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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. [ABSTRACT FROM AUTHOR]

Background: This study set out to assess the indications, feasibility, safety, and outcome of early cardiac catheterizations (CC) within 30 days after congenital heart surgery (CHS) in children. Methods and Results: This is a retrospective, single-center case review study of all CC within 30 days after CHS between 1/2010-12/2020. A total of 317 (138 diagnostic, 179 interventional) CC were performed in 245 patients at a median of 4 days (IQR 13) after CHS. The median age was 3 months (IQR 6), and body weight was 5 kg (IQR 4). A total of 194 (61.2%) CC were performed in patients with univentricular hearts. CC revealed significant pathologies leading to early redo-surgery in 37 patients (12%). The transcatheter interventions primarily were needed in patients after cavo-pulmonary connection (n = 69%, 21.8%), right ventricle to pulmonary artery conduit (n = 39%, 12.3%), and Norwood-I surgery (n = 34%, 10.7%) presenting with hypoxemia, prolonged postoperative course, and suspected arterial stenosis on echocardiography. The clinical impact of an early postoperative transcatheter intervention for the following clinical course was high in most cases. There were nine (2.8%) major and 20 (6.3%) minor intra-procedural complications. Risk factor analysis revealed no difference for the occurrence of complications for patients' age, weight, and time from initial CHS, underlying uni- vs. biventricular heart disease, or ECMO. Conclusion: Early CC within 30 days after CHS in children can be performed safely with a high diagnostic and therapeutic value. The rate of complications is low, while the therapeutic consequence is relevant. [ABSTRACT FROM AUTHOR]

Background. Nowadays, transcatheter device closure of an atrial septal defect (ASD) is a standard approach in children. Potential early and long-term side effects or complications related to the metal framework of the devices are a known issue. A bioresorbable device such as the Carag Bioresorbable Septal Occluder™ (CBSO) could resolve such complications. Material and Results. The Carag Bioresorbable Septal Occluder™ (CBSO; Carag AG, Baar, Switzerland) is a self-centering double disk, repositionable, and retractable device with a bioresorbable framework (polylactic-co-glycolic acid), which is almost completely resorbed by 18–24 months postimplantation. This manuscript reports the four first-in-child ASD device closures using a CBSO. The patients' age was median (IQ1-IQ3), 4.5 years (4–7.25). Weight was 21.3 kg (17.6–32.7). We demonstrated procedural feasibility and safety. Effective defect closure with the device was 100%. Echocardiographic measurements of the thickness of the interatrial septum did not show any relevant increase over a 12-monthfollow-up period. There were no residual defects found after the procedure or later during the resorption process. The patients showed no evidence of any local or systemic inflammatory reaction. Conclusions. The CBSO device system could offer a new treatment option for transcatheter ASD device closure in the pediatric and adult fields. In our first-in-child experience, it was effectively and safely implanted. During the first 12 months of follow-up, no complications occurred. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

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This study evaluates coagulation profiles of single ventricle (SV) patients in relationship to liver function, hemodynamic variables and outcome. Twenty-six children with SV anatomy were included. Advanced coagulation profiles, invasive preoperative hemodynamic parameters and clinical course were retrospectively analyzed. Median (interquartile range) age and weight at the time of blood sampling was 25.5 (31) months and 10.5 (6.9) kg. Sixteen patients (16/26; 62%) showed decreased antithrombin and/or protein C (PC) and/or free protein S (PS) function and/or free PS antigen. Two patients showed abnormal activated PC resistance ratio due to heterozygous factor V Leiden mutation and 1 heterozygous prothrombin G20210A mutation. Group comparison (abnormal coagulation profile [group 1; n = 16] versus normal coagulation profile [group 2; n = 10]) showed longer postoperative hospitalization time (p = .04), longer postoperative catecholamine support (p = .01), a higher incidence of thromboembolic events (p = .04), and chylothoraxes (p = .007) in group 1. In 5 (31%) of 16 group 1 patients, thromboembolic complications occurred: cerebral stroke (n = 1), intestinal ischemia (n = 2), thrombus formation in inferior caval vein (n = 1), and pulmonary vein (n = 1). Abnormalities in coagulation parameters are common in SV patients. Coagulation abnormalities constitute a preoperative risk factor and affect postoperative course. [ABSTRACT FROM AUTHOR]

Objective: This study sets out to compare morbidity, mortality and reintervention rates after stenting of the right ventricular outflow tract (RVOT) versus modified Blalock-Taussig shunt (mBTS) for palliation in patients with tetralogy of Fallot (ToF)-type lesions.Methods: Retrospective case review study evaluating 101 patients (64 males) with ToF lesions who underwent palliation with either mBTS (n=41) or RVOT stent (n=60) to augment pulmonary blood flow over a 10-year period. Procedure-related morbidity, mortality and reintervention rates were assessed and compared.Results: Admission rate to paediatric intensive care unit (PICU) was lower in the RVOT stent group (22% vs 100%; p<0.001). Thirty-day mortality in the RVOT stent group was (1/60 (1.7%)) compared with (2/41 (4.9%)) in the mBTS group (p=0.565). Mortality until surgical repair was comparable in both groups (5/60, 8.4%, including three non-cardiac death in the RVOT stent group vs 2/41, 4.9% (p=0.698)). Total hospital length of stay was shorter for the RVOT stent group (median 7 days vs 14 days; p<0.003). Time to surgical repair was shorter in the RVOT stent group (median 232 days, IQR 113-360) compared with the mBTS group (median 428 days, IQR 370-529; p<0.001) due to improved pulmonary arterial growth.Conclusion: RVOT stenting in Fallot-type lesions can be accomplished safely, with lower PICU admission rate, a shorter hospital length of stay and shorter duration of palliation until complete repair compared with mBTS palliation. [ABSTRACT FROM AUTHOR]

Anticoagulation using intravenous bolus administration of unfractionated heparin (UFH) aims to prevent thromboembolic complications in children undergoing cardiac catheterization (CC). Optimal UFH dosage is needed to reduce bleeding complications. We analyzed the effect of bolus UFH on activated clotting time (ACT) in children undergoing CC focusing on age-dependent, anesthesia-related, or disease-related influencing factors. This retrospective single-center study of 183 pediatric patients receiving UFH during CC analyzed ACT measured at the end of CC. After bolus administration of 100 IU UFH/kg body weight, ACT values between 105 and 488 seconds were reached. Seventy-two percent were within target level of 160 to 240 seconds. Age-dependent differences were not obtained (P = .407). The ACT values were lower due to hemodilution (total fluid and crystalloid administration during CC, both P < .001), with premedication of acetylsalicylic acid (P = .014) and low-molecular-weight heparin (P = .049). Arterial thrombosis (3.85%), venous thrombosis (0.55%), and bleeding (1.65%) following CC did not correlate with ACT values but occurred more frequently in children between 1 month and 1 year of age (91%). In conclusion, with a bolus of 100 IU UFH/kg, an ACT target level of 160 to 240 seconds can be achieved during CC in children in 72%, which is influenced by hemodilution and anticoagulant and antiplatelet premedication but not by age. [ABSTRACT FROM AUTHOR]

Aims: This study set out to assess indications, feasibility, complications, and clinical outcome of percutaneous transcatheter device closure of atrial septal defects (ASDs) in infants with a bodyweight below 10 kg.Methods and Results: Retrospective single center chart and echocardiography review study from 8/2005-12/2013. Twenty-eight children with ASD (13 female) with a median age of 1.15 years (0.2-2.8) and a median weight of 7.2 kg (4.5-9.9) were analyzed. Indications for early ASD closure were failure to thrive (n = 15, 54%), bronchopulmonary dysplasia (BPD) with supplemental oxygen dependency (n = 7, 25%), and genetic syndromes with suspected pulmonary hypertension (n = 12, 43%). Device implantation was successful in all patients without any periprocedural mortality or major complication. Clinical outcome after a median follow-up period of 2.1 years (0.25-7.3) revealed no residual shunt and a significant decrease of right ventricular volume load. Patients with pulmonary hypertension experienced a significant reduction of pulmonary artery/RV pressure. Patients also showed decreased supplemental oxygen dependency and less cardiac medications, but no significant "catch-up growth" in those with failure to thrive.Conclusion: Interventional ASD closure in children weighing less than 10 kg can be performed without any additional major risks and shows a favorable outcome, especially in selected patients with significant non cardiac co-morbidities. [ABSTRACT FROM AUTHOR]

Objectives: The aim of this study was to evaluate the effectiveness of balloon angioplasty (BAP) for recurrent aortic coarctation (ReCoA) in infants comparing simple and complex type of aortic arch pathology (Norwood I procedure).Background: ReCoA is a known complication after cardiovascular surgery for coarctation of the aortic arch.Methods and Results: In a single center case study, we analyzed 20 infants undergoing BAP for ReCoA comparing simple (n = 10) and complex type of aortic arch pathology (n = 10). At catherization diameter of ReCoA stenosis was 3.2 ± 0.7 mm (mean ± SD) with short localized (11/20) or long hypoplastic stenosis (9/20) before and 4.8 ± 1.2 mm after BAP (P < 0.001). Invasive systolic pressure gradient was reduced from 27.5 ± 16.2 mmHg before to 5.1 ± 6.6 mmHg after BAP (P < 0.001), comparable in simple and complex type of ReCoA. At day 1 after catherization noninvasive systolic arterial blood pressure gradient was reduced from 20.2 ± 23.1 to 6.7 ± 9.9 mmHg (P < 0.001), respectively, calculated continuous wave Doppler echo gradient from 36.3 ± 22 to 16.8 ± 9.6 mmHg (P < 0.01). Complications were aortic arch dissection (1/20), discrete aortic arch aneurysm formation (1/20), pericardial effusion (1/20), and peripheral arterial thrombosis (4/20). At a mid-term follow up of 9.5 months (1-40) after BAP, 3 infants needed early surgical reintervention due to secondary ReCoA, all with long hypoplastic aortic arch segments, but simple type of aortic arch pathology.Conclusions: At mid-term follow up, BAP remains an effective catheter intervention for ReCoA for infants with localized ReCoA, but not with long hypoplastic aortic arch segments, even in simple type of aortic arch pathology. [ABSTRACT FROM AUTHOR]

Abstract Background Stent implantation into growing vessels is a common treatment option in infants and children with congenital heart disease (CHD) and corresponding vessel lesions. After stent implantation in small children, repetitive stent redilations are frequently necessary to accommodate for somatic growth. Until now, all available stents have limited final expansion diameters. Material and results The new Bentley BeGrow™ stent system for newborns and infants is a L605 cobalt‑chromium, pre-mounted, balloon expandable stent, which is compatible with a 4 French sheath and 0.014 inch guide wire thus allowing implantation in small vessels (4–6 mm). It offers a new, unique stent design that allows post-dilation steps up to Ø11.5 mm. While re-dilating up to Ø11.5 mm this new stent maintains radial force and shows uniform expansion with only minimal foreshortening. Predetermined breaking points allow the stent struts to break in a controlled manner when exceeding a diameter of 11.5 mm. Residual radial force maintains even after stent opening due to spiral arrangement of the predetermined breaking points. The 2 first-in-man pulmonary artery stent implantations in a newborn with univentricular circulation and a toddler with biventricular circulation are reported as part of the currently performed licencing trial (ClinicalTrials.gov NCT03287024). Conclusion The low-profile BeGrow™ stent system offers new treatment options for transcatheter stent implantations in newborns and infants. In our first experience, it can be effectively implanted. Longer follow-up will evaluate multiple, stepwise redilations and controlled stent strut breakage, which have the potential to accommodate for somatic vessel growth and/or subsequent implantation of larger stents. Highlights • Repetitive stent redilations in children with CHD are frequently necessary to accommodate for somatic growth. • The Bentley BeGrow™ stent system offers a stent design allowing post-dilation steps up to Ø11.5mm to follow vessel growth. • Predetermined breaking points allow the stent struts to break in a controlled manner when exceeding a diameter of 11.5mm. • The BeGrow™ stent system offers new treatment options for transcatheter stent implantations in newborns and infants. • It can be effectively implanted. Longer follow-up will evaluate redilations and controlled stent strut breakage. [ABSTRACT FROM AUTHOR]

The European Pediatric Pulmonary Vascular Disease Network is a registered, non-profit organization that strives to define and develop effective, innovative diagnostic methods and treatment options in all forms of pediatric pulmonary hypertensive vascular disease, including pulmonary hypertension (PH) associated with bronchopulmonary dysplasia, PH associated with congenital heart disease (CHD), persistent PH of the newborn, and related cardiac dysfunction. The executive writing group members conducted searches of the PubMed/MEDLINE bibliographic database (1990–2018) and held face-to-face and web-based meetings. Ten section task forces voted on the updated recommendations, based on the 2016 executive summary. Clinical trials, meta-analyses, guidelines, and other articles that include pediatric data were searched using the term "pulmonary hypertension" and other keywords. Class of recommendation (COR) and level of evidence (LOE) were assigned based on European Society of Cardiology/American Heart Association definitions and on pediatric data only, or on adult studies that included >10% children or studies that enrolled adults with CHD. New definitions by the World Symposium on Pulmonary Hypertension 2018 were included. We generated 10 tables with graded recommendations (COR/LOE). The topics include diagnosis/monitoring, genetics/biomarkers, cardiac catheterization, echocardiography, cardiac magnetic resonance/chest computed tomography, associated forms of PH, intensive care unit/lung transplantation, and treatment of pediatric PH. For the first time, a set of specific recommendations on the management of PH in middle- and low-income regions was developed. Taken together, these executive, up-to-date guidelines provide a specific, comprehensive, detailed but practical framework for the optimal clinical care of children and young adults with PH. [ABSTRACT FROM AUTHOR]