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Studies assessing the long-term outcome after heart transplantation HTX in patients with cardiomyopathy (CM) in the paediatric age range are rare. The aim of this study was to determine the survival rate of children with CM undergoing HTX and to analyse how aetiology of cardiomyopathy influenced morbidity and mortality. We retrospectively analysed the medical records of children, who were transplanted in our centre between June 1988 and October 2019. 236 heart transplantations were performed since 1988 (9 re-transplants). 98 of 227 patients (43.2%) were transplanted because of CM. Survival rates were 93% after 1, 84% after 10 and 75% after 30 years. Overall, the aetiology of CM could be clearly identified in 37 subjects (37.7%). This rate increased up to 66.6% (12/19) by applying a comprehensive diagnostic workup since 2016. The survival rate was lower (p &lt, 0.05) and neurocognitive deficits were more frequent (p = 0.001) in subjects with systemic diseases than in individuals with cardiac-specific conditions. These data indicate that the long-term survival rate of children with CM after HTX in experienced centers is high. A comprehensive diagnostic workup allows unraveling the basic defect in the majority of patients with CM undergoing HTX. Aetiology of CM affects morbidity and mortality in subjects necessitating HTX.

Comprehensive stage II is the advanced surgical part of the staged treatment of a newborn with hypoplastic left heart syndrome (HLHS) palliated initially by a Giessen-Hybrid approach. We report an almost ideal course following comprehensive stage II operation with focus on postoperative intensive care strategy. Following a short introduction of the postnatally performed Giessen-Hybrid approach, in which the surgical part is focused on bilateral pulmonary banding and duct stenting as well as manipulation of the atrial septum is postponed to transcatheter approach, it should be emphasized, that the quality of inter-stage I is eminently important for the success of the following comprehensive stage II. Furthermore, the interplay of the responsible surgeon, anesthesiologist, cardiologist and intensivist is mandatory for working as a team with a similar pathophysiological background. Presupposed a sophisticated surgical and anesthesiologic management, the immediate post-operative intensive care is crucial for the patient's final outcome, not only in terms of mortality but even morbidity (long-term neurological condition). Detailed treatment strategies are presented by pathophysiological reasonable hypotheses and the current pharmacological knowledge. Aiming to improve systemic and regional oxygen delivery and lowering oxygen consumption, as a sine qua none for a favorable patient's outcome.

Objectives:Mechanical circulatory support for pediatric heart failure patients with the Berlin Heart EXCOR ventricular assist system is the only approved and established bridging strategy for recovery or heart transplantation. In recent years, the burden of thromboembolic events has led to modifications of the recommended antithrombotic therapy. Therefore, we aimed to assess modifications of antithrombotic practice among the European EXCOR Pediatric Investigator Group members.Methods:We sent a questionnaire assessing seven aspects of antithrombotic therapy to 18 European hospitals using the EXCOR device for children. Returned questionnaires were analyzed and identified antithrombotic strategies were descriptively compared to “Edmonton protocol” recommendations developed for the US EXCOR pediatric approval study.Results:Analysis of 18 received surveys revealed substantial deviations from the Edmonton protocol, including earlier start of heparin therapy at 6–12 h postoperatively and in 50% of surveyed centers, monitoring of heparin effectiveness with aPTT assay, administering vitamin K antagonists before 12 months of age. About 39% of centers use higher international normalized ratio targets, and platelet inhibition is changed in 56% including the use of clopidogrel instead of dipyridamole. Significant inter-center variability with multiple deviations from the Edmonton protocol was discovered with only one center following the Edmonton protocol completely.Conclusion:Current antithrombotic practice among European EXCOR users representing the treatment of more than 600 pediatric patients has changed over time with a trend toward a more aggressive therapy. There is a need for systematic evidence-based evaluation and harmonization of developmentally adjusted antithrombotic management practices in prospective studies toward revised recommendations.

s of the 51st Workshop for Pediatric Research 51st Workshop for Pediatric Research Gottingen, Germany 16-17 April 2015 This supplement has not been sponsored. Meeting abstracts Background Dilated cardiomyopathy (DCM) is a leading cause of cardiac death in children. Approximately 30% of children die or need cardiac transplantation in the first year after diagnosis. We established a protocol to improve the outcome in this high-risk population. Patients and methods We present our experience in 21 patients (mean age 8 month, mean weight 6 kg) treated in our institution from 2006 to 2015. The patients were diagnosed with DCM with a highly impaired function of the LV (mean EF 17%) and a conserved function of the RV (mean EF 52%). Our protocol for medical enhancement of left ventricular recovery in association with pulmonary artery banding involves the use of a highly specific β1-blocker, an angiotensin-converting enzyme inhibitor and an aldosterone antagonist. Our therapy aims to reduce oxygen consumption and improve oxygen delivery. Heart rate control is the most important goal; therefore clonidine is given after surgical procedure and digoxin in long-term treatment if heart rate remains high despite adequate β1-blocker therapy. To improve oxygen delivery our goal is to archive haemoglobin levels of 12-14 g/dl, therefore Erythropoietin is given as long term treatment. Additional treatments include supplementation of carnitine, coenzyme Q, riboflavin or thiamine. Results At a mean follow up of 36 month (range 2-120) freedom from death was 91% and freedom from heart transplantation was 85%. Surviving patients showed a significant improvement in left ventricular ejection fraction (from 17 ± 6 to 50 ± 11%) and LVEdD (z-score from +7 ± 2 to +1.7 ± 1.9). The levels of BNP improved significantly (from 3222 ± 2756 to 70 ± 56 pg/ml). Conclusion Our data suggest that the medical and surgical approach described may result in a markedly improved medium-term outcome in children with DCM. Further studies are required to evaluate the long-term-outcome of these patients.