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Objectives: Restrictive cardiomyopathy is rare and is generally associated with worse clinical outcomes compared to other cardiomyopathies. Ventricular assist device (VAD) support for these children is seldom applied and often hampered by the surgical difficulties., Methods: All paediatric (<19 years) patients with a restricted cardiomyopathy supported by a VAD from the EUROMACS database were included and compared to patients with a dilated cardiomyopathy (retrospective database analyses). Participating centres were retrospectively contacted to provide additional detailed echo and Swan Ganz measurements to analyse the effect of VAD support on pulmonary artery pressure and right ventricular function., Results: Forty-four paediatric VAD-supported patients diagnosed with restricted cardiomyopathy were included, with a median age at implantation of 5.0 years. Twenty-six of the 44 patient with a restricted cardiomyopathy survived to transplantation (59.1%), 16 died (36.4%) and 2 are still on ongoing VAD support (4.5%) after a median duration of support of 95.5 days (interquartile range 33.3-217.8). Transplantation probability after 1 and 2 years of VAD support in patients with a restricted cardiomyopathy were comparable to patients with a dilated cardiomyopathy (52.3% vs 51.4% and 59.5% vs 60.1%, P = 0.868). However, mortality probability was higher in the restricted cardiomyopathy cohort (35.8% vs 17.0% and 35.8% vs 19.0%, P = 0.005). Adverse event rates were high (cerebrovascular accident in 31.8%, pump thrombosis in 29.5%, major bleeding 25.0%, eventual biventricular support in 59.1%). In the atrially cannulated group, cerebrovascular accident and pump thrombosis occurred in twice as much patients (21.1% vs 40.0%, P = 0.595 and 15.8% vs 40.0%, P = 0.464; probably non-significant due to the small numbers). Pulmonary arterial pressures improved after implantation of a VAD, and 6 patients who were initially labelled as ineligible due to pulmonary hypertension could eventually be transplanted., Conclusions: VAD support in children with a restricted cardiomyopathy is rarely performed. Mortality and adverse event rates are high. On the other hand, survival to cardiac transplantation was 59.1% with all patients surviving the 1st 30 days after cardiac transplantation. Pulmonary arterial pressures improved while on support, potentially making cardiac transplantation a viable option for previously ineligible children., (© The Author(s) 2024. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

Objectives: The use of ventricular assist devices (VADs) in children is increasing. However, absolute numbers in individual centres and countries remain small. Collaborative efforts such as the Paedi-European Registry for Patients with Mechanical Circulatory Support (EUROMACS) are therefore essential for combining international experience with paediatric VADs. Our goal was to present the results from the fourth Paedi-EUROMACS report., Methods: All paediatric (<19 years) patients from the EUROMACS database supported by a VAD were included. Patients were stratified into a congenital heart disease (CHD) group and a group with a non-congenital aetiology. End points included mortality, a transplant and recovery. Cox proportional hazard models were used to explore associated factors for mortality, cerebrovascular accident and pump thrombosis., Results: A total of 590 primary implants were included. The congenital group was significantly younger (2.5 vs 8.0 years, respectively, P < 0.001) and was more commonly supported by a pulsatile flow device (73.5% vs 59.9%, P < 0.001). Mortality was significantly higher in the congenital group (30.8% vs 20.4%, P = 0.009) than in the non-congenital group. However, in multivariable analyses, CHD was not significantly associated with mortality [hazard ratio (HR) 1.285; confidence interval (CI) 0.8111-2.036, P = 0.740]. Pump thrombosis was the most frequently reported adverse event (377 events in 132 patients; 0.925 events per patient-year) and was significantly associated with body surface area (HR 0.524, CI 0.333-0.823, P = 0.005), CHD (HR 1.641, CI 1.054-2.555, P = 0.028) and pulsatile flow support (HR 2.345, CI 1.406-3.910, P = 0.001) in multivariable analyses., Conclusions: This fourth Paedi-EUROMACS report highlights the increasing use of paediatric VADs. The patient populations with congenital and non-congenital aetiologies exhibit distinct characteristics and clinical outcomes., (© The Author(s) 2024. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

Objectives: Myocardial recovery in children supported by a durable left ventricular assist device is a rare, but highly desirable outcome because it could potentially eliminate the need for a cardiac transplant and the lifelong need for immunosuppressant therapy and the risk of complications. However, experience with this specific outcome is extremely limited., Methods: All patients < 19 years old supported by a durable left ventricular assist device from the European Registry for Patients with Mechanical Circulatory Support database were included. Participating centres were approached for additional follow-up data after explantation. Associated factors for explantation due to myocardial recovery were explored using Cox proportional hazard models., Results: The incidence of recovery in children supported by a durable left ventricular assist device was 11.7% (52/445; median duration of support, 122.0 days). Multivariable analyses showed body surface area (hazard ratio 0.229; confidence interval 0.093-0.565; P = 0.001) and a primary diagnosis of myocarditis (hazard ratio 4.597; confidence interval 2.545-8.303; P < 0.001) to be associated with recovery. Left ventricular end-diastolic diameter in children with myocarditis was not associated with recovery. Follow-up after recovery was obtained for 46 patients (88.5%). Sustained myocardial recovery was reported in 33/46 (71.7%) at the end of the follow-up period (28/33; >2 year). Transplants were performed in 6/46 (11.4%) (in 5 after a ventricular assist device was reimplanted). Death occurred in 7/46 (15.2%)., Conclusions: Myocardial recovery occurs in a substantial portion of paediatric patients supported with durable left ventricular assist devices, and sustainable recovery is seen in around three-quarters of them. Even children with severely dilated ventricles due to myocarditis can show recovery. Clinicians should be attentive to (developing) myocardial recovery. These results can be used to develop internationally approved paediatric weaning guidelines., (© The Author(s) 2024. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

Purpose: The withdrawal of HVAD in 2021 created a concern for the pediatric population. The alternative implantable centrifugal blood pump HeartMate 3 has since been used more frequently in children. This paper analyses the outcome of children on LVAD support provided with an HVAD or HM3. Methods: A retrospective analysis of the EUROMACS database on children supported with VAD < 19 years of age from 1 January 2009 to 1 December 2021 was conducted. All patients with an LVAD and either an HVAD or HM3 were included. Patients with missing data on VAD status and/or missing baseline and/or follow up information were excluded. Kaplan-Meier survival analysis was performed to evaluate survival differences. Analyses were performed using Fisher's exact test. Results: The study included 150 implantations in 142 patients with 128 implants using an HVAD compared to 28 implants using an HM3. Nine patients (6%) needed temporary right ventricular mechanical support, which was significantly higher in the HM3 group, with 25% ( p : 0.01). Patients in the HVAD group were significantly younger (12.7 vs. 14.5 years, p : 0.01), weighed less (45.7 vs. 60 kg, p : <0.000) and had lower BSA values (1.3 vs. 1.6 m 2 , p : <0.000). Median support time was 204 days. Overall, 98 patients (69%) were discharged and sent home, while 87% were discharged in group HM3 ( p : ns). A total of 123 children (86%) survived to transplantation, recovery or are ongoing, without differences between groups. In the HVAD group, 10 patients (8%) died while on support, whereas in 12% of HM3 patients died ( p : 0.7). Conclusions: Survival in children implanted with an HM3 was excellent. Almost 90% were discharged and sent home on the device.

Background: Data on the use and outcome of children on ventricular assist device (VAD) support provided with an implantable cardioverter-defibrillator (ICD) remains poor., Methods: A retrospective analysis of the EUROMACS database on children supported with VAD < 19 years of age from January 1, 2009 to April 1, 2020. Patients with missing data on status of ICD, missing baseline and/or follow up information were excluded. The primary independent variable of interest was the concomitant presence or absence of an ICD at the time of VAD placement. Kaplan-Meier survival analysis was performed to evaluate survival differences between children on VAD with and without an ICD., Results: Out of 303 patients provided with a VAD, 7% (7♀, 15♂) had an ICD implanted and formed the study group. Median age was 14 years, median weight was 43.5 kg, and median BSA was 1.39. Median Intermacs stage was 2 (range: 1-7). Seventeen patients (77%) were transplanted, 4 (18%) died while on support, and 1 (5%) was weaned from device after myocardial recovery. Median time on support was 68 days compared to 361 days in the control group (p: 0.01). Three patients underwent device exchange due to thrombus formation in the pump. There was no difference in survival between groups (p = 0.342)., Conclusion: The presence of ICD in pediatric patients supported with a VAD is low (7%). Children on VAD support provided with an ICD do not have a survival benefit compared to children without an ICD., (© 2023 International Center for Artificial Organ and Transplantation (ICAOT) and Wiley Periodicals LLC.)

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., Competing Interests: Die Autorinnen/Autoren geben an, dass kein Interessenkonflikt besteht., (Thieme. All rights reserved.)

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 <19 years of age from the EUROMACS database supported with a BHE between 2000 and November 2021 were included. Maximally selected rank statistics were used to determine body surface area (BSA) cut-off values. Multivariable Cox proportional hazard regression using ridge penalization was performed to identify factors associated with outcomes., 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 <0.53 m2 (21.8% vs 4.3-7.6% at 1 year, P = 0.00534). Patients with a BSA of ≥0.73 m2 had a lower risk of early pump thrombosis but a higher risk of early bleeding compared to children with a BSA of <0.73 m2., 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 <0.53 m2). This underscores that the BHE is a viable therapeutic option, even for the smallest and youngest patients., (© The Author(s) 2022. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery.)

Background: The number of single ventricle patients undergoing Fontan palliation and surviving to adulthood worldwide has steadily increased in recent years. Nevertheless, the Fontan circulation is destined to fail. Ultimately, heart transplantation (HTx) remains the definitive treatment option. Due a shortage of organs, mechanical circulatory support in the form of ventricular assist devices (VADs) is widely used to bridge heart failure patients to HTx, but these devices have been mainly developed to address the needs of normal anatomies. A novel venous cannula has been developed as part of the EXCOR® VAD to provide subpulmonary support in these patients. Its clinical application is investigated in the "Registry to Assess the Safety and Feasibility of the Subpulmonary Support with the Novel Venous Cannula in Patients with Failing/Absence of the Right Heart" (RegiVe study, NCT04782232)., Methods: RegiVe is a multicenter, international, observational, prospective, non-randomized registry aiming to collect the routine clinical data of up to 20 patients. The primary endpoints address device performance and safety, while the secondary endpoints target organ status and overall safety (according to the Interagency Registry for Mechanically Assisted Circulatory Support - INTERMACS - definitions). Data analysis will be performed by means of descriptive statistics., Results: RegiVe has received the favorable opinion of an independent ethics committee and enrollment has recently started., Conclusion: RegiVe is the first study evaluating the use of a medical device specifically developed for subpulmonary support of failing Fontan patients. The study will provide important insight and further information on this cohort and help to improve a dedicated VAD strategy., Competing Interests: E. D. S., T. S., K.-J. S., and A. K. M. are employees of Berlin Heart GmbH. O. M. and F. D. R. received fees as EEPIG chairs. F. D. R. is proctor of Berlin Heart GmbH., (The Author(s). This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/).)

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., Competing Interests: Disclosure: Ms. Murray and Dr. Almond have received travel support to Advanced Cardiac Therapies Improving Outcomes Network (ACTION) meetings, which is supported by Berlin Heart, Inc and Abbott, Inc. Dr. Almond served as principle investigator (PI) on the US Food and Drug Administration (FDA) Orphan Product Development grant supporting the Berlin Heart Investigational Device Exemption (IDE) Trial (RO1 FDA 00033577) and currently serves as Medical PI of the NHLBI contract supporting the PumpKIN Trial (HHS N268201200001I). The other authors have no conflicts of interest to report., (Copyright © ASAIO 2022.)

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., (Copyright © 2022 The Authors. Published by Elsevier Inc. All rights reserved.)

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 <19 years of age supported by a Berlin Heart EXCOR between January 2011 and January 2021 from the European Registry for Patients with Mechanical Circulatory Support were included., Results: In total, 230 patients were included. A total of 140 (60.9%) patients had a diagnosis of dilated cardiomyopathy. 46 patients (20.0%) sustained 55 cerebrovascular accidents, with 70.9% of the episodes within 90 days after the ventricular assist device was implanted. The event rate of cerebrovascular accidents was highest in the first era (0.75). Pump thrombosis and secondary need for a right ventricular assist device were found to be associated with a cerebrovascular accident (hazard ratio 1.998, P = 0.040; hazard ratio 11.300, P = 0.037). At the 1-year follow-up, 44.4% of the patients had received a transplant, 13.1% were weaned after recovery and 24.5% had died. Event rates for mortality showed a significantly decreasing trend., Conclusions: Paediatric ventricular assist device support is associated with important adverse events, especially in the early phase after the device is implanted. Pump thrombosis and the need for a secondary right ventricular assist device are associated with cerebrovascular accidents. Furthermore, an encouragingly high rate of recovery in this patient population was shown, and death rates declined. More complete input of data into the registry, especially concerning anticoagulation protocols, would improve the data., (© The Author(s) 2022. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

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 (<19 years of age) performed from 1 January 2000 to 31 December 2020 were analysed. This report includes updates of patient characteristics, implant frequency, outcome (including mortality rates, transplants and recovery rates) as well as adverse events including neurological dysfunction, device malfunction, major infection and bleeding., Results: Twenty-five hospitals contributed 537 registered implants in 480 patients. The most frequent aetiology of heart failure was any form of cardiomyopathy (59%), followed by congenital heart disease and myocarditis (15% and 14%, respectively). Competing outcomes analysis revealed that a total of 86% survived to transplant or recovery or are ongoing; at the 2-year follow-up examination, 21.9% died while on support. At 12 months, 45.1% received transplants, 7.5% were weaned from their device and 20.8% died. The 3-month adverse events rate was 1.59 per patient-year for device malfunction including pump exchange, 0.7 for major bleeding, 0.78 for major infection and 0.71 for neurological events., Conclusions: The overall survival rate was 79.2% at 12 months following ventricular assist device implant. The comparison of survival rates of the early and later eras shows no significant difference. A focus on specific subgroups showed that survival was less in patients of younger age (<1 year of age; P = 0.01) and lower weight (<20 kg; P = 0.015). Transplant rates at 6 months continue to be low (33.2%)., (© The Author(s) 2022. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

Riociguat, a soluble guanylate cyclase stimulator, is approved for treatment of adults with pulmonary arterial hypertension (PAH). The safety, tolerability, and pharmacokinetics (PK) of oral riociguat in a pediatric population with PAH was assessed in PATENT-CHILD (NCT02562235), a multicenter, single-arm, 24-week, open-label, Phase 3 study. Patients aged 6-17 years in World Health Organization functional class (WHO-FC) I-III treated with stable endothelin receptor antagonists and/or prostacyclin analogs received riociguat equivalent to 0.5-2.5 mg three times daily in adults, as either oral pediatric suspension or tablets, based on bodyweight. Primary outcomes were safety, tolerability, and PK of riociguat. Twenty-four patients (mean age 12.8 years), 18 of whom were in WHO-FC II, were enrolled. Adverse events (AEs), mostly mild or moderate, were reported in 20 patients (83%). Four patients (17%) experienced a serious AE; all resolved by study end and two (8%) were considered study-drug related. Hypotension was reported in three patients and hemoptysis in one (all mild/moderate intensity). Riociguat plasma concentrations in pediatric patients were consistent with those published in adult patients. From baseline to Week 24, mean ± standard deviation increase in 6-minute walking distance was 23 ± 69 m ( n  = 19), and mean decrease in NT-proBNP was -66 ± 585 pg/ml ( n  = 14). There was no change in WHO-FC. Two patients experienced clinical worsening events of hospitalization for right heart failure. PK results confirmed a suitable riociguat dosing strategy for pediatric patients with PAH. The data suggest an acceptable safety profile with potential efficacy signals., Competing Interests: H. G. A. reports fees for lectures and member of advisory boards for Bayer and Janssen. D. D. I. reports the University of Colorado contracts with Acceleron, Actelion/Janssen, Altavant, Bayer, Eli Lilly, GlaxoSmithKline, and United Therapeutics for D. D. I. to be a consultant and perform clinical trials. S. M. reports consultancy from Actelion/Janssen and GlaxoSmithKline. J. P. reports fees for lectures and/or consultancy from Actelion/Janssen. T. P. reports fees for lectures, member of advisory boards, and research grants from Actelion/Janssen, Bayer, GlaxoSmithKline, and Pfizer. H. Y. reports research grants and lecture fees from Actelion, AbbVie, and Teijin Pharma; research grants from Tanabe‐Mitsubishi; lecture fees from Bayer, GE Healthcare Japan, Merck Sharp and Dohme, Novo Nordisk, Nihon Seiyaku, Nihon Shinyaku, Pfizer, and Termo. G. C., M. G., S. L., A. R., S. S., S. W., G. W. are employees and stockholders of Bayer AG. D. B. is a steering committee member for Eli Lilly and Company, and steering committee and advisory board member for Actelion Pharmaceuticals, Bayer, and Novartis. M. B. reports fees for lectures and/or consultancy from Acceleron, Actelion/Janssen, AOP, Bayer, Eli Lilly, GlaxoSmithKline, and Gossamer; unrestricted research grants from Actelion and Bayer. The remaining authors declare no conflict of interest., (© 2022 The Authors. Pulmonary Circulation published by John Wiley & Sons Ltd on behalf of Pulmonary Vascular Research Institute.)

Aims Worldwide applications of extracorporeal circulation for mechanical support in cardiac and circulatory failure, which are referred to as extracorporeal life support (ECLS) or veno-arterial extracorporeal membrane oxygenation (va-ECMO), have dramatically increased over the past decade. In spite of the expanding use and the immense medical as well as socio-economic impact of this therapeutic approach, there has been a lack of interdisciplinary recommendations considering the best available evidence for ECLS treatment. Methods and Results In a multiprofessional, interdisciplinary scientific effort of all scientific societies involved in the treatment of patients with acute cardiac and circulatory failure, the first evidence- and expert consensus-based guideline (level S3) on ECLS/ECMO therapy was developed in a structured approach under regulations of the AWMF (Association of the Scientific Medical Societies in Germany) and under use of GRADE (Grading of Recommendations Assessment, Development and Evaluation) criteria. This article presents all recommendations created by the expert panel, addressing a multitude of aspects for ECLS initiation, continuation, weaning and aftercare as well as structural and personnel requirements. Conclusions This first evidence- and expert consensus-based guideline (level S3) on ECLS/ECMO therapy should be used to apply the best available care nationwide. Beyond clinical practice advice, remaining important research aspects for future scientific efforts are formulated., (© 2021 The Authors. ESC Heart Failure published by John Wiley & Sons Ltd on behalf of European Society of Cardiology.)

Background: 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., Methods: 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., Results: 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., Conclusions: 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., (Copyright © 2021. Published by Elsevier B.V.)

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., (© 2021. Springer Medizin Verlag GmbH, ein Teil von Springer Nature.)

Objectives: 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., Methods: Infants <7 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., Results: 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., Conclusions: 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., (Copyright © 2020 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.)

Background: Left ventricular non-compaction cardiomyopathy (LVNC) has been reported in association with almost all types of congenital heart valve disease. The presence of LVNC-related ventricular dysfunction increases the perioperative risk in these patients. The advantages of transcatheter treatment modalities outweigh those of surgical strategies, as they avoid cardioplegic arrest and myocardial trauma. To our knowledge, there have been no reports on transcatheter treatment of pure aortic regurgitation in patients with a bicuspid aortic valve (BAV) and concomitant LVNC., Case Summary: In this article, we present the case of a 13-year-old boy with a regurgitant BAV and concomitant LVNC who presented with end-stage heart failure and severe pulmonary hypertension. As a bridge to definitive therapy, the patient underwent an uneventful transcatheter aortic valve implantation (TAVI) using a 26-mm balloon-expandable prosthesis. Device success without paravalvular regurgitation was achieved. At 17 months of follow-up, a steady reduction in pulmonary arterial pressure, persistent normalization of systolic left ventricular function and a tremendous improvement in the patient's physical resilience was observed. The initially considered heart-lung transplantation was avoided and will not be necessary., Discussion: To the best of our knowledge, this is the first case performed with TAVI for BAV regurgitation in the context of LVNC. With technical modifications and appropriate planning, TAVI in paediatric patients with a non-calcified BAV is feasible. Different imaging modalities revealed an intriguing relationship between aortic regurgitation and morphological signs of a left ventricular non-compaction myocardium., (© The Author(s) 2021. Published by Oxford University Press on behalf of the European Society of Cardiology.)

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.

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.

Background: Corrective surgery for congenital heart defects is known to trigger a severe immune reaction. There has been extensive research on the effects of inflammation after cardiopulmonary bypass (CPB). Interestingly, monocytes are observed to be non-responsive to stimulation with lipopolysaccharide (LPS) under these conditions, indicating a state of immunodepression, which lays the ground for second hit infections after cardiosurgery with CPB., Objectives: The aim of this prospective study was to analyze immunodepression after pediatric cardiopulmonary bypass and to differentiate the effects of monocytic anergy on postoperative outcome., Methods: In a prospective trial, we quantified the immune responses in 20 pediatric patients (median age 4.9months, range 2.3-38.2months; median weight 7.2kg, range 5.2-11.7kg) with congenital ventricular septal defect undergoing heart surgery with CPB. Ex vivo LPS-induced protein expression of IFN-γ, IL-1β, IL-1Ra, IL-6, IL-8, IL-10, IL-12, IL-17, TNF-α, and MCP-1 was measured before (T1), immediately after (T2) and 4h after (T3) cardiopulmonary bypass surgery using Luminex technology., Results: The innate immune system responds to CPB with an almost complete depression of monocytic function. Inflammatory IL-12, TNF-α, IL-1β, IL-6, IL-8 and IFN-y are completely suppressed. IL-10, IL-1Ra and MCP-1 are still produced during suppression with IL-1Ra being overly secreted during reversion. Suppression of TNF-α expression after LPS-stimulation correlates closely with longer mechanical ventilation time (r=-0.619, p=0.004)., Conclusion: Cardiosurgery with CPB causes a state of immunodepression making pediatric patients more vulnerable to second hit infections. MCP-1, IL-10, and IL-1Ra play an important role in monocyte recovery, eventually permitting new therapeutic options for controlling immunodepression and inflammation. Standardized glucocorticoid therapy should be evaluated carefully for each individual patient., (Copyright © 2017. Published by Elsevier Ltd.)

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 <2 years (recovery rate 27.8%, odds ratio [OR] 5.64, 95% confidence interval [CI] 2.0 to 16.6) and diagnosis of myocarditis (rate 57.1%; OR 17.56, 95% CI 4.6 to 67.4). After a median follow-up of 10.8 years, 15 patients (83.3%) were in Functional Class I and 3 (16.7%) in were in Class II. Mean left ventricular ejection fraction was 53% (range 28% to 64%). Ten- and 15-year event-free survival rates were both 84.1 ± 8.4%., Conclusions: Children <2 years of age and those diagnosed with myocarditis have the highest probability of recovery. Long-term survival after weaning from the VAD was better than after heart transplantation, as demonstrated in the excellent long-term stability of ejection fraction and functional class., (Copyright © 2018 International Society for Heart and Lung Transplantation. Published by Elsevier Inc. All rights reserved.)

In the summer of 2016, delegates from the German Respiratory Society (DGP), the German Society of Cardiology (DGK) and the German Society of Pediatric Cardiology (DGPK) met in Cologne, Germany, to define consensus-based practice recommendations for the management of patients with pulmonary hypertension (PH). These recommendations were built on the 2015 European Pulmonary Hypertension guidelines, aiming at their practical implementation, considering country-specific issues, and including new evidence, where available. To this end, a number of working groups was initiated, one of which was specifically dedicated to PH in adults associated with congenital heart disease (CHD). As such patients are often complex and require special attention, and the general PAH treatment algorithm in the ESC/ERS guidelines appears too unspecific for CHD, the working group proposes an analogous algorithm for the management of PH-CHD which takes the special features of this patient group into consideration, and includes general measures, supportive therapy, targeted PAH drug therapy as well as interventional and surgical procedures. The detailed results and recommendations of the working group on PH in adults with CHD, which were last updated in the spring of 2018, are summarized in this article., (Copyright © 2018 Elsevier B.V. All rights reserved.)

Objectives: Little is known about cerebral strokes in paediatric patients supported by intracorporeal continuous-flow ventricular assist devices., Methods: We retrospectively investigated patients younger than 19 years of age who were treated with an intracorporeal continuous-flow ventricular assist device in the European Registry for Patients with Mechanical Circulatory Support (EUROMACS) database. The patients were stratified by body surface area in Group 1 [<1.2 m2 (n = 13)] and Group 2 [≥1.2 m2 (n = 38)]. Cerebral strokes, both ischaemic and haemorrhagic, were studied., Results: Of the 2941 patients with ventricular assist device (VAD) implants listed in the database, 124 (4%) patients were less than 19 years of age. Fifty-one of them (2%) were supported with a continuous-flow ventricular assist device. Group 1 (6 female and 7 male) had a mean age (±SD) of 9 ± 2.3 years compared with 15.6 ± 1.8 years in Group 2 (21 female and 17 male). Three (23%) patients died in Group 1 on VAD support, whereas 5 (13%) patients died in Group 2 (P = 0.21; log-rank test). Seven (54%) patients with a VAD in Group 1 and 17 (45%) patients in Group 2 underwent transplantation (P = 0.29); of these, 1 (8%) patient recovered (Group 1) with subsequent device explantation. The other patients, 2 in Group 1 and 16 in Group 2, were still on device support at the time of the analysis. There were no cerebral strokes in Group 1, but 4 cerebral strokes (11% of Group 2, 8% of a total of 51 patients in Groups 1 and 2 combined) occurred in Group 2 (3 patients died; P = 0.26; log-rank test). Taken together, the incidence of cerebral strokes in this paediatric cohort of patients with an intracorporeal VAD was 0.1 per patient-year., Conclusions: The incidence of cerebral strokes in children with intracorporeal VADs (0.1 per patient-year) seems to be low irrespective of the body surface area., (© The Author 2017. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

Aim: To investigate if the serum biomarkers of cerebral injury, neuron-specific enolase and S100b protein, may classify unfavourable neurological outcome after paediatric cardiac arrest., Methods: We performed a retrospective study of neuron-specific enolase and S100b measurements from 95 children treated in our paediatric cardiac intensive care unit after cardiac arrest. Neurological outcome at discharge was evaluated using the paediatric cerebral performance category scale, with unfavourable outcome defined as a change of >1 compared to pre-arrest status or death., Results: Fifty-eight patients (61.1%) survived to discharge with 48 (50.5%) having a favourable neurological outcome. We observed significantly higher levels of both biomarkers in the unfavourable outcome group at designated time points (neuron-specific enolase at 24, 48, and 72h and S100b at 12, 24, and 48h after cardiac arrest, p<0.05). Receiver operating characteristic areas under the curve for neuron-specific enolase were 0.83, 0.80, and 0.73 at time points 24, 48, and 72h and 0.87, 0.81, and 0.82 for S100b at 12, 24, and 48h after cardiac arrest, respectively. Neuron-specific enolase measurement at 24h after cardiac arrest was an independent predictor of unfavourable outcome in a multivariable analysis., Conclusions: Neuron-specific enolase and S100b classify unfavourable neurological outcome in this large paediatric cardiac arrest cohort. Further multi-institutional prospective studies to comprehensively evaluate the diagnostic accuracy of these biomarkers under various clinical conditions and to determine reliable cut-off values in children are warranted., (Copyright © 2017 Elsevier B.V. All rights reserved.)

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.

Objectives: We aimed to compare early postoperative outcome after extracardiac (EC) Fontan operation between patients with right (RV) or left (LV) systemic ventricles., Methods: In total, 173 consecutive patients (median age 4 years, median weight 14 kg) underwent EC Fontan between 1995 and 2013. Pre- and intraoperative data as well as detailed postoperative haemodynamic variables were compared between patients with LV [ n  = 109 (63%)] and RV [ n  = 64 (37%)]., Results: : RV patients showed significantly lower mean arterial (median 55 vs 59 mmHg, P  = 0.04), higher atrial (median 8 vs 6 mmHg, P  = 0.03) and comparable pulmonary pressure (median 14 vs 14 mmHg, P  = 0.7) as well as lower mean systemic perfusion pressure (median 39 vs 43 mmHg, P  = 0.03) on Day 0 after EC Fontan. They suffered from longer intubation time (median 18 vs 12 h, P  = 0.008), higher incidence of ascites (46% vs 28%, P  = 0.04) and need for dialysis (21% vs 4%, P  = 0.003). Prolonged inotropic support (25% vs 8%, P  = 0.02) and pharmacological treatment to reduce pulmonary vascular resistance (71% vs 53%, P  = 0.002) were more often used in RV patients and they showed more often supraventricular tachyarrhythmia (27% vs 5%, P  < 0.001) and a longer intensive care unit-stay (median 4 vs 3 days, P  = 0.03). However, early mortality, need for Fontan takedown, use of mechanical circulatory support, pleural effusions and hospital stay were not significantly different between both groups., Conclusions: Patients with systemic RV demonstrate higher morbidity in the early postoperative course compared with patients with systemic LV anatomy and require intensified postoperative management to avoid postoperative Fontan failure., (© The Author 2016. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

Background and Objectives: Patients with severe influenza virus infection, multi-organ failure and organ replacement therapy may absorb and metabolize neuraminidase inhibitors differently. Systematic pharmacokinetic/pharmacodynamic clinical trials are currently lacking in this high-risk group. Inadequate dosing increases the risk of treatment failure and drug resistance, especially in severely ill patients with elevated virus loads. This study aims to explore the impact of organ replacement therapy on oseltamivir drug concentrations., Methods: Serial pharmacokinetic/pharmacodynamic measurements and Sieving coefficients were assessed in two patients with severe influenza B infection requiring organ replacement therapy., Results: Patient #1, a 9-year-old female with severe influenza B virus infection, biventricular assist device, and continuous veno-venous hemodiafiltration, received 75 mg oral oseltamivir twice-daily for 2 days, then intravenous oseltamivir with one-time renoprotective dosing (40 mg), followed by regular intravenous administration of 100 mg twice-daily. Plasma oseltamivir carboxylate concentrations were stable initially, but only regular administration of 100 mg resulted in virus load decline and clinical improvement. Patient #2, a 28-year-old female with influenza B virus infection requiring extracorporeal membrane oxygenation, received 75 mg oral oseltamivir twice-daily, resulting in erratic oseltamivir blood concentrations. In both patients, drug concentrations remained well within safety margins., Conclusions: In severe cases with multi-organ failure, administration of 100 mg intravenous oseltamivir twice-daily provided reliable drug concentrations, as opposed to renoprotective and oral dosing, thereby minimizing the risk of treatment failure and drug resistance. Evidence-based pediatric dosing recommendations and effective intravenous antiviral treatment modalities are needed for intensive care patients with life-threatening influenza disease.

Background: A novel formulation of bosentan was evaluated in children with pulmonary arterial hypertension (PAH) in FUTURE-1, which characterized its pharmacokinetic and clinical profile. The subsequent phase III, open-label, long-term extension study, FUTURE-2, aimed to provide long-term tolerability, safety and exploratory efficacy data., Methods: Children (≥2 and <12 years) with idiopathic or heritable PAH, who completed 12-week treatment in FUTURE-1 and for whom bosentan was considered beneficial were enrolled, and continued to receive bosentan 4 mg/kg twice-daily, which could be down-titrated to 2mg/kg if not tolerated. Safety and tolerability were evaluated via treatment-emergent adverse events (AEs), serious AEs, growth, and laboratory measurements. Exploratory efficacy endpoints included time to PAH worsening and long-term survival. All analyses were conducted on pooled data of both studies., Results: 36 patients were enrolled in FUTURE-1 and 33 continued in FUTURE-2. The overall median duration of exposure to bosentan was 27.7 (range 1.9-59.6) months. Treatment-emergent AEs occurred in 32 (88.9%) patients; AEs considered treatment-related in 15 (41.7%) patients. Of 51 serious AEs, three were considered treatment-related: two incidences of reported PAH worsening and one of autoimmune hepatitis. Six deaths occurred; none were considered treatment-related. Kaplan-Meier event-free estimates of PAH worsening were 78.9% and 73.6% at 2 and 4 years, respectively., Conclusions: The pediatric bosentan formulation was generally well tolerated, its safety profile comparable to that of the adult formulation when used in children. The results are in line with the efficacy profile of bosentan in previous pediatric and adult PAH studies of shorter duration., (Copyright © 2015. Published by Elsevier Ireland Ltd.)

Objective: We describe a repertoire of repair techniques according to type of Ebstein's anomaly to correct tricuspid valve (TV) incompetence, and report long-term ventricular function and functional outcomes., Methods: Sixty-eight patients (mean age, 26.9 ± 7.3 years) with Ebstein's anomaly (type A, n = 21; type B, n = 23; type C, n = 15; type D, n = 9) underwent correction of TV incompetence under normothermic cardiopulmonary bypass. The atrialized ventricle, TV, and subvalvar apparatus were inspected to analyze the precise morphology and determine which leaflet was the most mobile. Various repair strategies (anterior and/or posterior annulorrhapy, Sebening stitich, double-orifice valve technique, with bidirectional Glenn anastomosis if necessary) were used according to the presenting morphology and applied according to the type of Ebstein's anomaly. In all, the atrialized right ventricle (RV) was incorporated into the contractile RV by partial closure of the natural annulus using the most mobile leaflet for valve competence., Results: The mean duration of follow-up was 13.25 ± 1.3 years (median, 9.34 years; range, 1-24 years). The mean New York Heart Association class improved from 3.4 to 1.3 (P < .001). The mean severity of TV incompetence was reduced from 3.2 to 1.3 (P < .001). Exercise tolerance tests demonstrated improved maximal oxygen uptake from a mean of 15 ± 7.8 ng/kg/min preoperatively to a mean of 24.9 ± 2.0 ng/kg/min postoperatively (P < .02). Displacement tissue Doppler imaging was used to evaluate overall cardiac performance of the RV and left ventricle and interventricular septum. The mean basal, middle, and apical ventricular strain improved significantly from preoperative values of 18.08%, 15.6%, and 13.9%, respectively to postoperative values of 25.7% (P < .011), 23.7% (P < .001), and 19.36% (P < .05), respectively. Freedom from reoperation was 100% at 1 year, 98.3% at 5 years, and 92.9% at 20 years. Early mortality was 2.9%, and late mortality was 5.8%. The overall survival rate was 97.6% at 30 days, 92.7% at 5 years, and 91.26% at 20 years., Conclusions: The various repair techniques, all of which preserve the atrialized chamber and are used individually according to morphology, provide satisfactory long-term ventricular function and functional outcome even in severe types of Ebstein's anomaly., (Copyright © 2015 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.)

Objectives: Therapeutic hypothermia (TH) is an acknowledged strategy for neuroprotection for patients suffering from hypoxic-anoxic brain injury (HAI). Albeit similar pathomechanisms of HAI for both brain and heart, moderate TH (32-34°C) has not been established as a heart-protective measure. Therefore, we investigated the cardioprotective effects of moderate TH on oxygen-glucose deprivation/re-oxygenation (OGD/R)-induced injury in HL-1 cardiomyocytes., Methods: Cardiac OGD/R injury was induced by exposing HL-1 cardiomyocytes to 0.2% oxygen in serum/glucose-free medium for 6h. OGD injured cells were subsequently re-oxygenated with 21% oxygen in complete medium. Two hypothermic protocols were investigated: Post-OGD cooling to 33.5°C for 24 h initiated at the start of re-oxygenation and intra-OGD cooling to 33.5°C for 24 h initiated after 3 h of OGD and maintained throughout the re-oxygenation phase. Cell viability was determined by LDH and cTnT releases. Mitochondria dysfunction was evaluated by intracellular ATP content and cellular metabolic activity was accessed by MTT reduction. Activation of caspase 3 was analyzed by Western blot., Results: OGD/R-induced injury resulted in increased cell death (higher LDH and cTnT releases), mitochondrial impairment (decreased ATP content), and decreased cellular metabolic activity (decreased MTT reduction). Only intra-OGD cooling attenuated both OGD and OGD-R-induced injuries (significantly decreased LDH and cTnT releases and increased ATP contents and MTT reduction). Furthermore, caspase 3 activation was abated by intra-OGD cooling. No protective effects were observed by post-OGD cooling., Conclusions: Moderate TH initiated during OGD is a promising intervention for the protection of cardiomyocytes from OGD/R-induced injury. The attenuation of mitochondrial dysfunction and apoptosis by intra-OGD cooling are beneficial effects of hypothermia-induced cardioprotection, resulting in minimized myocardial cell death after OGD and OGD-R-induced injuries., (Copyright © 2014 Elsevier Inc. All rights reserved.)

Background: The pediatric Berlin Heart (BH) EXCOR device provides mechanical circulatory support as a bridge to transplantation or recovery in children. Despite the availability of various pump sizes, information on the impact of pump size on clinical outcome is still lacking. We aimed to evaluate whether pump size in relation to body surface area (BSA) has an impact on clinical outcome., Methods: Children requiring implantation of a BH between 2000 and 2013 were included in this retrospective study. Primary end-points were events leading to BH explantation (transplantation, recovery or death) and the secondary end-point was occurrence of thromboembolic events. Patients were categorized into three groups according to BH stroke volume per BSA: optimal (30 to 50 ml/m(2)); small (<30 ml/m(2)); and large (>50 ml/m(2))., Results: Eighty children (median age 2.2 years, median BSA 0.50 m(2)) underwent BH implantation. Fifty-five (69%) children had an optimally sized pump implanted, whereas 8 children (10%) had small pump and 17 (21%) large pump implantation. Overall survival rate was 69%. Weaning was possible in 15 children (19%), and 39 children (49%) were transplanted. Mortality, myocardial recovery and transplantation were not related to age, BSA or pump size. Thromboembolic events occurred significantly more frequently in children treated with large pumps., Conclusions: The broad range of body sizes in children from newborns to adolescents requires a wide choice of appropriately sized devices. Large pump size in relation to BSA is an independent risk factor for occurrence of thromboembolic events., (Copyright © 2014 International Society for Heart and Lung Transplantation. Published by Elsevier Inc. All rights reserved.)

Background: The objective of this prospective study was to assess the efficacy of exercise training as add-on to medical therapy in patients with congenital heart disease associated pulmonary arterial hypertension (CHD-APAH)., Methods: Patients with invasively confirmed CHD-APAH received in-hospital exercise training for 3 weeks and continued at home. Efficacy parameters were evaluated at baseline, after 3 and 15 weeks. Medical treatment remained unchanged. Worsening events and survival rate were assessed in a follow-up period of 21 ± 14 months., Results: Twenty consecutive CHD-APAH patients (16 female, 4 male, mean pulmonary arterial pressure 60 ± 23 mm Hg) were included. Patients significantly improved the mean distance walked in 6 min compared to baseline by 63 ± 47 m after 3 weeks (p<0.001) and by 67 ± 59 m after 15 weeks (p=0.001). Quality of life-score (p=0.05), peak oxygen consumption (p=0.002) and maximal workload (p=0.003) improved significantly by exercise training after 15 weeks. The 1- and 2-year survival rates were 100%, the transplantation-free survival rate was 100% after 1 year and 93% after 2 years., Conclusion: Exercise training as add-on to medical therapy may be effective in patients with CHD-APAH and improved work capacity, quality of life and further prognostic relevant parameters. It was associated with an excellent long-term survival. Further randomized controlled studies are needed to confirm these results., (Copyright © 2012 Elsevier Ireland Ltd. All rights reserved.)

Background: This report aims to introduce the extraanatomic bypass technique to treat the midaortic syndrome and to document its long-term effectiveness and durability., Methods: Fourteen patients (mean age, 6.7 ± 3.76 years; range 8 months to 11 years) received diagnoses of midaortic syndrome, characterized by severe narrowing of the abdominal aorta with involvement of the renal and visceral branches. Angiography showed variable lengths of high-grade midaortic stenosis, with 7 children having visceral artery involvement and 9 having renal artery involvement. All children were hypertensive (mean blood pressure, 165 ± 15.7 mm Hg). Three had had previous nephrectomies. Six patients had had previous percutaneous transluminal renal artery angioplasties. The midaortic obstruction was relieved by descending abdominal aorta bypass (left thoracoabdominal approach) and by an ascending abdominal aorta bypass (median sternotomy and transabdominal approach) in 12 patients. No visceral artery revascularization was done., Results: There was a considerable blood pressure reduction in all patients and relief of intermittent claudication in 6 affected patients. One patient had a bilateral renal artery bypass 2 weeks postoperatively because of recurrence of renal hypertension. At a mean follow-up time of 5.8 ± 1.36 years (range, 9 months to 15 years), there was no further reoperation nor mortality. Twelve patients had complete relief of hypertension, and 2 had mild hypertension. All patients have normal renal function and no signs or symptoms of visceral malperfusion. Growth and development have proceeded normally. Follow-up magnetic resonance tomography showed patent grafts without any strictures., Conclusions: Extraanatomic bypass provides very effective and long-term relief of hypertension and any malperfusion in midaortic syndrome., (Copyright © 2013 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: We analyzed risks for severe morbidity in the early period after extracardiac Fontan operation., Methods: Between November 1995 and May 2011, 140 patients (median age, 3.8 years) underwent extracardiac Fontan operation. We assumed as preoperative risk factors systemic right ventricle (n=51), heterotaxia (n=25), arterial oxygen saturation less than 75% (n=22), and adult age (>16 years, n=20) at time of surgery. Prolonged cardiopulmonary bypass time of longer than 120 minutes (n=30) and use of cardioplegia (n=26) were analyzed as intraoperative risks., Results: Heterotaxia was revealed as a risk factor for postoperative prolonged inotropic support, acute renal failure, prolonged mechanical ventilation, prolonged pleural effusions, and tachyarrhythmias. With the exception of pleural effusions, the same held true for right ventricle morphology. Low preoperative arterial oxygen saturation was found to be associated with an increased risk of prolonged inotropic support, acute renal failure, and prolonged mechanical ventilation. Adult age was identified as a risk factor for acute renal failure. Of the intraoperative factors, prolonged cardiopulmonary bypass time longer than 120 minutes was a risk factor for acute renal failure and prolonged pleural effusions, whereas use of cardioplegia was associated with an increased risk of prolonged inotropic support, prolonged mechanical ventilation, acute renal failure, and tachyarrhythmias. Multivariate analysis demonstrated heterotaxia, right ventricular morphology, and low preoperative arterial oxygen saturation to be independent risk factors for postoperative prolonged inotropic support and prolonged mechanical ventilation., Conclusions: Patients with heterotaxia, systemic right ventricle, and low preoperative arterial oxygen saturation are still at high risk for early Fontan failure after extracardiac Fontan operation and require special management for optimal outcome., (Copyright © 2013 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: The degree of involvement of left ventricular inflow tract obstruction is the predominant factor determining outcome in Shone's anomaly. In this series of patients with Shone's anomaly, we evaluated the impact of mitral valve (MV) repair strategies performed to correct the components of this anomaly on growth of the valve and long-term functional outcome in children., Methods: In the last 25 years, 45 children, mean age 5.16 ± 5.0 years (median, 3.9; range, 2 months-16.8 years), underwent surgical correction of Shone's anomaly. Coarctation of the aorta was found in 40%, subaortic stenosis due to fibromuscular hypertrophy was found in 55%, and subvalvar membrane was found in 66% of these patients. Left ventricular inflow tract obstruction was brought about by fused commissures with dysplastic and shortened chordae in 53.3%, valve hypoplasia in 11.1%, supravalvar mitral ring in 100%, and parachute valve in 17.8 of patients%., Results: Various repair strategies were performed according to the presenting morphologic characteristics in patients with either previously corrected or concomitant correction of the left-sided obstructive lesions. Mean duration of follow-up was 17.5 ± 1.5 years. Freedom from reoperation was 52.8% ± 11.8%, wherein 23 patients underwent repeated MV repair and 1 patient underwent MV replacement after failed attempts at repair. The cumulative survival rate was 70.3% ± 8.9% at 15 years. Severity and type of mitral abnormalities, left ventricular outflow tract lesions, and pulmonary hypertension are risk factors for reoperation and mortality (p < 0.05)., Conclusions: Repair allowed growth of the MV. Long-term outcome of MV repair in Shone's anomaly is related to the degree that the obstructive lesions can be relieved., (Copyright © 2013 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

The 2009 European Guidelines on Diagnosis and Treatment of Pulmonary Hypertension have been adopted for Germany. The guidelines contain detailed recommendations on the diagnosis of pulmonary hypertension (PH). However, the practical implementation of the European Guidelines in Germany requires the consideration of several country-specific issues and already existing novel data. This requires a detailed commentary to the guidelines, and in some aspects an update already appears necessary. In June 2010, a Consensus Conference organized by the PH working groups of the German Society of Cardiology (DGK), the German Society of Respiratory Medicine (DGP) and the German Society of Pediatric Cardiology (DGPK) was held in Cologne, Germany. This conference aimed to solve practical and controversial issues surrounding the implementation of the European Guidelines in Germany. To this end, a number of working groups was initiated, one of which was specifically dedicated to the treatment of pulmonary arterial hypertension (PAH). This commentary describes in detail the results and recommendations of the working group on treatment of PAH which were last updated in October 2011., (Copyright © 2011 Elsevier Ireland Ltd. All rights reserved.)

Background: Pediatric ventricular assist devices may be superior to extracorporeal membrane oxygenation in some respects, especially for medium- and long-term cardiac support. We present our nearly 20-year experience with pediatric ventricular assist devices., Methods: Between 1990 and April 2009, Berlin Heart EXCOR (Berlin Heart AG, Berlin, Germany) was implanted in 94 children. Patients were compared according to period of treatment: group I, implantation between 1990 and 2001 (n = 45), and group II, implantation since 2002 (n = 49)., Results: Preoperative serum creatinine (1.2 vs 0.7 mg/dL, P = .002) and bilirubin (1.5 vs 1 mg/dL, P = .002) were lower in period II, and fewer patients were artificially ventilated before surgery (26 vs 13, P = .002). In period I, more patients were supported with biventricular assist devices (64% vs 22.5%, P < .001). Median time on support was shorter in period I (10 vs 40 days, P < .001). Success (weaning from system or heart transplant) was achieved in 49% and 69%, respectively (P = .043). Whereas in period I 17% of children younger than 1 year were discharged home after transplant or weaning, rate during period II was 93% (P < .001), in particular because of improvement in discharge rate of patients with postcardiotomy heart failure (13% vs 50%). Rates of pump exchange for thrombus formation were 0.029/d in period I and 0.014/d in period II (P = 0.003)., Conclusions: Recent results show significant improvements in survival and discharge rate, especially for children younger than 1 year. Pediatric Berlin Heart EXCOR ventricular assist device may provide a safe mechanical support strategy in children with cardiogenic shock., (Copyright © 2011. Published by Mosby, Inc.)