Drowning is one of the leading causes of accidental deaths in children worldwide. However, the use of long-term extracorporeal life support (ECLS) in this setting is not widely established, and rewarming is often achieved by short-term cardiopulmonary bypass (CPB) treatment. Thus, we sought to add our experience with this means of support as a bridge-to-recovery or to-decision. This retrospective single-center study analyzes the outcome of 11 children (median 23 months, minimum-maximum 3 months-6.5 years) who experienced drowning and subsequent cardiopulmonary resuscitation (CPR) between 2005 and 2016 and who were supported by veno-arterial extracorporeal membrane oxygenation (ECMO), CPB, or first CPB then ECMO. All but one incident took place in sweet water. Submersion time ranged between 10 and 50 minutes (median 23 minutes), water temperature between 2 degrees C and 28 degrees C (median 14 degrees C), and body core temperature upon arrival in the emergency department between 20 degrees C and 34 degrees C (median 25 degrees C). Nine patients underwent ongoing CPR from the scene until ECMO or CPB initiation in the operating room. The duration of ECMO or CPB before successful weaning/therapy withdrawal ranged between 2 and 322 hours (median 19 hours). A total of four patients (36%) survived neurologically mildly or not affected after 4 years of follow-up. The data indicate that survival is likely related to a shorter submersion time and lower water temperature. Resuscitation of pediatric patients after drowning has a poor outcome. However, ECMO or CPB might promote recovery in selected cases or serve as a bridge-to-decision tool.

In Fontan patients, any pulmonary stenosis may impede free passive inflow into the pulmonary circuit and elevate central venous pressure. When stenting such pulmonary stenosis, dislocation of a stent is a feared complication. Here, we report on a successful retrieval of a dislocated Cook Formula stent into the peripheral left pulmonary artery in a Fontan patient with protein-losing enteropathy using a steerable sheath and a grasping forceps. Furthermore, a successful stent implantation for treating the stenosis was possible. Subsequently, we simulated the retraction technique ex vivo. To manage stent dislocation during complex pediatric catheter interventions, we can recommend the use of a steerable sheath guiding a grasping forceps. A dislocated stent can be grasped, completely longitudinally refolded, and safely retrieved.

ObjectivesDelayed sternal closure is a routine procedure to reduce hemodynamic and respiratory instability in pediatric patients following cardiac surgery, particularly in neonates and infants. In this setting, the possible links between sternal wound infection and delayed sternal closure are still a matter of debate. As a part of our routine, there was a low threshold for delayed sternal closure, so we reviewed our experience with sternal wound infections with a focus on potentially related perioperative risk factors, particularly delayed sternal closure.MethodsWe retrospectively identified 358 operated neonates (37%) and infants (mean age 3.6 months) in our local congenital heart disease database between January 2013 and June 2017. Potential risk factors for sternal wound infections, such as age, gender, complexity (based on Aristotle- and STS-EACTS mortality category), reoperation, use of cardiopulmonary bypass, extracorporeal membrane oxygenation, mortality and delayed sternal closure (163/358, 46%), were subjected to uni- and multivariate analysis.ResultsA total of 26/358 patients (7.3%) developed a superficial sternal wound infection. There were no deep sternal wound infections, no mediastinitis or sepsis. Applying univariate analysis, the prevalence of sternal wound infections was related to younger age, more complex surgery and delayed sternal closure. However, in multivariate analysis, sternal wound infection was only associated with delayed sternal closure (p = 0.013, odds ratio 8.6). Logistic regression revealed the prevalence of delayed sternal closure to be related to younger age, complexity, and the use of extracorporeal membrane oxygenation.ConclusionIn patients younger than one year, sternal wound infections are clearly related to delayed sternal closure. However, in our cohort, all sternal wound infections were superficial and acceptable, considering the improved postoperative hemodynamic stability.

Ebstein's anomaly is a rare congenital heart disease with malformation of the tricuspid valve and myopathy of the right ventricle. The septal and inferior leaflets adhere to the endocardium due to failure of delamination. This leads to apical displacement of their hinge points with a shift of the functional tricuspid valve annulus towards the right ventricular outflow tract with a possibly restrictive orifice. Frequently, a coaptation gap yields tricuspid valve regurgitation and over time the "atrialized" portion of the right ventricle may dilate. The highly variable anatomy determines the clinical presentation ranging from asymptomatic to very severe with need for early operation. Echocardiography and magnetic resonance imaging are the most important diagnostic modalities to assess the tricuspid valve as well as ventricular morphology and function. While medical management of asymptomatic patients can be effective for many years, surgical intervention is indicated before development of significant right ventricular dilatation or dysfunction. Onset of symptoms and arrhythmias are further indications for surgery. Modified cone reconstruction of the tricuspid valve is the state-of-the-art approach yielding the best results for most patients. Alternative procedures for select cases include tricuspid valve replacement and bidirectional cavopulmonary shunt depending on patient age and other individual characteristics. Long-term survival after surgery is favorable but rehospitalization and reoperation remain significant issues. Further studies are warranted to identify the optimal surgical strategy and timing before adverse right ventricular remodeling occurs. It is this article's objective to provide a comprehensive review of current literature and an overview on the management of Ebstein's Anomaly. It focuses on imaging, cardiac surgery, and outcome. Additionally, a brief insight into arrhythmias and their management is given. The "future perspectives" summarize open questions and fields of future research.

INTRODUCTION:Veno-arterial extracorporeal membrane oxygenation is well-established for pediatric patients with post-cardiotomy heart failure. However, extracorporeal membrane oxygenation support is associated with major complications, that is, hemorrhage and thromboembolism. We seek to report our experience with delayed systemic heparinization during neonatal cardiac extracorporeal membrane oxygenation and its impact on bleeding and thromboembolism.METHODS:We retrospectively identified 15 consecutive neonates who were placed on extracorporeal membrane oxygenation after congenital heart surgery during a period of 3 years (2015-2017). Our anticoagulation protocol consisted of full heparin reversal by protamine after switching from cardiopulmonary bypass to extracorporeal membrane oxygenation (target activated clotting time: 120 ± 20 seconds). Administration of systemic heparinization was delayed until postoperative drainage volume declined to <1 mL/kg/h. Primary study endpoints were thromboembolism, bleeding, and requirement of blood products on extracorporeal membrane oxygenation.RESULTS:Our cohort (mean age: 13 ± 2.6 days; mean weight: 3.1 ± 0.3 kg; 66.7% male) required post-cardiotomy extracorporeal membrane oxygenation with a mean support time of 4.5 ± 2.2 days. Systemic heparinization was delayed averagely for 18.1 ± 9.3 hours. No thromboembolic events were observed on extracorporeal membrane oxygenation or after weaning. Relevant surgical site bleeding occurred in two patients (13.3%) requiring re-thoracotomy on the first postoperative day. Analysis of transfusion volumes revealed 24.5 ± 21.9 mL/kg/d mean packed red blood cells, 9.6 ± 7.1 mL/kg/d mean fresh frozen plasma, and 7.5 ± 5.7 mL/kg/d mean platelets. In-hospital survival was 86.6% (n = 13).CONCLUSION:In this retrospective analysis, the results of delayed systemic heparinization in neonatal post-cardiotomy extracorporeal membrane oxygenation could lead one to conclude that this routine is safe and favorable with low risk for thromboembolic events, reduced postoperative hemorrhage, and reduced blood product utilization.

Several activity interventions in preschool settings exist, but little attention has been paid to effects on hemodynamic factors. The study aimed to assess the effectiveness of an exercise program on health‐related outcomes including blood pressure (BP) and markers of vascular function in preschoolers, with focus on socioeconomic background. This study is a cluster‐randomized controlled trial, with preschool as unit of randomization and children as unit of analysis. Preschools with 3‐ to 6‐year‐old children, stratified by social area, were randomly allocated to: intervention (three clusters, n = 92) including 2 d·wk−1/45 min (6 months) exercise lessons or control (two clusters, n = 43). In total, 135 children (4.8 ± 0.8 y) had minimum one outcome measurement at baseline and follow‐up. Primary outcome: peripheral BP. Secondary outcomes: central BP, pulse wave velocity (PWV), BMI, waist circumference, physical activity measures, motor skills. Maternal education was used as an indicator of socioeconomic status. Mixed models were applied to evaluate differences in mean change. Group allocation had no effect on primary or secondary outcomes. However, the intervention was effective in reducing increases in peripheral systolic BP (−3.4 mm Hg; 95% CI: −6.6; −0.2; P = 0.037), central systolic BP (−3.8 mm Hg; −6.4; −1.1; P = 0.006), and PWV (−0.1 m/s; −0.2; −0.0; P = 0.045) among children whose mothers had the lowest educational level. We found no evidence for effectiveness of a 6‐months preschool‐based exercise program on hemodynamics, anthropometrics, activity, or motor skills, but lack of process evaluations and poor fidelity preclude interpretation of the causal relation. However, the results indicate that children from lower social backgrounds could benefit from early exercise‐promoting interventions.

Background Ultra-high density mapping (HDM) is a promising tool in the treatment of patients with complex arrhythmias. In adults with congenital heart disease (CHD), rhythm disorders are among the most common complications but catheter ablation can be challenging due to heterogenous anatomy and complex arrhythmogenic substrates. Here, we describe our initial experience using HDM in conjunction with novel automated annotation algorithms in patients with moderate to great CHD complexity. Methods We studied a series of consecutive adult patients with moderate to great CHD complexity and an indication for catheter ablation due to symptomatic arrhythmia. HDM was conducted using the Rhythmia™ mapping system and a 64-electrode mini-basket catheter for identification of anatomy, voltage, activation pattern and critical areas of arrhythmia for ablation guidance. To investigate novel advanced mapping strategies, postprocedural signal processing using the Lumipoint™ software was applied. Results In 19 patients (53±3 years; 53% male), 21 consecutive ablation procedures were conducted. Procedures included ablation of atrial fibrillation (n=7; 33%), atrial tachycardia (n=11; 52%), atrioventricular accessory pathway (n=1; 5%), the atrioventricular node (n=1; 5%) and ventricular arrhythmias (n=4; 19%). A total of 23 supraventricular and 8 ventricular arrhythmias were studied with the generation of 56 complete high density maps (atrial n=43; ventricular n=11, coronary sinus n=2) and an average of 12,043±1,679 mapping points. Multiple arrhythmias were observed in n=7 procedures (33% of procedures; range of arrhythmias detected 2-4). A total range of 1-4 critical areas were defined per procedure and treated within a radiofrequency application time of 16 (interquartile range 12-45) minutes. Postprocedural signal processing using Lumipoint™ allowed rapid annotation of fractionated signals within specific windows of interest. This supported identification of a practical critical isthmus in 20 out of 27 completed atrial and ventricular tachycardia activation maps. Conclusions Our findings suggest that HDM in conjunction with novel automated annotation algorithms provides detailed insights into arrhythmia mechanisms and might facilitate tailored catheter ablation in patients with moderate to great CHD complexity.

Aortic root dilatation and its complications are known to be the most important and life limiting features in patients with Marfan syndrome (MFS). Since monitoring of patients, preventive medical and surgical treatments are available nowadays, other MFS pathologies are becoming more relevant for the outcome of the disease. Main pulmonary artery (MPA) dilatation is a cardiac finding, which has not been fully investigated in children. Due to the similarities in tissue composition of the aortic and pulmonary root, MPA dilatation may cause complications and require treatment. In addition, it may be a predictor for severe connective tissue involvement. We retrospectively examined 135 pediatric patients with MFS. 8.1% showed MPA dilatation. MPA dilatation was associated with earlier occurrence of aortic dilatation, mitral valve prolapse, and systemic manifestations of MFS compared with patients without MPA dilatation (p < 0.05). The presence of MPA dilatation was also associated with a higher incidence of ectopia lentis (p < 0.05). Medical treatment was started earlier in MPA dilatation patients than in those without (p < 0.05). We conclude that MPA dilatation is a sign of more severe vascular and connective tissue involvement. Regular examination of the pulmonary artery is essential in MFS to avoid complications. As medical treatment of life threatening MFS events has improved, other features of MFS need to be investigated to improve quality of life.

Objectives: Today medical care for chronic diseases like Marfan syndrome (MFS) is a big challenge. Thereby long-term outcome and quality of life (QoL) are essential and highest aim for patients. Early diagnosis, due to improvement of genetics and clinical judgment, allows insights of development of disease and possible preventive strategies in childhood. In consequence, all patients require lifelong follow-up examinations and need an effective transition to adult medicine. We hereby present more than 10 years of follow up with a distinguish insight of all aspects of diagnosis and prophylaxis in MFS.

AIMS:Endomyocardial biopsies (EMBs) are performed infrequently in children owing to significant past complication rates and controversial discussions about the therapeutic value of results. The objective of this study was to investigate the safety and feasibility of EMBs for suspected myocardial disease in relation to their clinical value.METHODS AND RESULTS:We performed a retrospective multicentre review of the Working Group for Interventional Cardiology of the German Society for Paediatric Cardiology. During three consecutive years, 206 EMBs (84 female/mean age 8.95±6.62 years) were performed and analysed at 15 heart centres. In the majority of cases, biopsies were taken from the right ventricle (RV/89.8%; p<0.001). The overall complication rate was 9.7%, whereas major complications occurred in only 0.97% of cases. Risk factors associated with a higher complication rate were biopsy during the first year of life (20.5%) and from the left ventricle (31.1%) (p<0.05). There was no procedure-related mortality. Treatment was changed in 18.0% of cases based on biopsy results.CONCLUSIONS:Today, endomyocardial biopsies in older children with suspected myocardial disease can be performed safely with a low risk of major complications and mortality, whereas the risk of complications if the biopsy is carried out in the first year of life or taken from the left ventricle remains high.

Objectives: The development of most pathologies in Marfan syndrome (MFS) is age dependent and thus not helpful for prediction of clinical outcome. Bicuspid aortic valve (BAV) is the most common congenital heart disease (2%). It is correlated with MFS and prevalence is up to 4.7% in adult patients with MFS. Nevertheless data in childhood about appearance and relevance for severity of phenotype is missing. Subsequently it may be a predictor for later onset of pathologies and helpful to determine individual patient care. We hereby evaluated correlation of appearance of BAV with other cardiovascular and systemic pathologies of MFS in childhood.

The aim of this registry is to provide data on age-related clinical features of suspected myocarditis and to create a study platform allowing for deriving diagnostic criteria and, at a later stage, testing therapeutic interventions in patients with myocarditis. STUDY DESIGN AND RESULTS:After an initial 6-month pilot phase, MYKKE was opened in June 2014 as a prospective multicenter registry for patients from pediatric heart centers, university hospitals, and community hospitals with pediatric cardiology wards in Germany. Inclusion criteria consisted of age<18 years and hospitalization for suspected myocarditis as leading diagnosis at the discretion of the treating physician. By December 31, 2015, fifteen centers across Germany were actively participating and had enrolled 149 patients. Baseline data reveal 2 age peaks (<2 years, >12 years), show higher proportions of males, and document a high prevalence of severe disease courses in pediatric patients with suspected myocarditis. Severe clinical courses and early adverse events were more prevalent in younger patients and were related to severely impaired leftventricular ejection fraction at initial presentation. SUMMARY:MYKKE represents a multicenter registry and research platform for children and adolescents with suspected myocarditis that achieve steady recruitment and generate a wide range of real-world data on clinical course, diagnostic workup, and treatment of this group of patients. The baseline data reveal the presence of 2 age peaks and provide important insights into the severity of disease in children with suspected myocarditis. In the future, MYKKE might facilitate interventional substudies by providing an established collaborating network using common diagnostic approaches.

In August 2010, the Nit-Occlud® Lê (EUREVECO) became available for transcatheter coil occlusion of ventricular septal defects (VSDs). Retrospective European Registry for VSD Closure using the Nit-Occlud® Lê-VSD-Coil; analysis of the feasibility, results, safety and follow-up of VSD-closure over a 3-year period in 18 European centers. In 102 of 111 patients (female 66), successful VSD closure was performed (mean age 8.2 years, mean weight 28.82 kg), 81 perimembranous VSDs (48 with aneurysm), 30 muscular VSDs, mean procedure time was 121.1 min, and mean fluoroscopy time was 26.3 min. Short- and midterm term follow-up was possible in 100/102 patients, there was 1 embolization and 1 explantation after 24 months. Immediate complete closure occurred in 49 of 101 patients (48.5%), trivial residual shunt was present in 51 (50.0%), closure rate was 95% after 6 months and 97% after 1 year. Out of the 102 patients, there were 2 severe complications (1.8%) (1 severe hemolysis, 1 embolization) and 8 moderate/transient (=7.2%) including 1 transient AV block. During a mean follow-up period of 31.3 months (range 24–48) and a total follow-up time of 224.75 patient years, no further problems occurred. VSD closure with the Nit-Occlud® Lê VSD coil is feasible and safe with a minimal risk of severe side effects. The long-term effects and safety require further clinical follow-up studies.

Marfan syndrome (MFS) is a progressive, life-threatening genetic disorder of the connective tissue, which causes impaired quality of life (QoL) in adults. This study investigated the quality of life in children and adolescents, taking into account their gender, age and how MFS affected their organs. This prospective nonrandomised single-centre study included 46 patients with verified MFS with a mean age of 10.98 years (±3.72). QoL was measured using the self-reported, multidimensional KINDL-R questionnaire and compared with an age-matched control group of 174 children and adolescents. No significant overall reduction of QoL was found. Total QoL scores for patients diagnosed at four to seven years were the same as the control group (77.65 ± 9.37 versus 77.06 ± 11.72), but they were higher for patients aged eight to 16 years (75.15 ± 9.19 versus 70.46 ± 11.35, p = 0.025). No gender-specific differences or impairments in QoL during adolescence were observed (p > 0.05). Analysis of the effect of organ manifestation on QoL showed better or equal QoL scores (p > 0.05), despite distinctive phenotypes such as ectopia lentis. QoL was fairly good in paediatric patients with MFS, and there was no impairment during adolescence. Despite the distinctive phenotype, quality of life was unimpaired in younger patients.

BACKGROUND:Cardiovascular biomarkers might help to identify fetuses or pregnancies at risk. AIM:To examine the umbilical cord neonatal and maternal levels of cardiovascular biomarkers at the time of delivery, and to correlate maternal and fetal biomarker levels to each other, to gestational age and to delivery mode. STUDY DESIGN:In a prospective, observational, cross-sectional, single-center study biomarkers were measured in paired maternal and umbilical venous cord blood samples. SUBJECTS:The sample cohort included 66 sets of fetal and maternal blood samples (11 after multiple gestation, 53 after cesarean section, 17 after exposure to labor). OUTCOME MEASURES:Midregional pro-adrenomedullin (MRproADM), midregional-pro atrial natriuretic peptide (MRproANP), brain natriuretic peptide (BNP), n-terminal-pro brain natriuretic peptide (NTproBNP), copeptin, and high sensitive troponin I (hsTnI) levels were measured. RESULTS:Mean ± SEM for biomarker levels in umbilical venous/maternal blood were: MRproADM [nmol/L] 1.02 ± 0.04/1.24 ± 0.08, MRproANP [pmol/L] 215.53 ± 12.96/54.65 ± 3.41, BNP [pg/mL] 32.02 ± 3.37/19.76 ± 3.29, NTproBNP [pg/mL] 1228.94 ± 91.73/71.48 ± 8.65, copeptin [pmol/L] 103.42 ± 22.89/10.41 ± 1.71, and hsTnI [pg/mL] 13.54 ± 5.17/4.91 ± 2.37. Fetal MRproANP, NTproBNP, and BNP were inversely correlated with gestational age. Maternal and fetal MRproANP (r=0.472, p=0.002) and copeptin (r=0.572, p<0.001) levels were correlated, whereas there was no feto-maternal correlation for the other biomarkers. Fetal copeptin was elevated after exposure to labor. CONCLUSIONS:Biomarker levels appear to be regulated independently in mother and fetus. Fetal biomarkers are influenced by gestational age and delivery mode. In this study on term and near term pregnancies without specific fetal pathology, correlation between paired maternal and fetal biomarker levels was weak or not demonstrable.

AimMarfan syndrome (MFS) is a progressive, life-threatening genetic disorder of the connective tissue, which causes impaired quality of life (QoL) in adults. This study investigated the quality of life in children and adolescents, taking into account their gender, age and how MFS affected their organs.MethodsThis prospective nonrandomised single-centre study included 46 patients with verified MFS with a mean age of 10.98 years (3.72). QoL was measured using the self-reported, multidimensional KINDL-R questionnaire and compared with an age-matched control group of 174 children and adolescents.ResultsNo significant overall reduction of QoL was found. Total QoL scores for patients diagnosed at four to seven years were the same as the control group (77.65 +/- 9.37 versus 77.06 +/- 11.72), but they were higher for patients aged eight to 16 years (75.15 +/- 9.19 versus 70.46 +/- 11.35, p = 0.025). No gender-specific differences or impairments in QoL during adolescence were observed (p > 0.05). Analysis of the effect of organ manifestation on QoL showed better or equal QoL scores (p > 0.05), despite distinctive phenotypes such as ectopia lentis.ConclusionQoL was fairly good in paediatric patients with MFS, and there was no impairment during adolescence. Despite the distinctive phenotype, quality of life was unimpaired in younger patients.

Objectives: Due to a risk of dynamic dilatation of sinus of Valsalvae (SV) a prophylactic medical treatment is suggested also in pediatric patients with Marfan Syndrome (MFS). Based on pathogenesis of MFS with increasing transforming growth factor-β (TGFß) signaling, prophylactic medication with angiotensin II type 1 receptor blockers (ATII) was expected to be a treatment with high potential. Now first published large randomized trials demonstrate failing of ATII being superior to prophylaxis with beta-blockers (BB). In childhood dose of treatment with ATII is variable and dose related effect on aortic root dilatation in children has not been investigated yet. High dose of ATII may show a significant impact on growth of SV and could then be superior to BB treatment.

Pentalogy of Cantrell (PoC) is a rare congenital midline defect. We present a case and its treatment of PoC with complete ectopia cordis and congenital heart disease. Postnatally the congenital heart defect was surgically corrected and the ectopic heart was covered by musculous mobilized flap. Due to cephalic orientation of the heart and limited intrathoracic space, replacement of the heart into the thoracic cavity was initially not performed. After 11 years of follow up our patient now is without relevant limitations solely wearing a thoracic shelter. This case elucidates the complexity of further management. The potential risk of disastrous hemodynamic compromise by intrathoracic shift is to compare with the limited safety of the ectopic heart.