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Prenatal prediction of postnatal univentricular versus biventricular circulation in patients with borderline left ventricle (bLV) remains challenging. This study investigated prenatal fetal echocardiographic parameters and postnatal outcome of patients with a prenatally diagnosed bLV. We report a retrospective study of bLV patients at four prenatal centers with a follow-up of one year. BLV was defined as z-scores of the left ventricle (LV) between -2 and -4. Single-ventricle palliation (SVP), biventricular repair (BVR), and no surgical or catheter-based intervention served as the dependent outcome. Prenatal ultrasound parameters were used as independent variables. Cut-off values from receiver operating characteristic curves (ROC) were determined for significant discrimination between outcomes. A total of 54 patients were diagnosed with bLV from 2010 to 2018. All were live births. Out of the entire cohort, 8 (15 %) received SVP, 34 (63 %) BVR, and 12 (22 %) no intervention. There was no significant difference with regard to genetic or extracardiac anomalies. There were significantly more patients with endocardial fibroelastosis (EFE) in the SVP group compared to the BVR group (80 % vs. 10 %), (p 0.001). Apex-forming LV (100 % vs. 70 %) and lack of retrograde arch flow (20 % vs. 80 %) were associated with no intervention (p 0.001). With respect to BVR vs. SVP, the LV sphericity index provided the highest specificity (91.7 %) using a cutoff value of ≤ 0.5. The majority of bLV patients maintained biventricular circulation. EFE, retrograde arch flow, and LV sphericity can be helpful parameters for counseling parents and further prospective studies can be developed.ZIEL: Die pränatale Vorhersage eines postnatal univentrikulären versus biventrikulären Kreislaufs bei Föten mit grenzwertigem linkem Ventrikel (bLV, “borderline left ventricle”) bleibt eine Herausforderung. Diese Studie untersuchte pränatale fetale echokardiografische Parameter und den postnatalen Outcome von Patienten mit einer pränatal diagnostizierten bLV. Wir berichten über eine retrospektive Studie von Föten mit bLV an 4 Pränatalzentren mit einer Nachverfolgung von einem Jahr. BLV wurde definiert als z-Scores des linken Ventrikels (LV) zwischen –2 und –4. Als abhängiger Outcome dienten die Einzelventrikel-Palliation (SVP), die biventrikuläre Korrektur (BVR) und keine chirurgische oder Katheter-basierte Intervention. Die pränatalen Ultraschallparameter wurden als unabhängige Variablen verwendet. Die Cut-off-Werte aus den Receiver-Operating-Characteristic (ROC) -Kurven wurden für eine signifikante Diskriminierung zwischen den Outcomes ermittelt. Von 2010–2018 wurden insgesamt 54 Patienten mit bLV diagnostiziert. Alle waren Lebendgeburten. Von der gesamten Kohorte erhielten 8 (15 %) SVP, 34 (63 %) BVR und 12 (22 %) keine Intervention. Es gab keinen signifikanten Unterschied in Bezug auf genetische oder extrakardiale Anomalien. Es gab signifikant mehr Patienten mit endokardialer Fibroelastose (EFE) in der SVP- im Vergleich zu BVR-Gruppe (80 % vs. 10 %; p 0,001). Ein an der Herzspitzenbildung beteiligter LV (100 % vs. 70 %) und fehlender retrograder Bogenfluss (20 % vs. 80 %) waren mit keiner Intervention assoziiert (p 0,001). Für die Differenzierung von BVR vs. SVP lieferte der LV-Sphärizitätsindex bei einem Cut-off-Wert von ≤ 0,5 die höchste Spezifität (91,7 %). Die Mehrheit der bLV-Patienten hielt einen biventrikulären Kreislauf aufrecht. EFE, retrograder Bogenfluss und LV-Sphärizität können hilfreiche Parameter bei der Beratung der Eltern und der Entwicklung weiterer prospektiver Studien sein.

Objectives To analyse prenatal parameters predicting biventricular (BV) outcome in pulmonary atresia with intact ventricular septum/critical pulmonary stenosis (PAIVS/CPS). Methods We evaluated 82 foetuses from 01/08 to 10/18 in 3 centres in intervals 1 ( 30 weeks). Results 61/82 (74.4%) were livebirths, 5 (8.2%) lost for follow-up, 3 (4.9%) had compassionate care leaving 53 (64.6% of the whole cohort and 86.9% of livebirths) with intention to treat. 9 died, 44/53 (83.0%) survived. 24/38 (63.2%) with information on postnatal outcome had BV outcome, 14 (36.8%) non-BV outcome (2 × 1.5 circulation). One with BV outcome had prenatal valvuloplasty. Best single parameter for BV outcome was tricuspid/mitral valve (TV/MV) ratio (AUC 0.93) in intervals 2 and 3 (AUC 0.92). Ventriculo-coronary-arterial communications (VCAC) were present in 11 (78.6%) in non-BV outcome group vs. 2 (8.3%) in BV outcome group (p 2.5 m/s was present in BV outcome group in75.0% (18/24) vs. 14.3% (2/14) in non-BV outcome group. Including the most predictive markers (VCAC presence, TI- Vmax 1 criterion was fulfilled in all cases. After recently published criteria for foetal intervention, only 4/9 (44.4%) and 5/14 (35.7%) in our interval 2 + 3 with predicted non-BV outcome would have been candidates for intervention. Two (1 × intrauterine intervention) in interval 2, two in interval 3 reached BV outcome and one 1.5 circulation without intervention. Conclusion TV/MV ratio as simple parameter has high predictive value. After our score, non-BV outcome was correctly predicted in all cases. Criteria for foetal intervention must further be evaluated.

BACKGROUND: The therapeutic success in patients with congenital heart disease (CHD) leads to a growing number of adults with CHD (adult CHD [ACHD]) who develop end-stage heart failure. We aimed to determine patient characteristics and outcomes of ACHD listed for heart transplantation. METHODS: Using data from all the patients with ACHD in 20 transplant centers in the Eurotransplant region from 1999 to 2015, we analyzed patient characteristics, waiting list, and post-transplantation outcomes. RESULTS: A total of 204 patients with ACHD were listed during the study period. The median age was 38 years, and 62.3% of the patients were listed in high urgency (HU), and 37.7% of the patients were in transplantable (T)-listing status. A total of 23.5% of the patients died or were delisted owing to clinical worsening, and 75% of the patients underwent transplantation. Median waiting time for patients with HU-listing status was 4.18 months and with T-listing status 9.07 months. There was no difference in crude mortality or delisting between patients who were HU status listed and T status listed (p = 0.65). In multivariable regression analysis, markers for respiratory failure (mechanical ventilation, hazard ratio [HR]: 1.41, 95% CI: 1.11-1.81, p = 0.006) and arrhythmias (anti-arrhythmic medication, HR: 1.42, 95% CI: 1.01-2.01, p = 0.044) were associated with a higher risk of death or delisting. In the overall cohort, post-transplantation mortality was 26.8% after 1 year and 33.4% after 5 years. CONCLUSIONS: Listed patients are at high risk of death without differences in the urgency of listing. Respiratory failure requiring invasive ventilation and possibly arrhythmias requiring anti-arrhythmic medication indicate worse outcomes on waiting list. (C) 2020 International Society for Heart and Lung Transplantation. All rights reserved.

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.

The aim of our retrospective evaluation was to compare the outcome of patients with prenatal and postnatal diagnosis of Tetralogy of Fallot (TOF) and to analyze prenatal echocardiographic parameters predicting intervention within 30 days postnatal. We evaluated 142 patients in our pediatric heart center and prenatal diagnosis center and prenatal practice Praenatal plus in Cologne between 01/08-06/16. Within the prenatal diagnosis group, 6/74 fetuses (8.1 %) had TOF with pulmonary atresia (TOF-PA), and 6 (8.1 %) had absent pulmonary valve syndrome (TOF-APVS). 14 (18.9 %) had an abnormal karyotype including 9/14 (64.3 %) with microdeletion 22q11.2. 25 (33.8 %) had extracardiac malformation. 4 (5.4 %) had agenesis of ductus arteriosus (DA), 22 (29.7 %) had right aortic arch (RAA) and 9 (12.2 %) had major aortopulmonary collateral arteries (MAPCAs). Within the postnatal diagnosis group, no patient had TOF-PA, 4/68 (5.9 %) had TOF-APVS. 12 (17.6 %) had extracardiac malformations, 9 (13.2 %) had an abnormal karyotype including 2/9 with microdeletion 22q11.2. 10 (14.7 %) had RAA, 9 (13.2 %) had MAPCAs. There were no cases with agenesis of DA. Increasing z-score values of the left/right pulmonary artery (LPA/RPA) prenatally were associated with a lower probability for early postnatal intervention (RPA: p = 0.017; LPA: p = 0.013). Within the prenatal diagnosis group, 12 of 41 (29.3 %) live-born patients with follow-up and intention to treat needed early intervention versus 7 (10.3 %) in the postnatal diagnosis group (p = 0.02). Within the postnatal diagnosis group, there were no deaths, while 2 (4.9 %) post-intervention deaths occurred in the prenatal diagnosis group. There are no significant differences concerning post-intervention survival in the prenatal diagnosis group versus the postnatal diagnosis group. Complex cases may be underrepresented in the postnatal diagnosis group. Smaller RPA/LPA values prenatally seem to be associated with early postnatal intervention.ZIEL: Ziel unserer retrospektiven Auswertung war der Outcome-Vergleich von Patienten mit pränatal und postnatal diagnostizierter Fallot-Tetralogie (TOF) sowie die Auswertung fetaler Echokardiografie-Parameter im Hinblick auf Prädiktion einer Intervention innerhalb 30 Tage postnatal. Ausgewertet wurden 142 Patienten, die von 01/08–06/16 in unserem Kinderherz-Zentrum, unserer Pränataldiagnostik oder in der Praxis Praenatal plus in Köln vorstellig waren. In der Pränatal-Diagnose-Gruppe waren 6 von 74 (8,1 %) mit TOF und Pulmonalatresie (TOF-PA), 6 (8,1 %) mit absent-pulmonary-valve-syndrome (TOF-APVS). 14 (18,9 %) hatten einen auffälligen Karyotyp, darunter 9/14 mit Mikrodeletion 22q11.2 (64,3 %). 25 (33,8 %) hatten extrakardiale Fehlbildungen. 4 (5,4 %) hatten eine Ductus arteriosus (DA) -Agenesie, 22 (29,7 %) einen rechten Aortenbogen (RAA), 9 (12,2 %) hatten aortopulmonale Kollateralen (MAPCAs). In der Postnatal-Diagnose-Gruppe waren keine Patienten mit TOF-PA, 4 von 68 (5,9 %) hatten TOF-APVS. 12 (17,6 %) hatten extrakardiale Fehlbildungen, 9 (13,2 %) hatten einen auffälligen Karyotyp, darunter 2/9 mit Mikrodeletion 22q11.2. 10 (14,7 %) hatten ein RAA, 9 (13,2 %) hatten MAPCAs. Es gab keine DA-Agenesien. Pränatal höhere z-Werte der linken/rechten Pulmonalarterie (LPA/RPA) waren mit einer niedrigeren Wahrscheinlichkeit für eine frühe postnatale Intervention assoziiert (RPA: p = 0,017; LPA: p = 0,013). Innerhalb der Pränatal-Diagnose-Gruppe benötigten 12 von 41 (29,3 %) Lebendgeburten mit follow-up und Intention-to-treat eine frühe Intervention versus 7 von 68 (10,3 %) innerhalb der Postnatal-Diagnose-Gruppe (p = 0,02). In der Postnatal-Diagnose-Gruppe gab es keine Todesfälle, in der Pränatal-Diagnose-Gruppe gab es 2/41 (4,9 %) Todesfälle postinterventionell. Es zeigten sich keine Unterschiede hinsichtlich post-interventionellem Überleben in der Pränatal-Diagnose-Gruppe versus Postnatal-Diagnose-Gruppe. Komplexe Fälle könnten in der Postnatal-Diagnose-Gruppe unterrepräsentiert sein. Pränatal kleinere RPA/LPA-Werte scheinen mit einer frühen postnatalen Intervention assoziiert zu sein.

Background. The absolute number of patients presenting with failure of the hemi-Fontan or Fontan circulation for cardiac transplantation (cTx) will continuously rise. We aimed to analyze the intraoperative differences in patients undergoing cTx for a failing hemi-Fontan or Fontan circulation (group A) with those undergoing cTx for cardiomyopathy and congenital heart disease (group B). Methods. Data of patients undergoing cTx during a 10-year period were compared between group A and group B patients. Results. cTx was performed in 83 patients (group A, n = 21; group B, n = 62). Surgical times including median incision-suture time (549 minutes vs 386 minutes, P < .05) and median organ ischemia time (305 minutes vs 233 minutes, P < .05) were longer in group A patients. After weaning off cardiopulmonary bypass a higher median modified Vasoactive Inotropic Score (16 vs 10, P < .05) was necessary in group A patients. During surgery more fresh frozen plasma (44 mL/kg vs 20 mL/kg, P < .05), platelet concentrates (20 mL/kg vs 14 mL/kg, P < .05), and coagulation factor concentrates were given in group A patients. Mortality during the first 90 days after cTx was higher in group A (23.8% vs 6.5%, P < .05). Conclusion. Patients undergoing cTx for a failing hemi-Fontan or Fontan circulation are challenging. They require an intensive vasoactive and inotropic support; furthermore, special attention should be paid to the management of bleeding complications. cTx for this group of patients is associated with higher 90 days mortality.

BACKGROUND Neonatal management of patients with hypoplastic left heart syndrome and complex remains a challenging task, whereby the "hybrid" palliation is often reserved for high-risk patients as a "rescue" procedure. AIM This study documents the anesthetic challenges and potential complications associated with the Giessen hybrid stage I approach. METHODS The Giessen hybrid stage I approach is focused on surgical bilateral pulmonary artery banding. Retrospective perioperative data were analyzed. Contrary to a stable group A, inotropic treatment before surgery for treatment of postnatal shock classified patients as unstable (Group B). Clinical outcomes considered were inhospital mortality, duration of postoperative mechanical ventilation, postoperative time at the intensive care unit, perioperative vasoactive medication requirements, and red blood cell transfusion. RESULTS From June 1998 to December 2015, 185 patients were allocated to Group A (n = 165) and Group B (n = 20). The inhospital mortality was 2.2% with no difference between the groups. There was also no difference in the postoperative time on mechanical ventilation and the time in the intensive care unit. Vasoactive medication was more often required in Group B (100%) compared to Group A (19%). In Group B, more red blood cells were transfused 6.0 ± 8.3 vs 2.0 ± 5.8 mL/kg in Group A (P

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.

Previous investigations assessing the genetic cause of pediatric hypertrophic cardiomyopathy (HCM) found underlying genetic mutations in 50–60% of cases. The purpose of our study was to analyze whether this number can be augmented by applying next-generation sequencing and directing further diagnostics by discussing unsolved cases in a multidisciplinary board. 42 patients with the diagnoses of HCM made before age 18 years were treated in our center from 2000 to 2016. Genetic analysis was performed in 36 subjects, a genetic defect was detected in 29 (78%) patients. 15 individuals (42%) had pathogenic variants in genes encoding sarcomere proteins, and 5 (14%) in genes coding for components of the RAS/MAPK signaling pathway. 4 subjects (11%) had mutations in the GAA gene (Pompe disease), and 3 (8%) had Frataxin repeat expansions (Friedreich’s ataxia). One patient each showed a mutation in BAG3 and LMNA. Discussion of unsolved HCM cases after performing next-generation sequencing (28 genes) in an interdisciplinary board unraveled the genetic cause in 9 subjects (25%). A definite genetic diagnosis can be reached in nearly 80% with HCM of childhood onset. Next-generation sequencing in conjunction with a multidisciplinary cooperation can enhance the diagnostic yield substantially. This may be important for risk stratification, treatment planning and genetic counseling.

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.

Noonan syndrome with multiple lentigines (NSML) frequently manifests with hypertrophic cardiomyopathy (HCM). Recently, it was demonstrated that mTOR inhibition reverses HCM in NSML mice. We report for the first time on the effects of treatment with a rapamycin analog in an infant with LS and a malignant form of HCM. In the boy, progressive HCM was diagnosed during the first week of life and diagnosis of NSML was established at age 20 weeks by showing a heterozygous Q510E mutation in the PTPN11 gene. Immunoblotting with antibodies against pERK, pAkt, and pS6RP in fibroblasts demonstrated reduced RAS/MAPK and enhanced Akt/mTOR pathway activities. Because of the patient’s critical condition, everolimus therapy was started at age 24 weeks and continued until heart transplantation at age 36 weeks. Prior to surgery, heart failure improved from NYHA stage IV to II and brain natriuretic peptide values decreased from 9600 to

BACKGROUND: Decision-making when offered a donor heart for transplantation is complex, and supportive data describing outcomes according to acceptance or non-acceptance choices are sparse. Our aim was to analyze donor heart acceptance decisions and associated outcomes at a single center, and after subsequent acceptance elsewhere.METHODS: This investigation was a retrospective analysis of data obtained from the University of Vienna Medical Center and Eurotransplant centers for the period 2001 to 2015RESULTS: Our center accepted 31.8% (699 of 2,199) of donor hearts offered. Unlike other centers, the acceptance rate, with or without transplantation, did not increase over time. Of the donor hearts rejected by our center, 38.1% (572 of 1,500) were later accepted elsewhere. Acceptance rates were twice as high for donor hearts initially rejected for non-quality reasons (339 of 601, 56.4%) compared with initial rejection for quality reasons (233 of 899, 25.9%). Three-year patient survival rate was 79% at Vienna; for donor hearts initially rejected by Vienna for non-quality reasons or quality reasons, it was 73% and 63%, respectively (p CONCLUSIONS: A less restrictive policy for accepting donor hearts at our center, particularly regarding rejection for non-quality reasons or for positive virology, high catecholamine levels, longer ischemic time, or low ejection fraction, could expand our donor pool while maintaining good outcomes. (C) 2018 International Society for Heart and Lung Transplantation. All rights reserved.

Presented is a retrospective outcome study of a 15-year single institutional experience with a contemporary cohort of patients with hypoplastic left heart syndrome and complex that underwent a “Giessen Hybrid” stage I as initial palliation. Hybrid approach consisting of surgical bilateral pulmonary artery banding and percutaneous duct stenting with or without atrial septum manipulation was developed from a rescue approach to a first-line procedure. Comprehensive Aristotle score defined pre-operative condition. Fifteen-year follow-up mortality is reported as occurring within the staged univentricular palliation or before and after biventricular repair. Hybrid stage I was performed in 154 patients; 107 should be treated by single ventricle palliation, 33 by biventricular repair (BVR), 7 received heart transplantation, and 7 were treated by comfort care, respectively. Overall 34 children died. The Aristotle score (mean value 18.2 ± 3) classified for univentricular circulations in newborns did not have statistical impact on the outcome. Two patients died during stage I (1.2 %), and the interstage I mortality was 6.7 %, and stage II mortality 9 %, respectively. Stage III was up to now performed in 57 patients without mortality. At 1 year, the overall unadjusted survival of HLHS and variants was 84 % and following BVR 89 %, respectively. The Fifteen-year survival rate for HLHS and variants was 77 %, with no significant impact of birth weight of less than 2.5 kg. In conclusion, Hybrid stage I fulfilled the criteria of life-saving approach. In our institution, Hybrid procedure replaced Norwood-staged palliation with a considerable mid- and long-term survival rate. Considering interstage mortality close surveillance is mandatory.

Background Dilated cardiomyopathy (DCM) in childhood has a considerable morbidity and mortality and high incidence of heart transplantation. Pulmonary artery banding (PAB) has been proposed in patients with corrected transposition of the great arteries to retrain the sub-pulmonic left ventricle (LV) and to improve a failing sub-aortic right ventricle. We evaluated the short-term and medium-term effects of PAB in young patients with LVDCM. Methods A retrospective single-center observational study was performed to evaluate the possible benefits of a dilatable surgical PAB in infants and young children with LVDCM. Results Reported are 12 patients (10 infants, 2 toddlers) with LVDCM referred for heart transplant who received a surgical PAB. There were no hospital deaths. Clinical functional status improved in all patients. The pressure gradient across the PAB increased within 20 days from 28 ± 7 to 43 ± 15 mm Hg. The LV ejection fraction increased from 14.5% ± 5% pre-PAB to 27% ± 13% at hospital discharge and to 47% ± 10% at 3 to 6 months. The LV end-diastolic diameter (z-score) decreased ( p > 0.001) from 46 ± 6.1 (+7.0 ± 1.3) to 35 ± 15 mm (+3.0 ± 1.3) after 3 to 6 months and to 34 ± 15 mm (+1.3 ± 1.14) after a median age of 2 years (maximum 6.6 years), respectively. Plasma B-type natriuretic peptide levels decreased from 3431 ± 2610 to 288 ± 321 pg/ml at discharge and to 102 ± 96 pg/ml 22 months later. Eight children were subsequently de-banded by transcatheter technique and 6 of them are currently at Ross Heart Failure Classification for Children class I. Two patients, both with non-compaction DCM, deteriorated at 5 and 6 months after PAB debanding and finally died. Conclusion In young children with LVDCM and still-preserved right ventricular function, PAB led to an improvement of LV and mitral valve function by ventricular interaction.

BACKGROUND: Patients who require extracorporeal membrane oxygenation (ECMO) postsurgery for congenital heart disease (CHD) frequently experience severe bleeding episodes. Whereas recombinant-activated factor VII (rFVIIa) has proven efficacy in counteracting intractable hemorrhage in various scenarios, its use in patients on ECMO is limited by the increased risk for thrombotic events. METHODS: Between December 2004 and January 2006, ECMO was used in 10 pediatric patients following cardiac surgery, of whom seven were treated with rFVIIa because of intractable hemorrhage. Their medical records were reviewed with respect to variations in chest tube output and transfusion requirements, occlusion of or thrombus formation in the ECMO circuit and the occurrence of thromboembolic events. Outcome and rate of ECMO circuit occlusion were compared with historic controls. RESULTS: Three patients died, and four survived (none of the deaths was attributable to thrombus formation or bleeding). All patients were treated with aprotinin prior to and during rFVIIa therapy. Two patients developed an occlusion of the oxygenator, one after receiving co-medication with a FXIII concentrate, another after RBC transfusion in the ECMO system. In two patients, thrombus formation was observed in the ECMO system on inspection after discontinuation. Thromboembolic events were not observed. CONCLUSIONS: Recombinant-activated factor VII in a median dosage of 90 microg.kg(-1) was used in seven pediatric patients on ECMO. Rates of ECMO system occlusions and mortality did not differ from historic controls. Neither the reduction of chest tube output nor the blood product transfusion requirements did reach statistical significance.

The outcome of patients with hypoplastic left heart (HLH) is determined by many factors, particularly by the first-step palliative procedure in newborns undergoing the Norwood procedure, its Sano modification, or, rarely, through challenging biventricular repairs. Duct stenting combined with bilateral pulmonary artery banding (PAB) is a new method employed as an alternative first-step approach in a number of centers worldwide. We describe this interventional-surgical "hybrid approach" as an additional strategy for the treatment of newborns with HLH syndrome and HLH complex. Between 1998 and April, 2006, 58 newborns underwent ductal stenting and bilateral PAB. These patients underwent surgical bilateral PAB initially, followed by percutaneous duct stenting; the only exception to this were patients in whom duct stenting was performed as a rescue procedure. Various balloon-expandable and self-expandable stents with different widths and lengths were used during the 8-year period of this study. Balloon dilatation of the atrial septum was performed when indicated. This included 5 patients in whom the atrial septum was stented. Aortic arch reconstruction (AAR) combined with a bidirectional cavopulmonary connection (BCPC) was performed at a median age of 4.8 months (range, 2.6-7.5), and total cavopulmonary connection (TCPC) was performed at a median age of 3.1 years (range, 2.5-4). Nine patients were listed for heart transplantation (HTX) and transplanted with AAR when a donor heart was available. Depending on growth of left ventricular structures, biventricular repair (BVR) was performed at a median age of 7.1 months (range, 3.5-10). Overall, 8 of 58 patients (13.8%) treated by the transcatheter-surgical hybrid approach died during the study period. The mortality rate for duct stenting was 1.7% (l/58), and it was 1.7% for bilateral PAB as well. Twenty-seven patients received an AAR/BCPC; 2 of them died (7.4%). Additionally, 1 of 2 patients with AAR/BCPC died while on the waiting list for HTX, resulting in a total mortality rate of 11% with an actuarial survival rate of 89%. One patient is still awaiting AAR + BCPC. Three patients died while on the waiting list for HTX despite successful bilateral PAB and duct stenting. The 30-day mortality rate for TCPC (n = 11), HTX (n = 8), and 18 patients with BVR was 0. The actuarial survival rate for patients with BVR is 93%. Postnatal transcatheter-surgical hybrid palliation expands the surgical options for newborns with HLH. Using hybrid palliation, Norwood stage I operation can be avoided in the neonatal period, the waiting period for children scheduled for cardiac transplantation can be extended, and observation for left ventricular growth suitable for biventricular repair as well.

This retrospective study presents our operative results, mortality, and morbidity with regard to pulmonary artery growth and reinterventions on the pulmonary artery and aortic arch, including key features of our institutional standards for the 3-stage hybrid palliation of patients with hypoplastic left heart syndrome.Between June 1998 and February 2015, 182 patients with hypoplastic left heart structures underwent the Giessen hybrid stage I procedure. Among these, 126 patients with hypoplastic left heart syndrome who received a univentricular palliation or heart transplantation were included in the main analysis. Median age and body weight of patients at hybrid stage I were 6 days (0-237) and 3.2 kg (1.2-7), respectively. Comprehensive stage II operation was performed at 4.5 months (2.9-39.5), and Fontan completion was established at 33.7 months (21.1-108.2). Operative and interstage mortality, morbidity, growth and reinterventions on the pulmonary arteries, and long-term operative results of the aortic arch reconstruction were assessed.Median follow-up time after Giessen hybrid stage I palliation was 4.6 years (0-16.8). Operative mortality at hybrid stage I, comprehensive stage II, and Fontan completion was 2.5%, 4.9%, and 0%, respectively. Cumulative interstage mortality was 14.2%. At 10 years, the probability of survival is 77.8%. Body weight (2.5 kg) and aortic atresia had no significant impact on survival. McGoon ratio did not differ at comprehensive stage II and Fontan completion (P = .991). Freedom from pulmonary artery intervention was estimated to be 32.2% at 10 years. Aortic arch reinterventions were needed in 16.7% of patients; 2 reoperations on the aortic arch were necessary.In view of the early results and long-term outcome, the hybrid approach has become an alternative to the conventional strategy to treat neonates with hypoplastic left heart syndrome and variants. Further refinements are warranted to decrease patient morbidity.

In a prospective investigation of perioperative cardiac edema formation requiring a delayed sternal closure, we identified thrombin increase combined with a simultaneous decrease of factor XIII as a probable cause. After experimental studies additionally revealed that factor XIII could protect endothelial barrier function, we did another prospective randomized trial in which factor XIII or placebo was preoperatively substituted. The substitution finally showed distinct effects minimizing the incidence of myocardial swelling. Therefore, the clinical application of factor XIII may have a valuable therapeutic benefit in cases of leakage syndrome during extracorporeal circulation in congenital heart surgery.

The application of a mechanical cardiac-assist device is now a common procedure in modern cardiac surgery in patients with end-stage failure, whereas in pediatric patients it is still a great challenge. In the recent literature, a broad range of survival and weaning rates have been reported, depending on the variety of mechanical devices and the choice of patients with different conditions before implantation or if the device is used in emergency surgery. In this article, the authors report their experience with pediatric cardiac-assist devices and the perioperative anesthesia management in this group of patients.From 1997 to 2001, 11 infants and children were supported with a left and/or right ventricular-assist device. Diagnosis included myocarditis and complex cardiac malformations (hypoplastic left heart syndrome, tetralogy of Fallot with cardiomyopathy, and combined heart defects). The data sets of all patients were recorded using the online anesthesia record-keeping system NarkoData (IMESO GmbH, Hüttenberg, Germany). The program collects all perioperative data during surgery and during a stay in the PACU, including vital signs, administered drugs, as well as the data set of the German Society of Anesthesiology and Intensive Care Medicine.All patients were divided into 2 groups: group 1 = survivors and group 2 = nonsurvivors. A total of 5 patients were in group 1, and group 2 consisted of 6 patients. The duration of anesthesia in group 1 patients (173.2 +/- 95.1 minutes) was significantly (p0.05) shorter than in group 2 (631.1 +/- 258.8 minutes) as well as the amount of packed red cells (group 1= 540.5 +/- 150.3 mL, group 2 = 880.6 +/- 400.3 mL). Cardiopulmonary bypass before implantation of a VAD was necessary only in 2 patients from group 1, whereas 5 patients in group 2 were on pump during the procedure. The rate of aortic cross-clamping was also significantly lower in group 1 than in group 2 (p0.05).The surgical outcome depends on the patient's condition at the time of surgery. Emergency surgery, preoperative multiorgan failure, and the need for an extracorporeal circulation with aortic cross-clamping seem to predict a negative outcome in this group of patients.

Acute rejection of the donor heart is a major cause of mortality in infant heart transplant recipients. The early diagnosis of acute cardiac rejection (ACR) is crucial. Non-invasive methods have shown poor sensitivity in detecting rejection when compared to endomyocardial biopsies (EMB). We assessed troponin I as a new marker to diagnose cardiac rejection. Serum cardiac troponin I (cTNI) levels were retrospectively analysed in 25 heart transplant patients (ages, 2 wk to 13 yr; mean age, 3 months) presenting 36 acute rejections. In early post-operative rejection and initially elevated cTNI levels, rejection was associated with a second increase of serum cTNI concentrations in 21% of the patients (p = 0.15). If cTNI levels were in normal range before ACR an elevation was monitored in 59% of the rejection periods (p

Objective This article presents the long-term results of our patients with a diagnosis of hypoplastic left heart syndrome (HLHS), hypoplastic left heart complex (HLHC), and variants who received a biventricular repair following hybrid stage I with ductal stenting and bilateral pulmonary artery banding. Methods Between June 1998 and June 2013, a total of 154 patients with hypoplastic left heart structures underwent a hybrid stage I procedure. Forty patients were definitely treated by creating a biventricular circulation. Median age and body weight of patients before hybrid stage I were 8.5 days (2-40) and 3.0 kg (1.6-3.8), respectively. The diagnoses were HLHS with mitral and aortic stenosis (n = 7), HLHC (n = 15), HLHC with interrupted aortic arch (n = 9), critical aortic stenosis with hypoplastic aortic arch (n = 4), imbalanced atrioventricular septal defect with hypoplastic aortic arch (n = 2), double-outlet right ventricle with hypoplastic aortic arch (n = 2), and d-transposition of the great arteries with interrupted aortic arch (n = 1). Median age at the time of biventricular correction was 6.7 months (1.6-13.8). The patients were treated with direct biventricular correction, including repair of intracardiac defects (n = 32), Norwood/Rastelli or Yasui (n = 4), arterial switch (n = 2), Rastelli (n = 1), and Ross–Konno (n = 1) operations with ascending aortic/aortic arch reconstruction. Results All patients survived hybrid stage I. Median survival after biventricular correction is 7.9 years (0.9-14.9). Overall mortality was 10% (4 patients) at 4 weeks, 5 weeks, 6 weeks, and 4 months after biventricular correction, respectively. One patient had to be switched to univentricular circulation and another patient underwent orthotopic heart transplantation 3 and 4 months after biventricular correction, respectively. Conclusions The Giessen hybrid approach is an alternative to the conventional strategy to treat neonates with HLHS, HLHC, and variants. Biventricular repair after hybrid stage I is feasible and can be performed with satisfactory long-term survival.

Peri- and early postoperative mortality significantly influences the probability of survival following heart transplantation in children. Main causes of death early after transplantation are rejection, non specific graft failure and RV failure due to pulmonary hypertension. Optimal therapy of pulmonary hypertension and aggressive use of assist devices as a bridge to recovery will substantially improve survival in the early period after transplantation. Furthermore, the use of marginal donor organs will be more acceptable because transient myocardial insufficiency may recover during extracorporeal life support.

Indications for catheter-based interventions in patent ductus arteriosus (PDA)-dependent pulmonary blood flow have yet to be defined. The aim of this study was to assess the acute and midterm outcome after stent implantation in the PDA. Between 1996 and 2002, ductal stenting was performed in 21 neonates and infants (14 females/7 males) to maintain pulmonary blood supply in cyanotic congenital heart disease (CHD). Balloon-expandable stents were implanted in the PDA with a final diameter of 4-5 mm without procedural deaths. Stent patency was achieved for 8-1,130 days (median, 142). Reintervention was necessary in nine patients. Overall survival rate after 6 years was 86%. Two neonates died a few days after the procedure due to right heart failure not related to PDA stenting. Corrective surgery was possible in six patients. An additional aortopulmonary shunt was needed in three patients; Fontan type operations were performed in six. One patient died after bidirectional Glenn shunt, another five reached palliation by additional perforation of the atretic valve/balloon valvuloplasty, and two are awaiting surgery. We conclude that in many patients with cyanotic CHD, especially in those with ductal pulmonary perfusion and additional forward flow from the right ventricle, ductal stenting is an effective transcatheter approach. Morphology of the PDA predicts the risk of restenosis and necessity of reintervention. Growth of the pulmonary vascular bed allows corrective or palliative surgery, and some patients can be cured by the intervention alone.

Objective: To present an institutional experience with stent placement in the arterial duct combined with bilateral banding of the pulmonary artery branches as a basis for various surgical strategies in newborns with hypoplastic left heart obstructive lesions. Design: Observational study. Setting: Paediatric heart centre in a university hospital. Patients: 20 newborns with various forms of left heart obstructive lesions and duct dependent systemic blood flow. Interventions: Patients underwent percutaneous ductal stenting and surgical bilateral pulmonary artery banding. Atrial septotomy by balloon dilatation was performed as required, in one premature baby by the transhepatic approach. Main outcome measures: Survival; numbers of and reasons for palliative and corrective cardiac surgery. Results: One patient died immediately after percutaneous ductal stenting. One patient died in connection with the surgical approach of bilateral pulmonary banding. Stent and ductal patency were achieved for up to 331 days. Two patients underwent heart transplantation and two patients died on the waiting list. Ten patients had a palliative one stage procedure with reconstruction of the aortic arch and bidirectional cavopulmonary connection at the age of 3.5–6 months. There was one death. One patient is still awaiting this approach. Two patients received biventricular repair. In one, biventricular repair will soon be provided. Conclusions: Stenting the arterial duct combined with bilateral pulmonary artery banding in newborns with hypoplastic left heart or multiple left heart obstructive lesions allows a broad variation of surgical strategies depending on morphological findings, postnatal clinical conditions, and potential ventricular growth.

Background — Outcome of patients with hypoplastic left heart (HLH) is mainly influenced by the successful first-step palliation according to the Norwood procedure. An alternative approach is heart transplantation (HTX). The feasibility of ductal stenting in newborns with duct-dependent systemic blood flow and bilateral pulmonary artery banding has been reported. But it remains to be elucidated whether this approach allows a new strategy for patients with HLH. Methods and Results — In patients with various forms of HLH (n=11) and prostaglandin E-1 administration, ductal stenting was performed with balloon expandable Jo stents or Saxx stents. Bilateral pulmonary artery banding was surgically accomplished 1 to 3 days after the transcatheter procedure. Unrestricted blood flow through the interatrial septum was secured by balloon dilatation atrial septotomy, as required. Interventional procedures were performed with no mortality. Stent and ductal patency were achieved for up to 331 days. Two patients underwent HTX, and 8 patients had a palliative 1-stage procedure with reconstruction of the aortic arch and bidirectional cavopulmonary connection at the age of 3.5 to 6 months. There were 2 deaths. One patient with preoperative right heart failure died after the reconstructive surgery, and 1 patient died 4 months after ductal stenting and bilateral banding awaiting HTX. Conclusions — The present study is the first clinical trial showing that stenting the duct followed by bilateral pulmonary artery banding in newborns with HLH allows the combination of neoaortic reconstruction, which is part of first-stage palliation of HLH, with the establishment of a bidirectional cavopulmonary connection. Additionally, it allows the chance for HTX after extended waiting periods.

Since 1988, 82 heart transplants have been performed in 80 infants and children. Diagnoses pretransplant were: hypoplastic left heart syndrome (HLHS) (n = 43); cardiomyopathy (n = 19); endocardial fibroelastosis (n = 6); and other complex congenital heart diseases (n = 12). Age at transplantation was < 1 yr in 61 patients. Overall survival rate was 79% at 1 yr and 73% at 5 and 10 yr. To date, 20 patients have died after transplantation. Causes of death were: rejection (eight patients); right ventricular failure (four patients); transplant coronary artery disease (TCAD) (two patients); and other causes (six patients). In the majority of patients somatic growth is not impaired, and renal function is reduced (but stable) in all patients. Two patients developed post-transplant lymphoproliferative disease, which was treated successfully. Major long-term morbidity is neurologic deficit - severe in three patients and minor in six. TCAD was present or suspected in six surviving patients. We conclude that heart transplantation in infants and children can be performed with good early and late results. Quality of life is excellent in most patients. TCAD, however, will become an increasing problem in the long term.

Although recent technological advances have dramatically improved the survival of preterm infants born at the “limit of viability,” coexisting congenital heart disease may limit resuscitation efforts. This case report is the first to describe successful transcatheter relief of right ventricular outflow tract obstruction of an extremely low birth weight infant with tetralogy of Fallot as rescue therapy after closure of the arterial duct. This interventional approach offers bridging to palliative or corrective surgery and could be used in fetal intervention when indicated.

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.