHODS: A prospective cohort of 134 children with CHD who underwent cardiopulmonary bypass surgery (CPB) was examined at 10 years of age. IQ was assessed with the WISC-IV and executive functions with the BRIEF (parent- and teacher-report). Parents reported on type and level of education and educational support, and leisure activity participation. Ordinal regression analyses assessed the association between cognitive deficits and educational outcome and participation. RESULTS: Total IQ (P = 0.023), working memory (P < 0.001), processing speed (P = 0.008), and teacher-reported metacognition (P = 0.022) were lower than norms. Regular school was attended by 82.4% of children with CHD compared to 97% of the general Swiss population (P < 0.001). Seventy-five percent of children participated in leisure activities. Lower total IQ and teacher-rated global executive functions were associated with more educational support and lower IQ was associated with less participation. CONCLUSION: As school-aged children with CHD experience cognitive deficits, follow-up is required to provide optimal support with regard to educational outcome and participation in leisure activities. IMPACT: Contemporary cohorts of children with congenital heart disease undergoing cardiopulmonary bypass surgery remain at increased risk for cognitive deficits. Cognitive deficits affect educational outcome and leisure activities. These findings underline the importance of early detection of cognitive deficits and recommend support with respect to cognitive functioning.

Possible effects of left pulmonary artery stenting in single ventricle patients on bronchial area, lung volume and lung function.

Background: Left pulmonary artery (LPA) stenting is often required in single ventricle (SV) patients. Due to their close anatomical relationship an LPA stent could potentially compress the left main bronchus (LMB). We assessed the impact of LPA stenting on bronchial size, pulmonary volumes, and lung function in a cohort of SV patients. Methods: Forty-nine patients underwent cardiovascular magnetic resonance (CMR) and 36 spirometry 11 (8-15) years after Fontan. All patients were free of respiratory symptoms. LPA stents were inserted in 17 (35%) patients at 8.8 (3.4-12.6) years. Area/shape of the main bronchi (n = 46) and lung volumes (n = 47) were calculated from CMR-ZTE images for each lung and transformed in right-to-left (r/l) ratio and indexed for BSA. The effect of early stent insertion (prior to stage III) was analyzed. Results: Patients with LPA stent had larger r/l ratio for main bronchus area (p < 0.001) and r/l ratio difference for lung volumes was slightly larger in patients with early stenting. A trend toward a deformation of LMB shape in patients with LPA stent and toward a higher prevalence of abnormal spirometry in patients with early stent implantation was observed. Conclusions: In this cohort of patients, early insertion of LPA stents seems to relate with smaller LMB sizes and a trend toward smaller left lung volume and higher prevalence of impaired lung function. Whether these findings are caused by the stent or, at least to a certain degree, present prior to the implantation needs to be verified.

A family-tailored early motor intervention (EMI-Heart) for infants with complex congenital heart disease: study protocol for a feasibility RCT.

BACKGROUND: Children with congenital heart disease (CHD) undergoing open-heart surgery are at risk for developmental impairments with motor delay manifesting first and contributing to parental concerns. Only a few interventional studies aim to improve neuromotor development in infants with CHD with inconclusive results. We thus developed a family-tailored early motor intervention (EMI-Heart), which aims to promote motor development and family well-being in the first year of life after open-heart surgery. The primary aim described in this protocol is to evaluate feasibility of EMI-Heart. The secondary aim is to describe the difference between the intervention and control group in motor outcomes and family well-being at baseline, post-treatment, and follow-up. METHODS: This prospective, parallel single-center feasibility randomized controlled trial (RCT) will compare EMI-Heart with standard of care in infants with complex CHD. Sixteen infants and their families, randomly allocated to EMI-Heart or the control group, will participate within the first 5 months of life. Infants assigned to EMI-Heart will receive early motor intervention for 3 months. The intervention's key is to promote infants' postural control to enhance motor development and partnering with parents to encourage family well-being. Feasibility outcomes will be (a) clinical recruitment rate and percentage of families completing EMI-Heart, (b) average duration and number of sessions, and (c) acceptability of EMI-Heart using a parental questionnaire post-treatment, and descriptive acceptability of EMI-Heart to the pediatric physiotherapist. Secondary outcomes of the intervention and control group will be infants' motor outcomes and questionnaires assessing family well-being at 3-5 months (baseline), at 6-8 months (post-treatment), and at 12 months of age (follow-up). We will evaluate feasibility using descriptive statistics. Non-parametric statistical analysis of secondary outcomes will assess differences between the groups at baseline, post-treatment, and follow-up. DISCUSSION: This feasibility RCT will provide information about a newly developed family-tailored early motor intervention in infants with complex CHD. The RCT design will provide a foundation for a future large-scale interventional trial for infants with CHD after open-heart surgery. TRIAL REGISTRATION: This study protocol (version 1.3, 01.02.2022) was approved by the Cantonal Ethics Commission Zurich (BASEC-Nr. 2019-01,787) and is registered by Clinicaltrials.gov (NCTT04666857).

Favourable Short- to Mid-Term Outcome after PDA-Stenting in Duct-Dependent Pulmonary Circulation.

BACKGROUND: Stenting of patent ductus arteriosus (PDA) is a minimally invasive catheter-based temporary palliative procedure that is an alternative to a surgical shunt in neonates with duct-dependent pulmonary perfusion. METHODS: An observational, single-centre, cross-sectional study of patients with duct-dependent pulmonary perfusion undergoing PDA-stenting as a stage I procedure and an analysis of short- to mid-term follow-up until a subsequent surgical procedure (stage II), with a focus on the interstage course. RESULTS: Twenty-six patients were treated with PDA-stenting at a median (IQR) age of 7 (4-10) days; 10/26 patients (38.5%) (6/10 single pulmonary perfusion) were intended for later univentricular palliation, 16/26 patients (61.5%) (13/16 single pulmonary perfusion) for biventricular repair. PDA diameter was 2.7 (1.8-3.2) mm, stent diameter 3.5 (3.5-4.0) mm. Immediate procedural success was 88.5%. The procedure was aborted, switching to immediate surgery after stent embolisation, malposition or pulmonary coarctation in three patients (each n = 1). During mid-term follow-up, one patient needed an additional surgical shunt due to severe cyanosis, while five patients underwent successful catheter re-intervention 27 (17-30) days after PDA-stenting due to pulmonary hypo- (n = 4) or hyperperfusion (n = 1). Interstage mortality was 8.6% (2/23), both in-hospital and non-procedure-related. LPA grew significantly (p = 0.06) between PDA-stenting and last follow-up prior to subsequent surgical procedure (p = 0.06). RPA Z-scores remained similar (p = 0.22). The subsequent surgical procedure was performed at a median age of 106 (76.5-125) days. CONCLUSIONS: PDA-stenting is a feasible, safe treatment option, with the need for interdisciplinary decision-making beforehand and surgical backup afterwards. It allows adequate body and pulmonary vessel growth for subsequent surgical procedures. Factors determining the individual patient's course should be identified in larger prospective studies.

Balloon angioplasty and stent implantation within 30 days postcongenital heart surgery (CHS) in children.

OBJECTIVES: This study aims to assess balloon angioplasty (BAP) and stent implantation (SI) procedures early after congenital heart surgery (CHS) in children. BACKGROUND: These interventions are considered potential high-risk procedures and often avoided or postponed. METHODS: This is a retrospective, single centre study of all BAP and SI procedures within 30 days after CHS (01/2001 until 01/2021). RESULTS: A total of 127 (96 SI, 31 BAP) procedures were performed in 104 patients at median 6.5 days (interquartile range: 1-15) after CHS. Balloon-to-stenosis ratio and balloon-to-reference vessel ratio were significantly smaller compared to stent-to-stenosis ratio and stent-to-reference vessel ratio (p < .001 and p = .005). There was a greater rise in absolute vessel diameter, greater rise in vessel diameter in relation to the stenosis and vessel diameter in relation to the reference vessel with SI (p < .001, p = .01, and p < .001). Up to 94% SIs fulfilled both success criteria (increase of vessel diameter ≥50% of minimal vessel diameter or achievement ≥75% of the reference vessel diameter). Major adverse events were more frequent in the BAP group (p = .05). Intraprocedural complications were 5/31 (16%) in the BAP group and 13/96 (13%) in the SI group (p = .77). CONCLUSION: BAP and SI procedures within 30 days post-CHS can be performed safely, with a greater stent-to-stenosis ratio and a greater rise in vessel diameter with stent implantation.

Bridging with Veno-Arterial Extracorporeal Membrane Oxygenation in Children: A 10-Year Single-Center Experience.

Veno-arterial extracorporeal membrane oxygenation (V-A ECMO) is frequently used in children with and without congenital heart disease (CHD). This study, of a single-center and retrospective design, evaluated the use and timing of V-A ECMO in a pediatric cohort who underwent V-A ECMO implantation between January 2009 and December 2019. The patients were divided into a pre-/non-surgical group and a post-surgical group. Among the investigated variables were age, gender, weight, duration of ECMO, ECMO indication, and ventricular physiology, with only the latter being statistically relevant between the two groups. A total of 111 children (58 male/53 female), with a median age of 87 days (IQR: 7-623) were supported using V-A ECMO. The pre-/non-surgical group consisted of 59 patients and the post-surgical group of 52 patients. Survival at discharge was 49% for the pre-/non-surgical group and 21% for the surgical group (p = 0.04). Single-ventricle physiology was significant for a worse outcome (p = 0.0193). Heart anatomy still has the biggest role in the outcomes of children on ECMO. Nevertheless, children with CHD can be successfully bridged with ECMO to cardiac operation.

Findings and Outcome of Transcatheter Right Ventricular Endomyocardial Biopsy and Hemodynamic Assessment in Children with Suspected Myocarditis or Cardiomyopathy.

OBJECTIVE: The study objective is assessing findings and outcome in children with suspected cardiomyopathy (CMP) or myocarditis undergoing cardiac catheterization with transcatheter right ventricular endomyocardial biopsy (RV-EMB). METHODS: All consecutive children undergoing cardiac catheterization with RV-EMB for suspected CMP/myocarditis between 2002-2021 were analysed regarding clinical presentation, cardiac biomarkers, periprocedural management, hemodynamic, histological/immunohistological findings, and outcome. RESULTS: Eighty-five RV-EMBs were performed in 81 patients at a median age of 6.8 (IQR 9.9) years and a bodyweight of 20 (32.2) kg. Histological/immunohistological findings of RV-EMB revealed dilated CMP in 10 (12%), chronic myocarditis in 28 (33%), healing myocarditis in 5 (6%), acute myocarditis in 9 (11%), other heart muscle diseases in 23 (27%) (7 restrictive CMP, 5 hypertrophic CMP, 4 toxic/anthracycline-induced CMP, 4 endocardfibroelastosis, 1 arrhythmogenic right ventricular CMP, 1 laminin CMP, 1 haemangioma), no conclusive histology in 7 (8%), and normal histology in 3 (4%) patients. Median LVEDP was 17 mmHg (IQR 9), LAP 15 mmHg (10), and PVR 1.83 (1.87) Wood Units/m2. There were 3 major complications (3%), all patients recovered without any sequelae. At follow-up (median 1153, IQR 1799 days) 47 (59%) patients were alive, 11 (13%) dead, 15 (18%) underwent cardiac transplantation, and 8 (9%) were lost to follow-up. Death/cardiac transplantation occurred within 3 years from RV-EMB. All patients with an acute myocarditis survived. NT-pro-BNP, echo parameters, and invasive hemodynamics correlate independently with death/cardiac transplant. CONCLUSION: Hemodynamic invasive data and morphological findings in RV-EMB complete clinical diagnosis in children with suspected CMP/myocarditis and provide important information for further clinical management.

Similarities and Differences in the Neurodevelopmental Outcome of Children with Congenital Heart Disease and Children Born Very Preterm at School Entry.

OBJECTIVE: To describe the similarities and differences in the neurodevelopmental outcome of children with congenital heart disease (CHD) undergoing cardiopulmonary bypass surgery compared with children born very preterm (VPT) at school entry. STUDY DESIGN: IQ, motor abilities, behavior, and therapy use were assessed in 155 children with CHD as part of a prospective, single-center, longitudinal study, and in 251 children born VPT as part of a national follow-up register at the same center. Group differences were tested using independent t-tests and χ2-tests. Equivalence testing was used to investigate similarities between the groups. RESULTS: Mild (ie, 70 ≤ IQ < 85) and severe intellectual impairments (ie, IQ < 70) occurred in 17.4% and 4.5% of children with CHD compared with 22.1% and 5.5% in children VPT, respectively. Motor and behavioral functions were impaired in 57.0% and 15.3% of children with CHD compared with 37.8% and 11.5% of children born VPT, respectively. Children with CHD had poorer global motor abilities (d = -0.26) and poorer dynamic balance (d = -0.62) than children born VPT, and children born VPT had poorer fine motor abilities than children with CHD (d = 0.34; all P < .023). Peer problems were statistically similar between the groups (P = .020). Therapies were less frequent in children with CHD compared with children born VPT (23.4% vs 40.3%; P < .001). CONCLUSIONS: Children with CHD undergoing cardiopulmonary bypass surgery and children born VPT share an overall risk for neurodevelopmental impairments that manifest in different domains. Despite this, children with CHD receive fewer therapies, indicating a lack of awareness of the neurodevelopmental burden these children face.

An Extremely Rare Congenital Muscle Bundle Crossing the Right Atrial Cavity.

Muscle bundles in the right atrium are an extremely rare congenital anomaly. We report the case of a patient with 2 atrial septal defects and a large muscle bundle crossing the right atrium. Only 3 comparable cases have previously been published. (Level of Difficulty: Intermediate.).

Advanced Imaging and New Cardiac Biomarkers in Long-term Follow-up After Childhood Cancer.

OBJECTIVES: Pathologic ejection fraction (EF), shortening fraction (FS), and standard heart failure biomarkers (high sensitive troponin T and N-terminal brain natriuretic peptide) during follow-up after childhood cancer have been associated with irreversible cardiac damage. We aimed to evaluate strain imaging values by echocardiography and new biomarkers for heart failure with preserved ejection fraction (HFpEF) as potential more sensitive parameters for cardiac deterioration in childhood cancer survivors (CCS). MATERIALS AND METHODS: Prospective study with 50 CCS (median 16.2 y) at a median follow-up of 13 years. In addition to standard echo and laboratory parameters for heart failure, strain measurements and new biomarkers, including myocardial inflammation (interleukin 6), extracellular matrix (ECM) remodeling (C-telopeptide for type I collagen, intact N-terminal propeptide of type III procollagen), and other heart failure biomarkers (galectin 3, solutable ST2, growth differentiation factor 15), were obtained and compared with 50 healthy controls. RESULTS: No significant differences in EF, FS, high sensitive troponin T, N-terminal brain natriuretic peptide, interleukin 6, solutable ST2, and galectin 3 were found between study and control groups. In contrast, strain imaging showed significant differences between both groups (global longitudinal strainGLS -16.1% vs. -20.4%, P<0.0001; global circumferential strain -14.3 vs. -21.4%, P<0.0001), detecting 66% (global longitudinal strain) and 76% (global circumferential strain) of patients with pathologic values in contrast to 6% (EF) and 16% (FS) for standard parameters. Markers for disturbances of ECM remodeling (C-telopeptide for type I collagen, intact N-terminal propeptide of type III procollagen, each P<0.0001) and growth differentiation factor 15 (P<0.0001) were significantly different between the groups. CONCLUSION: Strain imaging and new cardiac biomarkers used in HFpEF focusing on ECM remodeling appear to be more sensitive in detecting early remodeling processes in CCS than standard echo and laboratory parameters.