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

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.

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.

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.

Cardiac transplantation for children with end-stage cardiac disease with no other medical or surgical options is now standard. The number of children in need of cardiac transplant continues to exceed the number of donors considered "acceptable." Therefore, there is an urgent need to understand which recipients are in greatest need of transplant before becoming "too ill" and which "marginal" donors are acceptable in order to reduce waitlist mortality. This article reviewed primarily pediatric studies reported over the last 15 years on waitlist mortality around the world for the various subgroups of children awaiting heart transplant and discusses strategies to try to reduce the cardiac waitlist mortality.

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

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.

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.

Moderne Kinderkardiologie In Deutschland kommen jährlich etwa 6.000 Kinder mit einem Herzfehler zur Welt. Dank neuer Technologien in Diagnostik und Therapie verbessert sich die Prognose jedoch kontinuierlich. Die Autoren geben in diesem Buch einen profunden Überblick über die Therapieoptionen der kindlichen Herzfehler und bereiten die Leser optimal auf mögliche Probleme im Therapieverlauf vor. - Therapie von Kindern mit angeborenen und erworbenen Herzerkrankungen bis zur Herztransplantation - Umfangreiche Medikamentenliste - Neu in der 2. Auflage: - Präoperative Diagnostik - Fast Tracking

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.

We examined the hypothesis that major cardiac surgery triggers a more intense adrenal stress response than less intensive noncardiac surgery, which then alters cortisol inactivation. Urinary excretion rates of glucocorticoid metabolites were determined before and after surgery using gas chromatography-mass spectrometry in 29 children undergoing scheduled major cardiac surgery and 17 control children undergoing conventional noncardiac surgery in a prospective observational study. Excretion rates of glucocorticoid metabolites were summed and corrected for creatinine excretion to calculate cortisol production rates (mg/mmol creatinine/m(2) body surface area). Precursor/product ratios from individual metabolites were calculated to characterize cortisol inactivation (11β-hydroxysteroid dehydrogenase). Postoperatively, median cortisol production rates increased in both groups (from 2.7 to 9.3; controls: from 2.7 to 5.8; p0.001) with no significant difference between groups (p=0.12). Ratios of cortisol to cortisone metabolites, indicating the overall activity of 11β-hydroxysteroid dehydrogenase, increased postoperatively in both groups (p0.001). In conclusion, surgery resulted in a distinct postoperative increase in cortisol production. In contrast to our hypothesis, children undergoing major cardiac surgery did not show an increased adrenal stress response compared to children undergoing conventional surgery. Furthermore, the reduction in cortisol inactivation appears to be an essential part of the stress response to pediatric surgery in general.

While the neuroprotective benefits of estrogen and progesterone in critical illness are well established, the data regarding the effects of androgens are conflicting. Surgical repair of congenital heart disease is associated with significant morbidity and mortality, but there are scant data regarding the postoperative metabolism of sex steroids in this setting. The objective of this prospective observational study was to compare the postoperative sex steroid patterns in pediatric patients undergoing major cardiac surgery (MCS) versus those undergoing less intensive non-cardiac surgery. Urinary excretion rates of estrogen, progesterone, and androgen metabolites (μg/mmol creatinine/m(2) body surface area) were determined in 24-h urine samples before and after surgery using gas chromatography-mass spectrometry in 29 children undergoing scheduled MCS and in 17 control children undergoing conventional non-cardiac surgery. Eight of the MCS patients had Down's syndrome. There were no significant differences in age, weight, or sex between the groups. Seven patients from the MCS group showed multi-organ dysfunction after surgery. Before surgery, the median concentrations of 17β-estradiol, pregnanediol, 5α-dihydrotestosterone (DHT), and dehydroepiandrosterone (DHEA) were (control/MCS) 0.1/0.1 (NS), 12.4/11.3 (NS), 4.7/4.4 (NS), and 2.9/1.1 (p=0.02). Postoperatively, the median delta 17β-estradiol, delta pregnanediol, delta DHT, and delta DHEA were (control/MCS) 0.2/6.4 (p=0.0002), -3.2/23.4 (p=0.013), -0.6/3.7 (p=0.0004), and 0.5/4.2 (p=0.004). Postoperative changes did not differ according to sex. We conclude that MCS, but not less intensive non-cardiac surgery, induced a distinct postoperative increase in sex steroid levels. These findings suggest that sex steroids have a role in postoperative metabolism following MCS in prepubertal children.

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.