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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.

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.

ObjectiveTo compare the results of elective open surgical repair (OSR) and total endovascular repair of juxtarenal aortic aneurysms (JAA), with either the Cook Zenith or the Vascutek Anaconda fenestrated stent grafts (F-EVAR) in a university hospital setting.Patients and methodsBetween April 1999 and July 2014, of 926 patients with an abdominal aortic aneurysm, 69 were juxtarenal, where 34 had an elective OSR and 35 had F-EVAR. A post-operative rise of baseline creatinine by >50% and/or deterioration of estimated glomerular filtration rate by 25% were defined as renal failure.ResultsThe demographics of the patients were similar except for heart insufficiency, peripheral arterial disease, and pre-existing renal artery stenosis (p

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.

The hypothesis driving this study was that photodynamic therapy (PDT) may limit abdominal aortic aneurysm growth due to matrix changes. The aortas of 12 rats were incubated with elastase using a newly modified experimental aneurysm model (3.5 mg/ml). Rats were allocated to an elastase-only group (n = 6) to study the elastase-induced aneurysm growth and an elastase ± PDT group to evaluate if PDT limited aneurysm growth (n = 6). PDT was performed with the photosensitizer methylene blue, and thermoneutral laser light (660 nm) was applied (120 J/cm2, 100 mW/cm2) using a diode laser. Four untreated rats served as controls. The arteries were analysed after 4 weeks based on histology, immunohistochemistry and morphometry. This modified rat elastase model led to reproducible aneurysm development with no elastase-induced mortality compared with control animals (circumference, controls: 2.9 ± 0.2 vs. elastase: 5.5 ± 0.9 mm; P

INTRODUCTION: The aim of this study was to evaluate the role of intra-operative Prostavasin® application during crural bypass surgery. 68 patients, who underwent infrainguinal autologous vein bypass surgery, were prospectively analyzed. RESULTS: Intra-operative flow measurements and Prostavasin administration were performed in all patients. The mean flow was 105 ml / min (minimal flow 18 ml / min, maximal flow 321 ml / min). The means of maximal and minimal flow were 329 und 30 ml / min, respectively. The mean of the calculated Pulsatility index was 3.1. The mean flow volume increased after administration of Prostavasin in all patients with a mean increase of 103 %. The Pulsatility index decreased by 39 %. Primary and secondary patency rates after 1 year were 70.3 % und 73.1 %, respectively. The differentiated analyses of the region of the distal anastomoses (popliteal / crural) showed a statistically significant bigger increase of Q after Prostavasin application for infrainguinal popliteal bypasses compared to patients with crural reconstructions (p = 0.05). In addition, the decrease of the Pulsatility index was significantly higher in popliteal grafts (p = 0.03). Patients with early bypass graft failure showed a significantly smaller decrease of PI after Prostavasin application compared to the other patients. CONCLUSION: The profit of intra-operative arterial Prostavasin® application is first of all an immediate one. The injection of Prostavasin® leads to an instant vessel dilatation. This allows the surgeon to get an impression of the flow capacity of the bypass. The Pulsatility index as an indicator for resistance is an important factor for bypass patency.

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.

Die Morbiditat im Langzeitverlauf nach einer Herztransplantation im Kindesalter manifestiert sich im Wesentlichen durch eine Erkrankung des transplantierten Herzens selbst, durch die Nebenwirkungen der lang dauernden medikamentosen Immunsuppression und schlieslich als psychosoziale Morbiditat, bedingt durch die Sondersituation des Lebens und Aufwachsens mit einem transplantierten Herzen. Die Transplantatvaskulopathie als eigenstandige Erkrankung des transplantierten Organs stellt eine haufige Komplikation nach Herztransplantation dar und ist ein wesentlicher Morbiditats- und Mortalitatsfaktor, der die Langzeitprognose erheblich limitiert. Progrediente Nierenfunktionsstorungen und die gehaufte Inzidenz von Malignomen sind weitere bedeutende prognoselimitierende Erkrankungen, deren Ursache die lang dauernde immunsuppressive Therapie darstellt.

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.

Summary Background: SvO2-guided therapy, using fiberoptic oximetric catheters can help to improve the outcome after complex congenital heart surgery especially in infants undergoing the Stage 1-Norwood-Procedure. So far, fiberoptic catheters have to be placed transthoracically by the surgeon into the vena cava or the pulmonary artery putting the infant at an additional risk of bleeding at the time of catheter removal. Methods: We used a new percutaneously applicable fiberoptic probe for continuous monitoring of central venous saturation in three infants undergoing modifications of the Stage 1-Norwood-Procedure (reconstruction of the aortic arch), two in combination with a bidirectional cavopulmonary connection (Glenn shunt), the third with reconstruction of the pulmonary arteries (biventricular repair). The approved clinical monitoring system consisted of a small (2 F) fiberoptic probe and a bed-side-monitor. The probe was inserted via the routine central venous access for such a case. Results: Continuous SvO2 measurement is feasible in infants using the CeVOX system in combination with routine central venous access, and the advantages of continuous monitoring are discussed. Conclusions: We believe that compared with transthoracically inserted oximetric catheter, the presented percutaneous technique avoids additional risks at the time of catheter removal (i.e. bleeding) and has become part of our standard management.

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.

Epigastrische Schmerzen, Ubelkeit, Erbrechen und eine obere gastrointestinale Blutung werden gelegentlich durch heterotopes Pankreasgewebe der Magenwand verursacht. Trotz moderner diagnostischer Verfahren gestaltet sich die Diagnose heterotopen Pankreasgewebes oftmals schwierig. Daher wird von vielen Autoren die Magenwandresektion und Schnellschnittuntersuchung als diagnostische und therapeutische Masnahme befurwortet. Zwei typische Kasuistiken werden im Folgenden mit einer Literaturubersicht vorgestellt.

OBJECTIVE The aim of the present study was to characterize pulmonary surfactant properties in children undergoing cardiovascular surgery with cardiopulmonary bypass. DESIGN Prospective clinical trial. SETTING University hospital pediatric intensive care unit. PATIENTS Fifty pediatric patients with congenital cardiac defects undergoing cardiovascular surgery with (n = 35) and without (n = 15) cardiopulmonary bypass procedure. INTERVENTIONS Tracheal aspirates were collected by saline lavage during routine suctioning before (baseline) and after cardiopulmonary bypass, as well as 4, 8, and 24 hrs after admission to the pediatric intensive care unit. MEASUREMENTS AND MAIN RESULTS Total protein and phospholipid concentrations were assessed in native tracheal aspirates, in large surfactant aggregates, and in small surfactant aggregates. Phospholipid profiles and phosphatidylcholine fatty acids; surfactant apoproteins SP-A, SP-B, and SP-C (enzyme-linked immunosorbent assay); and surface activity (Pulsating Bubble Surfactometer) were all analyzed in large surfactant aggregates. With cardiopulmonary bypass, an initial increase in total protein content was followed by an increase in phospholipid concentration in tracheal aspirates. Large surfactant aggregates decreased 4 hrs after cardiopulmonary bypass (4 hrs, 22.6 +/- 5.6%; mean +/- SEM; p

OBJECTIVES: Epidemiology of candidacy for intestinal transplantation (ITx) and timing for referral for ITx are unknown. Patient candidacy and physician attitudes toward ITx were investigated among centers that participated in previous European surveys on home parenteral nutrition (HPN). METHODS: Patients on HPN for benign intestinal failure (IF) were evaluated by a structured questionnaire. Candidacy was assessed by USA Medicare and American Transplantation Society criteria, categorized as: (1) life-threatening HPN complications; (2) high risk of death because of the gastrointestinal disease; (3) IF with high morbidity or patient HPN refusal. Physicians judged candidacy as immediate or potential. RESULTS: Forty-one centers from nine countries enrolled 688 adults (> 18 yr) and 166 pediatric patients; 70% of patients were from five countries which collected 60-100% of their HPN patients. Candidacy was 15.7% in adults and 34.3% in pediatrics (HPN failure, 62.1% and 28.1%; gastrointestinal disease, 25.9% and 59.6%; high morbidity IF or HPN refusal, 12.0% and 12.3%, respectively). Immediate candidacy was required for 14.8% of adult and 15.8% of pediatric candidates (< 50% of candidates because of HPN-related liver failure). Among centers, the candidacy rate ranged 0-100% and was negatively associated with the number of patients enrolled in the survey (R = -0.463, p = 0.002). Among the major contributing countries, candidacy ranged 0.3-0.8/million inhabitants for adults and 0.9-2/million inhabitants < or = 18 yr for pediatric candidates. CONCLUSIONS: The rate of candidacy and the indications for ITx candidacy differed greatly among age groups and HPN centers; within countries candidacy was more homogeneous; physicians had a generally reserved attitude toward ITx.

Fragestellung: Das hypoplastische Linksherz (HLH) ist eine komplexe Entwicklungsfehlbildung der linken Herzkammer, der Aorta und der assoziierten Herzklappen. Unbehandelt wird die Neonatalperiode nur selten uberlebt. Die Prognose ist jedoch nicht langer hoffnungslos. Eine postnatale Prostaglandin-E1-Therapie ist zur Stabilisierung des Neugeborenen mit HLH in jedem Fall berechtigt, bis auf der Basis eines ausfuhrlichen Elterngesprachs eine Entscheidung fur oder gegen eine chirurgische Behandlung getroffen werden kann. Methode: Zur Beurteilung der derzeitigen Behandlungsstrategie bei Neugeborenen mit HLH analysierten wir die prospektiv erhobenen Daten von 23 im Jahr 1996 zugewiesenen Neugeborenen mit HLH. Ergebnisse: Funf der 23 Patienten wurden nicht chirurgisch behandelt. Eine Norwood-I-Operation wurde bei 8 Kindern durchgefuhrt. Davon konnte ein spat postnatal operierter Saugling nicht von der Herz-Lungen-Maschine entwohnt werden. Ein weiteres Kind starb 6 Wochen nach der Norwood-I-Operation an einem Leberversagen. Neun Kinder wurden herztransplantiert, kein Kind starb nach der Transplantation. Somit betrug die Uberlebensrate aller zugewiesenen Neugeborenen 70%. Schlusfolgerungen: Unsere chirurgischen Strategien der stufenweisen Palliativrekonstruktion nach Norwood und der Herztransplantation im Sauglingsalter erlauben einen Behandlungsalgorithmus fur das HLH. Die Ergebnisse der Behandlungsmoglichkeiten reduzieren die traditionelle „do nothing” Haltung fur alle Neugeborenen mit HLH auf ausgewahlte Patienten.

Von Juni 1988 bis Dezember 1996 wurden am Kinderherzzentrum Giesen 36 Patienten, im Alter von weniger als einem Jahr, einer Herztransplantation zugefuhrt. Zugrundeliegende Diagnosen waren ein hypoplastisches Linksherzsyndrom (n = 26), Endokardfibroelastose (n = 4), Kardiomyopathie (n = 3) und komplexe Vitien (n = 3). Die mittlere Wartezeit von Transplantation betrug 52 Tage, das Verhaltnis der Korpergewichte von Spender zu Empfanger lag im Mittel bei 1,8. Sieben Patienten (19%) verstarben nach Transplantation, uberwiegend innerhalb des ersten Monats nach der Transplantation. Die kumulative Uberlebenswahrscheinlichkeit liegt im Geamtkollektiv bei 79%. Der Einflus zunehmender Erfahrung zeigt sich beim Vergleich der Patienten mit Transplantationen von 1988 bis 1993 (n ? 15) und von 1994 bis 1996 (n = 21). Wahrend im ersten Zeitraum lediglich 50% uberlebten, betrug die Uberlebensrate der spater transplantierten Kinder 92%, die 1-Jahres-Uberlebensrate lag bei 100%. Bei 20 Patienten traten insgesamt 31 Abstosungsepisoden auf, wobei 2 Sauglinge verstarben. 71% aller Abstosungen traten innerhalb des ersten Monats nach der Transplantation auf. Die Nierenfunktion ist ein Jahr nach der Transplantation nur masiggradig eingeschrankt ohne Tendenz zur Verschlechterung. Die somatische Entwicklung verlauft bei nahezu allen Kindern perzentilengerecht. Die Lebensqualitat der Patienten nach der Transplantation ist ausgezeichnet. Alle Patienten leben ohne Einschrankungen zu Hause. Bei zwei Patienten liegt allerdings ein neurologisches Defizit vor. Bislang gibt es im Gesamtkollektiv keine Anzeichen fur eine Transplantatvaskulopathie oder eine maligne Erkrankung. Eine Herztransplantation stellt nach diesen Ergebnissen eine uberdenkenswerte Alternative in der Behandlung sehr komplexer Vitien und Kardiomyopathien im Neugeborenen- und Sauglingsalter dar.

Der klinische Nutzen der perioperativen Ernahrungstherapie wurde auf der Basis von 28 kontrollierten Studien analysiert. Eine praoperative enterale oder parenterale Ernahrung ist nur bei Patienten mit hochgradiger Mangelernahrung indiziert und kann bei diesen zu einer Verminderung der postoperativen Komplikationsrate und Klinikletalitat beitragen. Bei nicht oder grenzwertig Mangelernahrten ist dagegen nicht auszuschliesen, das die supportive Ernahrung den postoperativen Verlauf negativ beeinflust. Ein klinischer Nutzen der postoperativen Ernahrungstherapie ist nur bei Patienten erkennbar, die sich operationsbedingt langer als eine Woche nicht ausreichend oral ernahren konnen. Dabei ist bei etwa 20 % von ihnen mit erheblichen kardiopulmonalen Nebenwirkungen zu rechnen. In der grosen Mehrzahl der chirurgischen Patienten ist auch nach grosen Operationen die Zufuhr einer Wasser- und Elektrolytlosung ausreichend.

Home parenteral nutrition (HPN) has been shown to delay deterioration in cancer patients with malnutrition. Its risk-benefit ratio, however, is determined by the threat of central venous catheter (CVC) complications. Only few prospective studies on this subject exist, most of them based on small samples. The objective of this study was to provide reliable estimates of incidence rates of CVC complications in everyday HPN patient care in Germany.Aiming for a large prospective cohort study, we cooperated with a service provider caring for HPN patients nationwide. Between July 1 and November 30, 2006, all consecutive adult patients with more than 10 infusion days and no previous history of HPN were recruited. Follow-up ended on January 31, 2007. Data were collected in a standardised way by the provider's staff. To prevent underreporting, we used computer-assisted telephone interviews with medical caregivers as a provider-independent data source.481 patients met the inclusion criteria, contributing a total of 31,337 catheter days. 52 patients experienced a total of 63 CVC complications, resulting in an incidence rate of 2.01 CVC complications per 1,000 catheter days including 1.02 CVC infections per 1,000 catheter days.HPN administration can be safely performed with a relatively low rate of CVC complications.

Objective: To prove safety and effectiveness of a lipid emulsion enriched with n-3 fatty acids from fish oil (Lipoplus) within the setting of parenteral nutrition of patients after major abdominal surgery and to determine whether there are effects on outcome parameters. Design: Prospective, randomized, double-blind, multicenter trial. Setting: University and surgical teaching hospitals. Patients: After obtaining informed consent, 256 patients undergoing major abdominal surgery were randomized. Parameters of safety, effectiveness, and outcome were routine laboratory parameters, complication rates, length of stay in the intensive care unit, and length of hospital stay. In addition we determined in patient subgroups of 30 patients each, the changes of the content of selected long-chain polyunsaturated fatty acids, the leukotriene synthetic capacity and the antioxidant -tocopherol. Interventions: Participating patients were randomized to receive either Lipoplus (group I; n 127 patients) or Intralipid (group II; n 129 patients). Parenteral nutrition was initiated immediately after surgery and ended on day 5 after surgery. Measurements and Main Results: No significant differences between groups I and II were observed when comparing routine laboratory parameters during the perioperative period. Plasma levels of eicosapentaenoic acid, leukotriene B5, and antioxidant content were significantly increased in group I. Furthermore, there was a significantly shorter length of hospital stay of 21% (17.2 vs. 21.9 days; p .0061) in group I. Conclusions: Our findings indicate that the administration of Lipoplus in the postoperative period after major abdominal surgery is safe and results in a significantly shorter length of hospital stay. Administration of n-3 polyunsaturated fatty acids in the postoperative period can be considered a valuable choice for patients requiring parenteral nutrition after major abdominal surgery. (Crit Care Med 2007; 35:700‐706)