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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]

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]

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]

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]

Background: Long-term survival of patients with a single ventricle palliated with a Fontan procedure is still limited. No curative treatment options are available. To investigate the pathophysiology and potential treatment options, such as mechanical circulatory support (MCS), appropriate large animal models are required. The aim of this review was to analyze all full-text manuscripts presenting approaches for an extracardiac total cavopulmonary connection (TCPC) animal model to identify the feasibility and limitations in the acute and chronic setting., Methods: A literature search was performed for full-text publications presenting large animal models with extracardiac TCPCs on Pubmed and Embase. Out of 454 reviewed papers, 23 manuscripts fulfilled the inclusion criteria. Surgical procedures were categorized and hemodynamic changes at the transition from the biventricular to the univentricular condition analyzed., Results: Surgical procedures varied especially regarding coronary venous flow handling and anatomic shape of the TCPC. In most studies (n = 14), the main pulmonary artery was clamped and the coronary venous flow redirected by additional surgical interventions. Only in five reports, the caval veins were connected to the right pulmonary artery to create a true TCPC shape, whereas in all others (n = 18), the veins were connected to the main pulmonary artery. An elevated pulmonary vascular resistance was identified as a limiting hemodynamic factor for TCPC completion in healthy animals., Conclusions: A variety of acute TCPC animal models were successfully established with and without MCS, reflecting the most important hemodynamic features of a Fontan circulation; however, chronic animal models were not reported.

Objective: To find risk factors for a complicated early postoperative course after arterial switch operation (ASO) in neonates with d-transposition of the great arteries (dTGA). In addition to anatomical and surgical parameters, the predictive value of early postoperative troponin T (TnT) values in correlation to the early postoperative course after ASO is analyzed., Methods: Seventy-nine neonates (57 (72%) male) with simple dTGA treated by ASO between 2009 and 2016 were included in the analysis. A complicated early postoperative course (30 days) was defined by one of the following criteria: (A) moderate to severe cardiac dysfunction without rhythm disturbances, (B) rhythm disturbances causing hemodynamic instability with the need for medical treatment, (C) signs for ischemia in ECG, (D) need for surgical or catheter interventional reinterventions other than diagnostic, or (E) other reasons., Results: Forty of 79 patients (51%) showed a complicated early postoperative course after ASO, with 2 patients dying after 13 and 16 days. Patients with a complicated early postoperative course had a longer PICU stay (P < .001), needed longer mechanical ventilator support (P = .001) and longer inotropic support (P = .03), and more reinterventions (surgical or catheter interventional) were necessary (P = .001). Only the presence of a VSD (P = .001) and longer surgery duration (P = .026) were associated to a complicated postoperative course. TnT values only showed a trend toward higher values in patients with a complicated postoperative course (P = .06). A secondary rise in TnT was seen in 10 patients, ranging from 11.6% to 410.2%, of whom 7 could be classified in the complicated postoperative group., Conclusions: The postoperative course after ASO in dTGA neonates is influenced by other cardiac comorbidities like a VSD with the need for surgical treatment, influencing surgery duration. Postoperative higher TnT values reflect a longer and more vulnerable intraoperative course with limited predictive value on the early postoperative course., (© 2018 Wiley Periodicals, Inc.)

Introduction: There is growing evidence that interleukin 17 (IL-17) producing T cells are involved in the pathogenesis of systemic lupus erythematosus (SLE). Previous studies showed that increased percentages of T-cell subsets expressing the costimulatory molecules CD80 and CD134 are associated with disease activity and renal involvement in SLE. The aim of this study was to investigate the distribution and phenotypical characteristics of IL-17 producing T-cells in SLE, in particular in patients with lupus nephritis, with emphasis on the expression of CD80 and CD134., Methods: Thirty-four patients (3 male, 31 female, mean age 41 ± 15 years) fulfilling at least four of the American College of Rheumatology (ACR) revised criteria for the diagnosis of SLE and 24 healthy controls were enrolled. T-cells from the peripheral blood were analysed by fluorescence activated cell sorting (FACS) for their expression levels of CD80, CD134 and CCR6. In vitro stimulated CD3+IL17+ cells were also investigated for the expression of these costimulatory markers. Finally, renal biopsies from SLE patients were evaluated for the presence of CD134 expressing T-cells., Results: Percentages of IL-17 expressing T-cells were significantly increased in patients with active disease as compared to healthy controls (1.46 ± 0.58% versus 0.93 ± 0.30%, P = 0.007). The percentage of IL-17 producing T-cells was correlated with disease activity as assessed by systemic lupus erythematosus disease activity index (SLEDAI) (r = 0.53, P = 0.003). In patients, most of the IL-17 producing T-cells were confined to the CCR6+ T-cell subset (80 ± 13%). Expression of CD80 and CD134 on the IL-17 producing T-cell subset was higher in SLE than in healthy controls (HC) (CD134: 71.78 ± 14.51% versus 51.45 ± 16.58%, P = 0.002; CD80: 25.5 ± 14.99% versus 14.99 ± 5.74%, P = 0.02). Also, patients with lupus nephritis expressed higher levels of CD134+ on CD3+IL-17+ cells as compared to HC (72.69 ± 11.54% versus 51.45 ± 16.58%, P = 0.006). Furthermore, renal biopsies of lupus nephritis patients showed infiltration of CD134+ T cells., Conclusions: Percentages of IL-17 expressing T-cells correlate with disease activity. Further, these cells show increased expression of costimulatory markers such as CD134 and CD80. The presence of CD134+ T-cells in renal biopsies of lupus nephritis patients suggest that these cells migrate to the kidney and might contribute to inflammatory processes through IL-17 secretion.

Objectives: Accurate tube placement of orogastric and nasogastric feeding tubes in neonates is important to ensure safe and effective enteral feeding. Errors in placement and position of feeding tubes are described in literature, but there is little evidence of the exact prevalence of improperly placed tubes, especially in neonates., Materials and Methods: To evaluate the prevalence of improperly placed feeding tubes, we reviewed 381 consecutive radiographs and defined the position of the feeding tubes., Results: We found that in 41% of the placements, the optimal position of the feeding tubes was achieved, whereas overall 59% of the feeding tubes had been placed incorrectly., Conclusions: There is a need for both better rules to measure the distance between nose (lips) and the body of the stomach and improved methods to confirm correct tube position in neonates.