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Stroke, thromboembolism, and bleeding are the most recognized complications associated with pediatric ventricular assist devices (VADs) and the leading cause of death and disability on VAD support. Recently, newer antithrombotic strategies like bivalirudin have emerged that appear to be associated with a reduction in the neurologic event rates, especially for smaller pediatric-specific VADs like the Berlin Heart and PediMag/CentriMag systems where the risk of stroke is the highest. While contemporary antithrombotic therapies have likely contributed to lowering adverse event rates, we speculate that clotting and bleeding adverse events may have dropped because of a variety of other seemingly small changes to antithrombotic management that are independent of the antithrombotic agents used. This view is supported by recent reports documenting low stroke rates with anticoagulants other than bivalirudin, a drug that may have a wider therapeutic window but is not available in all locations throughout the world. The primary purpose of this report is 1) to summarize contemporary antithrombotic regimens used for smaller pediatric VADs today associated with low event rates in the United States and abroad and () to review 10 practical lessons learned and pitfalls to avoid that we believe to be important to reducing bleeding and clotting events based on our collective experience managing pediatric VADs over the past 20 years irrespective of the antithrombotic agents used.

ZusammenfassungIn den vergangenen Jahren hat der Einsatz mechanischer Unterstützungssysteme für Patienten mit Herz- und Kreislaufversagen kontinuierlich zugenommen, sodass in Deutschland mittlerweile jährlich etwa 3000 ECLS/ECMO-Systeme implantiert werden. Vor dem Hintergrund bislang fehlender umfassender Leitlinien bestand ein dringlicher Bedarf an der Formulierung evidenzbasierter Empfehlungen zu den zentralen Aspekten der ECLS/ECMO-Therapie.Im Juli 2015 wurde daher die Erstellung einer S3-Leitlinie durch die Deutsche Gesellschaft für Thorax-, Herz- und Gefäßchirurgie (DGTHG) bei der zuständigen Arbeitsgemeinschaft der Wissenschaftlichen Medizinischen Fachgesellschaften e. V. (AWMF) angemeldet. In einem strukturierten Konsensusprozess mit Einbindung von Experten aus Deutschland, Österreich und der Schweiz, delegiert aus 11 AWMF-Fachgesellschaften, 5 weiteren Fachgesellschaften sowie der Patientenvertretung, entstand unter Federführung der DGTHG die Leitlinie „Einsatz der extrakorporalen Zirkulation (ECLS/ECMO) bei Herz- und Kreislaufversagen“, die im Februar 2021 publiziert wurde.Die Leitlinie fokussiert auf klinische Aspekte der Initiierung, Fortführung, Entwöhnung und Nachsorge und adressiert hierbei auch strukturelle und ökonomische Fragestellungen. Dieser Artikel präsentiert eine Übersicht zu der Methodik und den konsentierten Empfehlungen.

Vor allem im Rahmen der COVID-19-Pandemie wird die ECLS/ECMO-Therapie noch häufiger in der Intensivmedizin eingesetzt und oft auch in den allgemeinen Medien genannt. Seit Februar 2021 ist die S3-Leitlinie „Einsatz der extrakorporalen Zirkulation (ECLS/ECMO) bei Herz- und Kreislaufversagen“ bei der Arbeitsgemeinschaft der Wissenschaftlichen Medizinischen Fachgesellschaften (AWMF) online abrufbar. Die Autorenschaft fasst die Inhalte in der Leitlinie zusammen und legt dabei einen Schwerpunkt auf die Intensivpflege und Physiotherapie.

OBJECTIVES The Berlin Heart EXCOR (BHE) offers circulatory support across all paediatric ages. Clinically, the necessary care and the outcomes differ in various age groups. The EUROMACS database was used to study age- and size-related outcomes for this specific device. METHODS All patients RESULTS In total, 303 patients were included [mean age: 2.0 years (interquartile range: 0.6–8.0, males: 48.5%)]. Age and BSA were not significantly associated with mortality (n = 74, P = 0.684, P = 0.679). Factors associated with a transplant (n = 175) were age (hazard ratio 1.07, P = 0.006) and aetiology other than congenital heart disease (hazard ratio 1.46, P = 0.020). Recovery rates (n = 42) were highest in patients with a BSA of CONCLUSIONS Mortality rates in Berlin Heart-supported patients cannot be predicted by age or BSA. Recovery rates are remarkably high in the smallest patient category (BSA

Seit einigen Jahren ist eine stetige Zunahme des Einsatzes von mechanischen extrakorporalen Herz-Kreislauf- und Lungenunterstutzungssystemen (ECLS/ECMO) zu verzeichnen. So wurden seit 2015 in Deutschland jahrlich bei etwa 3000 Patienten ECLS/ECMO-Systeme implantiert. Trotz dieser haufigen Anwendung der passageren Unterstutzungssysteme existieren zurzeit national und international nur Leitlinien zum Einsatz der ECMO/ECLS, die erkrankungsbezogene Teilaspekte komplexer Therapiekaskaden adressieren. Vor diesem Hintergrund erschien es notwendig, evidenzbasierte Empfehlungen zur ECLS/ECMO-Therapie im Hinblick auf Indikationen und das komplexe Patientenmanagement zu verfassen, in denen personelle, prozessuale und infrastrukturelle Anforderungen definiert werden. Aus diesem Grund erfolgte im Juli 2015 durch die Deutsche Gesellschaft fur Thorax‑, Herz- und Gefaschirurgie (DGTHG) die Anmeldung einer diesbezuglichen S3-Leitlinie bei der zustandigen Arbeitsgemeinschaft der Wissenschaftlichen Medizinischen Fachgesellschaften e. V. (AWMF). Im Mittelpunkt der Leitlinie steht die klinische Anwendung der ECLS/ECMO-Therapie; erganzend werden auch strukturelle und okonomische Aspekte adressiert. Unter Federfuhrung der DGTHG wurde mit Einbindung von Experten aus Deutschland, Osterreich und der Schweiz, delegiert aus 11 AWMF-Fachgesellschaften, 5 weiteren Fachgesellschaften sowie der Patientenvertretung, die S3-Leitlinie im Februar 2021 publiziert. Dieser Artikel stellt eine kurze Zusammenfassung des methodischen Konzepts sowie aller konsentierten Empfehlungen fur jede bearbeitete Fragestellung der Leitlinie dar.

Background: Acute kidney injury (AKI) is a common complication observed after neonatal aortic arch repair. We studied its incidence after procedures carried out using deep hypothermic circulatory arrest (DHCA) versus moderate hypothermia with distal aortic perfusion (MHDP), usually through the common femoral artery. In both groups, continuous regional cerebral perfusion (RCP) was used during the time required for aortic arch repair. Methods: A total of 125 neonates underwent aortic arch repair. Between 2007 and 2012, DHCA with RCP was used in 51 neonates. From 2013 to 2019, MHDP with RCP was performed on 74 newborns. Operative complexity was similar in both periods. Acute kidney injury was defined as a significant elevation of serum creatinine and was classified according to the neonatal modified n-KDIGO (neonatal Kidney Disease: Improving Global Outcomes) stages 1 to 3 (Kidney Disease Improving: Global Outcomes). Results: Acute kidney injury was observed in a total of 68 patients (68/125: 54.4%). In the majority (44/68: 64.7%), n-KDIGO stage 1 occurred. Stage 2 (n = 14) and stage 3 (n = 10) were observed more frequently after DHCA versus MHDP: 29.4% (15/51) versus 12.2% (9/74), P = .02. At cardiopulmonary bypass end, lactate levels were significantly higher ( P = .001) after DHCA: 3.4 (2.9-4.3) mmol/L compared to 2.7 (2.3-3.7) mmol/L after MHDP. Early mortality was 12% (15/125) in the entire cohort. It was 17.6% (9/51) after DHCA versus 8.1% (6/74) after MHDP, however not statistically significant ( P = .16). Conclusion: Mild (stage 1) AKI occurred frequently after neonatal aortic arch repair. The use of MHDP was associated with a significantly lower incidence of moderate (stage 2) and severe (stage 3) AKI forms.

In recent years, the use of mechanical support for patients with cardiac or circulatory failure has continuously increased, leading to 3,000 ECLS/ECMO (extracorporeal life support/extracorporeal membrane oxygenation) implantations annually in Germany. Due to the lack of guidelines, there is an urgent need for evidence-based recommendations addressing the central aspects of ECLS/ECMO therapy. In July 2015, the generation of a guideline level S3 according to the standards of the Association of the Scientific Medical Societies in Germany (AWMF) was announced by the German Society for Thoracic and Cardiovascular Surgery (GSTCVS). In a well-structured consensus process, involving experts from Germany, Austria and Switzerland, delegated by 16 scientific societies and the patients' representation, the guideline "Use of extracorporeal circulation (ECLS/ECMO) for cardiac and circulatory failure" was created under guidance of the GSTCVS, and published in February 2021. The guideline focuses on clinical aspects of initiation, continuation, weaning and aftercare, herein also addressing structural and economic issues. This article presents an overview on the methodology as well as the final recommendations.In den vergangenen Jahren hat der Einsatz mechanischer Unterstützungssysteme für Patienten mit Herz- und Kreislaufversagen kontinuierlich zugenommen, sodass in Deutschland mittlerweile jährlich etwa 3000 ECLS-/ECMO-Systeme implantiert werden. Vor dem Hintergrund bislang fehlender umfassender Leitlinien bestand ein dringlicher Bedarf an der Formulierung evidenzbasierter Empfehlungen zu den zentralen Aspekten der ECLS-/ECMO-Therapie. Im Juli 2015 wurde daher die Erstellung einer S3-Leitlinie durch die Deutsche Gesellschaft für Thorax-, Herz- und Gefäßchirurgie (DGTHG) bei der zuständigen Arbeitsgemeinschaft der Wissenschaftlichen Medizinischen Fachgesellschaften e. V. (AWMF) angemeldet. In einem strukturierten Konsensusprozess mit Einbindung von Experten aus Deutschland, Österreich und der Schweiz, delegiert aus 11 AWMF-Fachgesellschaften, 5 weiteren Fachgesellschaften sowie der Patientenvertretung, entstand unter Federführung der DGTHG die Leitlinie „Einsatz der extrakorporalen Zirkulation (ECLS/ECMO) bei Herz- und Kreislaufversagen“, die im Februar 2021 publiziert wurde. Die Leitlinie fokussiert auf klinische Aspekte der Initiierung, Fortführung, Entwöhnung und Nachsorge und adressiert hierbei auch strukturelle und ökonomische Fragestellungen. Dieser Artikel präsentiert eine Übersicht zu der Methodik und den konsentierten Empfehlungen.

OBJECTIVES A third paediatric report has been generated from the European Registry for Patients with Mechanical Circulatory Support (EUROMACS). The purpose of EUROMACS, which is operated by the European Association for Cardio-Thoracic Surgery, is to gather data related to durable mechanical circulatory support for scientific purposes and to publish reports with respect to the course of mechanical circulatory support therapy. Since the first report issued, efforts to increase compliance and participation have been extended. Additionally, the data provided the opportunity to analyse patients of younger age and lower weight. METHODS Participating hospitals contributed pre-, peri- and long-term postoperative data on mechanical circulatory support implants to the registry. Data for all implants in paediatric patients (, European Association for Cardio-Thoracic Surgery (EACTS), The European Association for Cardio-Thoracic Surgery (EACTS) supported this study and made resources available to execute this work.

OBJECTIVES: Ventricular assist device support as a bridge to transplant or recovery is a well-established therapy in children on the cardiac transplant waiting list. The goal of this study was to investigate the incidence of and the associated factors for cerebrovascular accidents in paediatric patients supported by a Berlin Heart EXCOR. METHODS: All patients, European Association for Cardio-Thoracic Surgery, The European Association for Cardio-Thoracic Surgery supported this study and made resources available to execute this work. No funding was received for this work.

The Berlin Heart EXCOR offers circulatory support across all paediatric ages. Clinically, the necessary care as well as outcomes differ in various age groups. The EUROMACS database was used to study age and size-related outcomes for this specific device.All patients19 years of age from the EUROMACS database supported with a Berlin Heart EXCOR between 2000 and November 2021 were included. Maximally Selected Rank statistics were used to determine BSA cut-off values. Multivariable Cox Proportional-Hazard Regression using ridge penalization was performed to identify factors associated with outcomes.In total, 303 patients were included (mean age: 2.0 years (interquartile range: 0.6-8.0, males: 48.5%)). Age and BSA were not significantly associated with mortality (n = 74, p = 0.684, p = 0.679). Factors associated with transplantation (n = 175) were age (HR 1.07, p = 0.006) and aetiology other than congenital heart disease (HR 1.46, p = 0.020). Recovery rates (n = 42) were highest in patients with a BSA0.53 m2 (21.8% vs 4.3-7.6% at 1 year, p = 0.00534). Patients with a BSA ≥0.73 m2 had a lower risk of early pump thrombosis, but a higher risk of early bleedings compared to children with a BSA0.73 m2.Mortality rates in Berlin Heart supported patients cannot be predicted by age or BSA. Recovery rates are remarkably high in the smallest patient category (BSA0.53 m2). This underscores that the Berlin Heart EXCOR is a viable therapeutic option, even for the smallest and youngest patients.

Background Right ventricular outflow tract (RVOT) stenosis after repair of tetralogy of Fallot has been linked with favorable right ventricular remodeling but adverse outcomes. The aim of our study was to assess the hemodynamic impact and prognostic relevance of right ventricular pressure load in this population. Methods and Results A total of 296 patients with repaired tetralogy of Fallot (mean age, 17.8±7.9 years) were included in a prospective cardiovascular magnetic resonance multicenter study. Myocardial strain was quantified by feature tracking technique at study entry. Follow‐up, including the need for pulmonary valve replacement, was assessed. The combined end point consisted of ventricular tachycardia and cardiac death. A higher echocardiographic RVOT peak gradient was significantly associated with smaller right ventricular volumes and less pulmonary regurgitation, but lower biventricular longitudinal strain. During a follow‐up of 10.1 (0.1–12.9) years, the primary end point was reached in 19 of 296 patients (cardiac death, n=6; sustained ventricular tachycardia, n=2; and nonsustained ventricular tachycardia, n=11). A higher RVOT gradient was associated with the combined outcome (hazard ratio [HR], 1.03; 95% CI, 1.00–1.06; P =0.026), and a cutoff gradient of ≥25 mm Hg was predictive for cardiovascular events (HR, 3.69; 95% CI, 1.47–9.27; P =0.005). In patients with pulmonary regurgitation ≥25%, a mild residual RVOT gradient (15–30 mm Hg) was not associated with a lower risk for pulmonary valve replacement. Conclusions Higher RVOT gradients were associated with less pulmonary regurgitation and smaller right ventricular dimensions but were related to reduced biventricular strain and emerged as univariate predictors of adverse events. Mild residual pressure gradients did not protect from pulmonary valve replacement. These results may have implications for the indication for RVOT reintervention in this population.

Background: Duration of mechanical ventilation is an important variable used by German Diagnosis-Related Groups (G-DRG) system to establish cost weight values for reimbursement after congenital heart surgery. Infants are commonly ventilated after open heart surgery. As of year 2015, we strived to achieve early postoperative extubation. This work studies how this approach impacted reimbursement after infant open heart surgery. Methods: Data of infants who underwent surgery on cardiopulmonary bypass (CPB) from 2014 to 2018 were reviewed. Successful early extubation was defined as end of mechanical ventilation within 24 hours postoperatively, without reintubation at a later point. Mean cost weight values (case mix index [CMI]) of achieved DRGs were used for estimation of reimbursement. Evolutions over years of early extubation and of reimbursement were compared. Results: A total of 521 infants underwent operations on CPB. Of these, 161 (31%) procedures were of higher risk Society of Thoracic Surgery and the European Association for Cardio-Thoracic Surgery (STAT) categories 3 and 4. Early extubation was achieved in 205 (39%) patients. The rate increased from 14% (year 2014) to 57% (year 2018). Case mix index amounted to 8.87 ± 7.00 after early extubation, and 12.37 ± 7.85 after late extubation: P value Conclusion: Early extubation could be progressively obtained in the majority of infants. This resulted in lower reimbursement. Surgical complexity was disregarded. The current G-DRG system appears to favor longer mechanical ventilation durations after infant open heart surgery.

Objectives Extracorporeal cardiopulmonary resuscitation in children with refractory cardiac arrest has been shown to improve survival, however, risk factors associated with mortality and neurologic impairments are not well defined. We analyzed our recent institutional experience with pediatric extracorporeal cardiopulmonary resuscitation to identify variables associated with survival and neurocognitive outcome. Design Retrospective observational study. Setting Pediatric cardiology and congenital heart surgery departments of a tertiary referral heart center. Patients Seventy-two consecutive children (median age, 0.3 yr [0.0-1.9 yr]) who underwent extracorporeal cardiopulmonary resuscitation at our institution during the study period from 2005 to 2016. Interventions Not applicable. Measurements and main results Median duration of resuscitation was 60 minutes (42-80 min) and median extracorporeal support duration was 5.4 days (2.2-7.9 d). Forty-three (59.7%) extracorporeal cardiopulmonary resuscitation events occurred during off-hours, however, neither duration of resuscitation (65 min [49-89 min] vs 51 min [35-80 min]; p = 0.16) nor survival (34.9% vs 37.9%; p = 0.81) differed significantly compared to working hours. Congenital heart disease was present in 84.7% of the patients. Survival to hospital discharge was 36.1%; younger age, higher lactate levels after resuscitation, acute kidney injury, renal replacement therapy, hepatic injury, and complexity of prior cardiothoracic surgical procedures were significantly associated with mortality. At mid-term follow-up (median, 4.1 yr [3.7-6.1 yr]), 22 patients (84.6% of discharge survivors) were still alive with 77.3% having a favorable neurologic outcome. High lactate levels, arrest location other than ICU, and requirement for renal replacement therapy were associated with unfavorable neurologic outcome. Interestingly, longer duration of resuscitation did not negatively impact survival or neurologic outcome. Conclusions Extracorporeal cardiopulmonary resuscitation is a valuable tool for the treatment of children with refractory cardiac arrest and a favorable neurologic outcome can be achieved in the majority of survivors even after prolonged resuscitation. Mortality after extracorporeal cardiopulmonary resuscitation in postcardiac surgery children is associated with procedural complexity.

The number of potential pediatric heart transplant recipients continues to exceed the number of donors, and consequently the waitlist mortality remains significant. Despite this, around 40% of all donated organs are not used and are discarded. This document (62 authors from 53 institutions in 17 countries) evaluates factors responsible for discarding donor hearts and makes recommendations regarding donor heart acceptance. The aim of this statement is to ensure that no usable donor heart is discarded, waitlist mortality is reduced, and post-transplant survival is not adversely impacted.

We describe the experience with biventricular HeartWare VAD (HVAD) support in the pediatric population. As of May 2017, using the Heartware database, 11 centers were identified. Seven centers participated providing information for 10 patients (four females, six males). Median age at the time of implantation was 12.7 ± 4.6years (5.3-6.9), median body surface area was 1.56. The indications for biventricular assist device (BiVAD) support included: myocarditis (n = 1), cardiomyopathy (n = 8), and one posttransplant heart failure. Six patients had a primary BiVAD implantation. Out of 10 patients with BiVAD, five were transplanted, one is ongoing (postoperatively day 207), and none were weaned from the device. Two patients (20%) were discharged from hospital on BiVAD support. Median support time was 52 days (16-235). The overall success rate was 60% surviving to transplant (median support time: 51 days) or are ongoing. Reasons for death included bleeding (n = 2), intracerebral hemorrhage (n = 1), and multisystem organ failure (n = 1). Eight out of 10 patients had at least one major complication, that is, major bleeding requiring re-operation (n = 6), severe neurologic injury (n = 1), and pump thrombosis in two patients requiring device exchange (n = 1) or thrombolysis therapy (n = 1). BiVAD HVAD implantation in children is a rare procedure, with high mortality and low discharge rates. Bleeding requiring re-operation was the most common postoperative complication, despite fresh sternotomies in the majority of patients. The risk and benefit ratio of such an approach should be carefully reviewed, and compared with the standard strategy using the Berlin EXCOR.

Therapeutic hypothermia during cardiac surgery has been widely used for neuroprotection and to attenuate the systemic inflammatory response due to cardiopulmonary bypass (CPB). Experimental data suggest that cold-shock protein RNA-binding motif 3 (RBM3), which is induced in response to hypothermia, plays a key role in hypothermia-induced organ protection. To date, investigation on RBM3 has been performed exclusively in vitro or in animal models, and the detection and regulation of RBM3 in human blood has not been investigated until now. The aim of this study was to investigate the level of RBM3 protein and cytokine expression profile involved in the inflammatory response in patients with congenital heart disease undergoing cardiac surgery involving CPB and therapeutic hypothermia. A single-center prospective trial with 23 patients undergoing cardiac surgery with CPB was performed. RBM3 protein was quantified in blood serum samples collected from patients and healthy individuals employing a new developed enzyme-linked immunosorbent assay. Cytokine levels were analyzed from dry blood spot samples using a Quanterix Simoa Immunoassay. For the first time, RBM3 protein was detected in blood samples of patients with congenital heart disease undergoing cardiac surgery. Hereby, RBM3 protein concentrations were significantly elevated in patients after cardiac surgery with CPB and mild hypothermia as compared with pre-surgery levels. Moreover, a complex immune reaction with significant induction of pro-inflammatory cytokines (interleukin [IL]-1 beta, IL-6, IL-8, IL-16, IL-18, monocyte chemotactic protein 1, CC-chemokine ligand [CCL]3, CCL4, intercellular adhesion molecule-1) in response to CPB was detected. Significantly elevated vascular endothelial growth factor and matrix metallopeptidase 3 concentrations reflecting ischemia/reperfusion-induced injury were observed 24 hours after weaning from CPB. The use of CPB is still associated with a complex inflammatory response. RBM3 protein is measurable in blood samples of patients with significantly higher concentrations after cardiac surgery with CPB and mild-to-moderate hypothermia. RBM3 is a new candidate as a biomarker for therapeutic hypothermia and a possible new therapeutic target for organ protection.

Left ventricular assist-device (LVAD) implantation is a life-saving therapy for patients with advanced heart failure (HF). With chronic unloading and circulatory support, LVAD-supported hearts often show significant reverse remodeling at the structural, cellular and molecular level. However, translation of these changes into meaningful cardiac recovery allowing LVAD explant is lagging. Part of the reason for this discrepancy is lack of anticipation and hence promotion and evaluation for recovery post LVAD implant. There is additional uncertainty about the long-term course of HF following LVAD explant. In selected patients, however, guided by the etiology of HF, duration of disease and other clinical factors, significant functional improvement and LVAD explantation with long-term freedom from recurrent HF events has been demonstrated to be feasible in a reproducible manner. The identified predictors of myocardial recovery suggest that the elective therapeutic use of potentially less invasive VADs for reversal of HF earlier in the disease process is a future goal that warrants further investigation. Hence, it is prudent to develop and implement tools to predict HF reversibility prior to LVAD implant, optimize unloading-promoted recovery with guideline directed medical therapy and monitor for myocardial improvement. This review article summarizes the clinical aspects of myocardial recovery and together with its companion review article focused on the biological aspects of recovery, they aim to provide a useful framework for clinicians and investigators.

In Germany, a remarkable increase regarding the usage of extracorporeal membrane oxygenation (ECMO) and extracorporeal life support (ECLS) systems has been observed in recent years with approximately 3000 ECLS/ECMO implantations annually since 2015. Despite the widespread use of ECLS/ECMO, evidence-based recommendations or guidelines are still lacking regarding indications, contraindications, limitations and management of ECMO/ECLS patients. Therefore in 2015, the German Society of Thoracic and Cardiovascular Surgery (GSTCVS) registered the multidisciplinary S3 guideline "Use of extracorporeal circulation (ECLS/ECMO) for cardiac and circulatory failure" to develop evidence-based recommendations for ECMO/ECLS systems according to the requirements of the Association of the Scientific Medical Societies in Germany (AWMF). Although the clinical application of ECMO/ECLS represents the main focus, the presented guideline also addresses structural and economic issues. Experts from 17 German, Austrian and Swiss scientific societies and a patients' organization, guided by the GSTCVS, completed the project in February 2021. In this report, we present a summary of the methodological concept and tables displaying the recommendations for each chapter of the guideline.Seit einigen Jahren ist eine stetige Zunahme des Einsatzes von mechanischen extrakorporalen Herz-Kreislauf- und Lungenunterstützungssystemen (ECLS/ECMO) zu verzeichnen. So wurden seit 2015 in Deutschland jährlich bei etwa 3000 Patienten ECLS/ECMO-Systeme implantiert. Trotz dieser häufigen Anwendung der passageren Unterstützungssysteme existieren zurzeit national und international nur Leitlinien zum Einsatz der ECMO/ECLS, die erkrankungsbezogene Teilaspekte komplexer Therapiekaskaden adressieren. Vor diesem Hintergrund erschien es notwendig, evidenzbasierte Empfehlungen zur ECLS/ECMO-Therapie im Hinblick auf Indikationen und das komplexe Patientenmanagement zu verfassen, in denen personelle, prozessuale und infrastrukturelle Anforderungen definiert werden. Aus diesem Grund erfolgte im Juli 2015 durch die Deutsche Gesellschaft für Thorax‑, Herz- und Gefäßchirurgie (DGTHG) die Anmeldung einer diesbezüglichen S3-Leitlinie bei der zuständigen Arbeitsgemeinschaft der Wissenschaftlichen Medizinischen Fachgesellschaften e. V. (AWMF). Im Mittelpunkt der Leitlinie steht die klinische Anwendung der ECLS/ECMO-Therapie; ergänzend werden auch strukturelle und ökonomische Aspekte adressiert. Unter Federführung der DGTHG wurde mit Einbindung von Experten aus Deutschland, Österreich und der Schweiz, delegiert aus 11 AWMF-Fachgesellschaften, 5 weiteren Fachgesellschaften sowie der Patientenvertretung, die S3-Leitlinie im Februar 2021 publiziert. Dieser Artikel stellt eine kurze Zusammenfassung des methodischen Konzepts sowie aller konsentierten Empfehlungen für jede bearbeitete Fragestellung der Leitlinie dar.

Zusammenfassung Eine pathologische Druckerhöhung im pulmonalen Gefäßsystem (pulmonale Hypertonie, PH) wurde bisher definiert durch einen invasiv gemessenen mittleren pulmonalarteriellen Druck (mPAP) ≥25 mm Hg in Ruhe. Auf dem 6th World Symposium on Pulmonary Hypertension (WSPH) in Nizza 2018 wurde eine neue Definition der PH vorgeschlagen, die die Senkung der Obergrenze des normalen mPAP von 24 auf 20 mm Hg beinhaltet. Obwohl keine Evidenz aus pädiatrischen Studien hierfür vorliegt, wurde diese neue PH-Definition (mPAP >20 mm Hg) aus Gründen der Einheitlichkeit auch von der pädiatrischen „Task Force“ des WSPH 2018 übernommen. Die vorliegende Stellungnahme der Arbeitsgemeinschaft Pulmonale Hypertonie (AGPH) der Deutschen Gesellschaft für Pädiatrische Kardiologie und angeborene Herzfehler e. V. (DGPK) erläutert die zugrunde liegende Rationale und mögliche Konsequenzen dieser Definitionsänderung. Insbesondere stellt sie klar, dass diese Änderung der Definition aktuell keinen Einfluss auf die Verschreibung von Medikamenten zur gezielten spezifischen Therapie der pulmonalarteriellen Hypertonie hat.

Background This study reports midterm results of high-risk patients with hypoplastic left ventricle treated with initial bilateral pulmonary artery banding (PAB) before secondary Norwood procedure (NP). Methods Retrospective study of 17 patients admitted between July 2012 and February 2017 who underwent this treatment strategy because diagnosis or clinical status was associated with high risk for NP. Survival was compared with that of patients who underwent primary NP. Results Mean Aristotle comprehensive complexity score for NP would have been 19.7 ± 2.6. Risk factors included obstructed pulmonary venous return (n = 9), body weight Conclusions PAB before NP in high-risk patients constituted salvage management. Primary PAB provided enough time for stabilization and control of most risk factors. It allowed midterm survival equivalent to the survival after primary NP in lower risk neonates.

Ventricular assist devices (VAD) are increasingly used in patients with end-stage heart failure due to acquired heart disease. Limited data exists on the use and outcome of this technology in children.All children (18 years of age) with VAD support included in the German National Register for Congenital Heart Defects were identified and data on demographics, underlying cardiac defect, previous surgery, associated conditions, type of procedure, complications and outcome were collected.Overall, 64 patients (median age 2.1 years; 45.3% female) receiving a VAD between 1999 and 2015 at 8 German centres were included in the analysis. The underlying diagnosis was congenital heart disease (CHD) in 25 and cardiomyopathy in 39 children. The number of reported VAD implantations increased from 13 in the time period 2000-2004 to 27 implantations in the time period 2010-2014. During a median duration of VAD support of 54 days, 28.1% of patients experienced bleeding complications (6.3% intracerebral bleeding), 14.1% thrombotic (10.9% VAD thrombosis) and 23.4% thromboembolic complications (including cerebral infarction in 18.8% of patients). Children with cardiomyopathy were more likely to receive a cardiac transplantation (79.5% vs. 28.0%) compared to CHD patients. Survival of cardiomyopathy patients was significantly better compared to the CHD cohort (p 0.0001). Multivariate Cox-proportional analysis revealed a diagnosis of CHD (hazard ratio [HR] 4.04, p = 0.001), age at VAD implantation (HR 1.09/year, p = 0.04) and the need for pre-VAD extracorporeal membrane oxygenation (ECMO) support (HR 3.23, p = 0.03) as independent predictors of mortality.The uptake of VAD therapy in children is increasing. Morbidity and mortality remain high, especially in patients with congenital heart disease and those requiring ECMO before VAD implantation.

To compare the safety and resource-efficacy of the fast-track (FT) concept (extubation ≤8 hours after surgery) versus the conventional approach (non-FT,8 hours postoperatively) in infants undergoing open-heart surgery.Infants7 kg operated on cardiopulmonary bypass between 2014 and 2018 were analyzed. Propensity score matching (1:1) was performed for group comparison (FT vs non-FT). Intensive care unit (ICU) personnel use and unit performance were evaluated. Postoperative outcome and reimbursement based on German diagnosis-related groups were compared.Of 717 infants (median age: 4 months, Society of Thoracic Surgeons-European Association for Cardio-Thoracic Surgery mortality score: 0.1-4), FT extubation was achieved in 182 infants (25%). After matching, 123 pairs (FT vs non-FT) were formed without significant differences in baseline characteristics. FT versus non-FT showed a significantly shorter ICU stay (in days): 1.8 (0.9-2.8) versus 4.2 (1.9-6.4), P .01, and postoperative length of stay (in days): 7 (6-10) versus 10 (7-15.5), P .01; significantly lower postoperative transfusion rates: 61.3% versus 77%, P .01; and tendency toward lower early mortality: 0% versus 2.8%, P = .08. Reintubation rate did not differ between the groups (P = .7). Despite a decrease in personnel capacity (2014 vs 2018), the unit performance was maintained. The mean case-mix-index of FT versus non-FT was 8.56 ± 6.08 versus 11.77 ± 12.10 (P .01), resulting in 27% less reimbursement in the FT group.FT concept can be performed safely and resource-effectively in infants undergoing open-heart surgery. Since German diagnosis-related group systems reimburse costs, not performance, there is little incentive to avoid prolonged mechanical ventilation. Greater ICU turnover rates and excellent postoperative outcomes are not rewarded adequately.

Introduction: Pulmonary hypertension (PH) is a common complication in patients with congenital heart disease (CHD), aggravating the natural, post-operative, or post-interventional course of the underlying anomaly. The various CHDs differ substantially in characteristics, functionality, and clinical outcomes among each other and compared with other diseases with pulmonary hypertension. Objective: To describe current management strategies and outcomes for adults with PH in relation to different types of CHD based on real-world data. Methods and results: COMPERA (Comparative, Prospective Registry of Newly Initiated Therapies for Pulmonary Hypertension) is a prospective, international PH registry comprising, at the time of data analysis, &gt, 8200 patients with various forms of PH. Here, we analyzed a subgroup of 680 patients with PH due to CHD, who were included between 2007 and 2018 in 49 specialized centers for PH and/or CHD located in 11 European countries. At enrollment, the patients&acute, median age was 44 years (67% female), and patients had either pre-tricuspid shunts, post-tricuspid shunts, complex CHD, congenital left heart or aortic disease, or miscellaneous other types of CHD. Upon inclusion, targeted therapies for pulmonary arterial hypertension (PAH) included endothelin receptor antagonists, PDE-5 inhibitors, prostacyclin analogues, and soluble guanylate cyclase stimulators. Eighty patients with Eisenmenger syndrome were treatment-na&iuml, ve. While at inclusion the primary PAH treatment for the cohort was monotherapy (70% of patients), with 30% of the patients on combination therapy, after a median observation time of 45.3 months, the number of patients on combination therapy had increased significantly, to 50%. The use of oral anticoagulants or antiplatelets was dependent on the underlying diagnosis or comorbidities. In the entire COMPERA-CHD cohort, after follow-up and receiving targeted PAH therapy (n = 511), 91 patients died over the course of a 5-year follow up. The 5-year Kaplan&ndash, Meier survival estimate for CHD associated PH was significantly better than that for idiopathic PAH (76% vs. 54%, p &lt, 0.001). Within the CHD associated PH group, survival estimates differed particularly depending on the underlying diagnosis and treatment status. Conclusions: In COMPERA-CHD, the overall survival of patients with CHD associated PH was dependent on the underlying diagnosis and treatment status, but was significantly better as than that for idiopathic PAH. Nevertheless, overall survival of patients with PAH due to CHD was still markedly reduced compared with survival of patients with other types of CHD, despite an increasing number of patients on PAH-targeted combination therapy.

We aimed to review current literature on the discard rate of donor hearts offered to pediatric recipients and assess geographical differences. Consequences and ways to reduce the discard rate are discussed. A systemic review on published literature on pediatric transplantation published in English since 2010 was undertaken. Additionally, a survey was sent to international OPOs with the goal of incorporating responses from around the world providing a more global picture. Based on the literature review and survey, there is a remarkably wide range of discard and/or refusal for pediatric hearts offered for transplant, ranging between 18% and 57% with great geographic variation. The data suggest that that the overall refusal rate may have decreased over the last decade. Reasons for organ discard were difficult to identify from the available data. Although the refusal rate of pediatric donor hearts seems to be lower compared to that reported in adults, it is still as high as 57% with geographic variation.

Optimal management of pediatric cardiac donors is essential in order to maximize donor heart utilization and minimize the rate of discarded organs. This review was performed after a systematic literature review and gives a detailed overview on current practices and guidelines. The review focuses on optimal monitoring of pediatric donors, donor workup, hormonal replacement, and obliterating the adverse effects of brain death. The current evidence on catecholamine support and thyroid hormone replacement is also discussed. Recognizing and addressing this shall help in a standardized approach toward donor management and optimal utilization of pediatric heart donors organs.

OBJECTIVES: A second paediatric report has been generated from the European Registry for Patients with Mechanical Circulatory Support (EUROMACS). the purpose of EUROMACS, which is operated by the European Association for Cardio-Thoracic Surgery, is to gather data related to durable mechanical circulatory support for scientific purposes and to publish reports with respect to the course of mechanical circulatory support therapy. Since the first report issued, efforts to increase compliance and participation have been extended. Additionally, the data provided the opportunity to analyse patients of younger age and lower weight. METHODS: Participating hospitals contributed pre-, peri- and long-term postoperative data on mechanical circulatory support implants to the registry. Data for all implants in paediatric patients (, European Association for Cardio-Thoracic Surgery; Stiftung Charite'r Cardio-Thoracic Surgery [BIH_PRO_430], This work was supported by European Association for Cardio-Thoracic Surgery; and Stiftung Charite ' [BIH_PRO_430 to O.M.].

A late preterm baby girl presented with feeding problems and desaturation. Septic work-up was negative, however. Severe right ventricular cardiac hypertrophy is revealed by echocardiography. Based on recent scientific findings on cardiodynamics and the inner antagonism in myocardial architecture, the baby is treated with very low-dose beta blockers instead of higher standard dosage. The therapy leads to immediate improvement in right ventricular function and reduction of right ventricular hypertrophy. Oxygen supply could be stopped, normal feeding was possible. Here we report the first case of very low-dose beta blocker therapy in a neonate for resolution of myocardial inner antagonism.

OBJECTIVES We analysed our 29-year experience of surgical repair of atrioventricular septal defect (AVSD) to define risk factors for mortality and reoperation. METHODS Between 1988 and 2017, 508 patients received AVSD repair in our institution; 359 patients underwent surgery for complete AVSD, 76 for intermediate AVSD and 73 for partial AVSD. The median age of the patients was 6.1 months (interquartile range 10.3 months), and the median weight was 5.6 kg (interquartile range 3.2 kg). The standard AVSD repair was performed using 2-patch technique (n = 347) and complete cleft closure (n = 496). The results were divided into 2 surgical eras (early era 1986-2004 and late era 2004-2017). Risk factors were analysed to determine the impact of patient age, weight, the presence of trisomy 21 and complex AVSD on mortality and reoperation rate. RESULTS In-hospital mortality decreased from 10.2% (n = 26) in early surgical era to 1.6% (n = 4) in late surgical era (P

Publications on the paediatric Berlin Heart EXCOR ventricular assist device have revealed that low body weight10 kg is a significant risk factor for mortality with children weighing5 kg being at the highest risk. However, these studies are limited to implantation periods prior to 2011. Since then, progress has been made in the optimization of patient selection and management. This study investigated whether the survival of children weighing10 kg supported with the EXCOR assist device has improved in recent years and sought to determine the risk factors for mortality.The Berlin Heart EXCOR prospective registry (n = 1832) was retrospectively reviewed between 2000 and 2017 to compare the outcomes of different weight cohorts: A (5 kg; n = 204), B (5-10 kg; n = 633) and C (10 kg; n = 995) in different eras [era 1: January 2000-December 2012 (n = 1089) and era 2: January 2013-June 2017 (n = 743)].Overall survival in groups A and B significantly increased from era 1 to era 2 (group A 51% vs 65%, P 0.001; group B 74% vs 78%, P = 0.001), whereas it remained stable in group C (78% vs 73%). In era 2, the survival of group B was not significantly different from group C. On the multivariable analysis of children weighing5 kg, congenital heart disease, preoperative extracorporeal life support and biventricular support were independently associated with increased mortality in era 1 [hazard ratio 2.04 (95% confidence interval 1.18-3.53); 2.44 (1.36-4.37) and 1.93 (1.11-3.34), respectively] but not in era 2.Paediatric EXCOR ventricular assist device therapy has significantly improved for patients weighing10 kg. Withholding a ventricular assist device is not justified on the basis of the body weight alone.

BACKGROUND The majority of children supported with ventricular assist devices (VADs) are bridged to heart transplantation. Although bridge to recovery has been reported, low recovery patient numbers has precluded systematic analysis. The aim of this study was to delineate recovery rates and predictors of recovery and to report on long-term follow-up after VAD explantation in children. METHODS Children bridged to recovery at our institution from January 1990 to May 2016 were compared with a non-recovery cohort. Clinical and echocardiographic data before and at pump stoppages and after VAD explantation were analyzed. Kaplan‒Meier estimates of event-free survival, defined as freedom from death or transplantation after VAD removal, were determined. RESULTS One hundred forty-nine children (median age 5.8 years) were identified. Of these, 65.2% had cardiomyopathy, 9.4% had myocarditis, and 24.8% had congenital heart disease. The overall recovery rate was 14.2%, and was 7.1% in patients with dilated cardiomyopathy. Predictors of recovery were age

Purpose Recently, a novel mobile driving unit (MDU) of the only approved pediatric ventricular assist device (VAD) system for both short- and long-term support of children of all sizes and ages received market approval. The MDU was specifically developed to improve mobility and autonomy of children supported with this paracorporeal (pc) VAD. This case series collects initial information with the aim to assess the first clinical experience. Methods A retrospective case series gathered anonymized patient health data of pediatric patients supported with MDU. The usability was assessed by means of a patient standardized questionnaire (numerical scale, 1= worst to 5= best). Results From February 2020 until September 2020, eight children (3 females) have been supported with a pc VAD in combination with the MDU. The median age was 1.4 years (range 0.5 - 7.5), median body surface area 0.4 m2 (0.3 - 0.9). Primary diagnosis was cardiomyopathy (CM) in 7 patients (5 dilated and 2 restrictive CM), and congenital heart disease in one child. Five patients (62.5%) received a pc VAD as left-sided and two patients as biventricular support (BVAD, 25%). One had a secondary right-sided pc VAD in combination with an implantable left-sided centrifugal VAD. The distribution of pc pump chambers by size was 10 ml pump: 2 patients (25%), 15 ml pump: 4 subjects (50%) and 30 ml pump: 2 children (25%), plus 15 ml pumps in both pc BVAD patients. The mean time of VAD support was 406 ± 241 days, with 148 ± 76 days on MDU. At the end of the observational period, 5 patients (62.5%) were still on system, 2 (25%) were transplanted and 1 died (12.5%) for causes not related to the driving unit. The survey found increased mobility (score 4.2/5), enlarged activity radius (5/5) and reduced noise nuisance (4.5/5) with MDU in comparison to the stationary unit. There were no complaints in MDU handling reported and the easiness to use was rated (4.5/5). In addition the parents reported a significant less heat generation by the MDU. The safety profile of MDU showed five adverse events: one fatal case (12.5%), one respiratory failure (12.5%), both not device-related, and two pump malfunctions (25%). One patient (12.5%) underwent pump replacement due to fibrin debris. Conclusion The case series suggests that the novel MDU is safe and effective for VAD support in the pediatric population. It is likely that MDU will increase the quality of life in this cohort.

Children frequently require red blood cell transfusion after cardiac surgery, which can generate an inflammatory response that is associated with increased morbidity and mortality. The aim of this study was to evaluate the inflammatory response and clinical effect of RBC transfusion in children. In a pediatric prospective controlled clinical trial 48 children were randomized to an unwashed or washed red blood cell transfusion group after cardiac surgery. Red blood cells were washed using an auto transfusion device with blood gas analyses of the packed red blood cells performed before transfusion. Vital parameters, blood analyses, and plasma concentrations of cytokines were measured before and 24 h after transfusion. Forty-five children were included with 23 receiving unwashed and 22 washed red blood cells. Washing reduced hemoglobin, potassium, and glucose while increasing sodium concentration in the packed red blood cells. Increased levels of hemoglobin, hematocrit, and erythrocyte counts were observed in both groups after transfusion. Increased levels of cytokine expression (IL-1α, IL-6, IL-8, MCP-1, and TARC) were detected in the group receiving unwashed red blood cells, while IL-8 levels were reduced in patients receiving washed red blood cells. Also, the percentage increase of cytokine concentrations (IL-6, IL-8, MCP-1, and GP-VI) before in comparison to after transfusion was lower in patients receiving washed red blood cells. No significant differences in clinical outcome parameters were observed between the two groups. Transfusion of red blood cells induces a pro-inflammatory reaction which can be diminished by washing the red blood cells before transfusion. Further clinical trials with larger patient cohorts receiving multiple transfusions are needed to better evaluate the clinical outcome.

A restrictive transfusion strategy led us to routinely try to conduct donor-blood free open-heart surgery even in neonates. The cardio-pulmonary bypass (CPB) circuit was minimized by priming volumina at 73 ml for the smallest patients with body weight up to 2.5 kg and 85-95 ml for those with body weight of more than 2.5 kg, and by positioning the console as close as possible to operation table. Measures were applied to save blood during the procedure. Transfusion threshold of 8 g/dl hemoglobin was retained. Effort was made to avoid transfusion while on CPB or to postpone transfusion towards CPB end. From 2013 to 2015, 149 consecutive neonates underwent 150 open-heart procedures without blood in priming volume. Weight was lower than 2.5 kg in five instances. The most frequent operations were arterial switch operation (n = 54) and Norwood procedure (n = 17). Transfusion-free operation was achieved in 44 procedures. The great majority (42/44 = 95%) involved biventricular repair and included 50% (27/54) of arterial switch operations. 106 patients were transfused: 63 mostly towards CPB end, and 43 after coming off bypass. Transfusion-free procedures were associated with postoperative lower lactate concentration (p = 0.0013) and shorter duration of mechanical ventilation (p = 0.0009). Seven patients were discharged from hospital without getting any transfusion of blood or blood products. In conclusion, routine application of bloodless priming in neonatal cardiopulmonary bypass is safe and beneficial. It results into a good number (29%= 44/150) of transfusion-free operations. Postponing transfusion towards CPB end favors an overall restrictive transfusion strategy for all patients.