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Funding Acknowledgements Type of funding sources: Private grant(s) and/or Sponsorship. Main funding source(s): Stiftung KinderHerz Weserstr. 101 45136 Essen. Background During the last years, lung function tests became of high interest in patients with a congenital heart defect (CHD). However, data are still rare and mostly concentrate on spirometry, investigating forced vital capacity (FVC), forced expiratory volume in 1 second (FEV1), and its ratio (FEV1/FVC). Purpose This study prospectively examined patients with CHD regarding their lung function. Data reported were FVC, FEV1, and FEV1/FVC (spirometry) as well as total lung capacity (TLC) and residual volume, presented as its ratio to TLC (RV/TLC) and compared to the latest Global Lung Initiative reference values (2021). Methods From April 2018 to October 2022, one hundred eighty-six adults with CHD (32 ± 10 years, 83 female) participated in the study. Their results (z-scores) were compared with the student’s T-test against zero. Results Patients mostly reached normal values but differed significantly in FVC-z (-1.1 ± 1.2, p-1.645 as normal results, 33% had striking results: 43 showed a reduced FVC (FVC-z-1,645), which stands for a non-specific pattern, and 26 patients (60%) had a real restriction, which was only detected via body plethysmography. Conclusions Patients with CHD and a reduced FVC in spirometry need to undergo body plethysmography. Most of them have unrevealed ventilatory restrictions that should be further analyzed and treated if needed.

Funding Acknowledgements Type of funding sources: Private grant(s) and/or Sponsorship. Main funding source(s): Stiftung KinderHerz Weserstr. 101 45136 Essen. Background For the last decades, surgical progress lead to better functional outcomes in patients with congenital heart defect (CHD), which may also affect lung function. Reduced lung volumes were found in about half of all patients with a CHD. Purpose This prospective study investigated lung function with spirometry and body plethysmography in children with CHD. Data reported were FVC, FEV1, and its ratio (spirometry), together with total lung capacity (TLC), and residual volume, presented as its ratio to TLC (RV/TLC) from body plethysmography. Methods From April 2018 to October 2022 fifty-eight young patients (14.0 ± 2.5 years, 16 girls) with CHD participated in the study. Their results are presented as z-scores, sticking to the latest Global Lung Initiative reference values (2021). Values of z-scores -1.645, and a reduced TLC (z-1.645, and an increased RV/TLC-z>1,645 represent a non-specific pattern. FEV1/FVC-z Results Results in FVC-z were -1.0 ± 1.3, FEV1-z -1.0 ± 1.2, and its ratio 0.0 ± 1.0. TLC-z was -0.4 ± 1.1 and RV/TLC-z reached 0.7 ± 0.7. Sixty-seven percent had normal results. However, 33% had a reduced FVC in spirometry. Only three patients showed decreased FEV1/FVC ratio. However, only five children show restriction (TLC Conclusions Still, about one-third of children with CHD show striking results in lung volumes. Although studies suggest better results in nowadays patients, children’s lung function with spirometry and body plethysmography should be tested regularly in children with congenital heart disease. Since lung function is trainable, these patients might benefit from early diagnosis.

Stenting of the patent ductus arteriosus (PDA) has been accepted as alternative option to surgical shunting to enable additional pulmonary blood flow or for palliation of patients with a truly duct-dependent pulmonary circulation. The procedure can be challenging given the variable and often tortuous anatomy of the PDA and various technical approaches are reported.To report an alternative technique to treat tortuous ducts with microcatheter assistance and by transfemoral approach.We applied this technique of PDA stenting in 5 consecutive patients (4/5 age 1 week, weight 2,7-3,2 kg; 1/5 re-do PDA stenting at 6,5 month and 5,9 kg). A soft coronary guidewire was advanced by microcatheter assistance into the branch pulmonary arteries and thereafter replaced by an extrastiff guidewire to enable the placement of long coronary stents.Successful PDA stenting with this stepwise approach and with femoral access only could be achieved in all patients (n = 5/5). A single stent was used in 2 patients (one with re-do stenting and previous stents). 3/5 patients had 2 stents implanted by telescopic technique. Stent sizes used were 4,5 × 15 mm (n = 2) and 4,5 × 18 mm (n = 6). No guide wire or stent dislodgement appeared through all procedures with microcatheter assistance.This technique enables PDA stenting via transfemoral approach in complex and tortuous ducts and thereby offers an attractive addition to the interventional management of truly duct-dependent pulmonary circulation.

Herzrhythmusstorungen im Kindesalter sind relativ seltene Ereignisse und stellen gerade aus diesem Grund eine hohe kognitive und emotionale Herausforderung fur die Padiatrie dar. In der akuten Situation ist jedoch eine spezifische Diagnose der vorliegenden Rhythmusstorung in aller Regel nicht erforderlich, da sich die initiale Therapie vor allem nach dem klinischen Zustand des Kindes richtet. Anhand eines uberschaubaren Leitfadens kann daher jeder Padiater eine adaquate Akuttherapie durchfuhren.

Herzrhythmusstorungen im Kindesalter sind relativ seltene Ereignisse und stellen gerade aus diesem Grund eine hohe kognitive und emotionale Herausforderung fur den Padiater dar. In der akuten Situation ist jedoch eine spezifische Diagnose der vorliegenden Rhythmusstorung nicht erforderlich, da sich die initiale Therapie v. a. nach dem klinischen Zustand des Kindes richtet. Anhand eines uberschaubaren Leitfadens kann daher jeder Padiater eine adaquate Akuttherapie durchfuhren.

The Queensland Government’s Long Paddock website has been redeveloped on Amazon Web Services cloud computing platform, to provide Australian rangelands and grazing communities (i.e. rural landholders, managers, pastoralists (graziers), researchers, advisors, students, consultants and extension providers) with easier access to seasonal climate and pasture condition information. The website provides free, tailored information and services to support management decisions to maximise productivity, while maintaining the natural resource base. For example, historical rainfall and pasture analyses (i.e. maps, posters and data) have been developed to assist in communicating the risk of multi-year droughts that are a feature of Queensland’s highly variable climate.

Die arterielle Hypertonie ist im Kindesalter eine diagnostische und therapeutische Herausforderung. Die erste Blutdruckmessung sollte bei Kindern ab dem dritten Lebensjahr erfolgen. Bei prahypertensiven Kindern sollten nichtmedikamentose Masnahmen wie Gewichtsreduktion, ausgewogene Ernahrung und sportliche Betatigung zum Einsatz kommen. Bei Kindern mit arterieller Hypertonie muss eine medikamentose Therapie zur Blutdrucksenkung eingeleitet werden. Zudem gilt es bei diesen Patienten, Endorganschaden in Auge, Herz und Nieren auszuschliesen. Da die arterielle Hypertonie einen der starksten Risikofaktoren fur die Entwicklung atherosklerotischer Gefasveranderungen im Kindes- und Erwachsenenalter darstellt, muss sie konsequent therapiert und untersucht werden.

Ein Herzgerausch wird bei rund 70 % aller Kinder festgestellt; bei einem Grosteil dieser Gerausche handelt es sich um harmlose, funktionelle Systolika. Nur bei rund 1 % aller Kinder liegt ein Herzfehler vor. Die Unterscheidung zwischen pathologischem und harmlosem Herzgerausch gelingt dem geubten Untersucher bereits aufgrund des Auskultationsbefunds; trotzdem wird zum sicheren Ausschluss eines organischen Herzfehlers die Durchfuhrung eines 12-Kanal-Elektrokardiogramms gefordert. Der Unerfahrene sollte Kinder mit Herzgerausch einem Kinderkardiologen vorstellen. Im Neonatalalter konnen allerdings gerade kritische Herzfehler ohne Herzgerausch vorliegen. Deshalb wird dem Neonatalscreening mittels Pulsoxymetrie eine besondere Rolle in der Diagnosestellung von bedrohlichen Herzfehlern zugeschrieben.

Adipositas im Kindesalter – damit werden dem Kind und Jugendlichen aus kardiovaskularer Sicht bereits in fruhen Jahren zahlreiche Risikofaktoren auferlegt, die gemeinsam vorzeitige Atherosklerose fordern. Adipositas ist zum einen bereits per se ein Risikofaktor, fuhrt aber auch zu einem gehauften Auftreten weiterer Risikofaktoren wie arterielle Hypertonie, Dyslipidamie und gestorte Glukosetoleranz. Daher muss in der Adipositasbehandlung die kardiovaskulare Pravention einen hohen Stellenwert einnehmen. Diese sollte vor allem eine konsequente Behandlung der Grunderkrankung beinhalten. Gewichtsabnahme, aber auch verbesserte Fitness gelten als vasoprotektive Faktoren. Zusatzlich muss eine umfassende kardiovaskulare Diagnostik und Therapie der Sekundarerkrankungen durchgefuhrt werden. Moderne bildgebende Verfahren wie die Messung der Intima-media-Dicke der A. carotis konnen bei ubergewichtigen Kindern bereits fruh subklinische Gefasveranderungen feststellen. Ganz allgemein sollte das ubergewichtige Kind als potenzieller Gefaspatient von morgen gelten! Daher soll im folgenden Beitrag auf die Pravalenz und die Diagnostik kardiovaskularer Risikofaktoren und auf entsprechende therapeutische Ansatze bei ubergewichtigen Kindern eingegangen werden.

Objectives Growth failure is a permanent sequelae in juvenile idiopathic arthritis (JIA). The aim of the study was to compare pubertal growth in control and growth hormone (GH) treated JIA subjects. Design 64 children with JIA at a mean age of 10.38 ± 2.80 years were enrolled and followed until final height (measured in standard deviation (SD) scores). 39 children (20 m) received GH therapy and 24 (9 m) served as controls. GH dose was 0.33 mg/kg/week. Linear regression analysis was performed to identify factors influencing total pubertal growth. Results Mean total pubertal growth was 21.1 ± 1.3 cm (mean ± SD) in GH treated JIA patients and 13.8 ± 1.5 cm in controls. Final height was significantly higher with GH treatment (− 1.67 ± 1.20 SD) compared to controls (− 3.20 ± 1.84 SD). Linear regression model identified age at onset of puberty (s = − 4.2,CI: − 5.9, − 2.6 in controls and s = − 2.3,CI: − 3.6, − 1.1 in GH treated) as the main factor for total pubertal growth. Final height SDS was determined by the difference to target height at onset of puberty (s = − 0.59;CI: − 0.80, − 0.37 in controls and s = − 0.30,CI: − 0.52, − 0.08 in GH treated), age at onset of puberty (s = 0.47;CI:0.02,0.93 in controls and 0.23;CI: − 0.00,0.46 in GH treated) and height gain during puberty (s = 0.13;CI:0.05,0.21 in controls and s = 0.11;CI:0.07,0.16 in GH treated). Conclusion Total pubertal growth in JIA patients treated with GH was increased by a factor of 1.5 greater in comparison to controls leading to a significantly better final height. To maximize final height GH treatment should be initiated early to reduce the height deficit at onset of puberty.

Die Fallot-Tetralogie (TOF) ist mit einer Pravalenz zwischen 0,26 und 0,8‰ aller Lebendgeburten der haufigste Herzfehler mit primarer Zyanose. Durch verbesserte Operationstechniken erreichen heute nahezu alle Patienten das Erwachsenenalter. Bei fast allen Patienten tritt eine postoperative Pulmonalinsuffizienz (PI) auf, die entscheidenden Einfluss auf die langfristige Morbiditat und Mortalitat hat. Die kardiale Magnetresonanztomographie (MRT) ist fur Quantifizierung von Ventrikelfunktion und Klappeninsuffizienzen etabliert und aufgrund ihrer fehlenden Invasivitat und Strahlenexposition sehr gut fur regelmasige Verlaufskontrollen der heranwachsenden Patienten geeignet. Insbesondere der Zeitpunkt fur einen Pulmonalklappenersatz darf nicht verpasst werden, da sich die rechtsventrikularen (RV) Volumina nur normalisieren, wenn das praoperative enddiastolische Volumen unter 170 ml/m2 und das endsystolische Volumen unter 85 ml/m2 liegen. Stenosen der Pulmonalarterien betreffen bis zu 15% der Patienten nach der Korrekturoperation. Distale Stenosen konnen eine vorbestehende PI verstarken und uber eine kombinierte Druck- und Volumenbelastung zu einer Rechtsherzinsuffizienz fuhren. Sie mussen daher fruhzeitig erkannt und mit einer Ballondilatation und eventuell einer Stenteinlage behandelt werden.

Die Synkope – ein kurz dauernder, spontan reversibler Anfall von Bewusstlosigkeit – stellt auch im Kindes- und Jugendalter ein haufiges Krankheitsbild dar. In der praklinischen diagnostischen Abklarung steht die Unterscheidung zwischen harmloseren, neural vermittelten Ohnmachtsanfallen sowie Synkopen aufgrund organischer Erkrankungen im Mittelpunkt. Mit Hilfe einer exakten Anamnese, korperlichen Untersuchung sowie einem Echokardiogramm ist es in vielen Fallen moglich, die Genese naher einzuordnen. Bei Verdacht auf eine organisch bedingte Synkope sind weitergehende Untersuchungen indiziert sowie eine adaquate Behandlung des Grundleidens einzuleiten. Unter den organisch bedingten Synkopen sind vor allem die kardialen Synkopen wegen ihrer hohen Mortalitat hervorzuheben. Die neural vermittelten Synkopen sind in der Regel harmlos und durch einen exzellenten Langzeitverlauf gekennzeichnet. Im Folgenden soll das Krankheitsbild der Synkope in Kindes- und Jugendalter dargestellt werden. Besonderes Augenmerk gilt hierbei der Entscheidungshilfe fur das praktische Vorgehen bezuglich Diagnostik und Therapie.

Because reduction in baroreceptor sensitivity (BRS) has been associated with hypertension in the normal population and with increased cardiovascular morbidity and mortality in patients with diabetes mellitus, we measured BRS in a patient cohort of children with type 1 diabetes mellitus.Two hundred and eight children (150 patients with type 1 diabetes mellitus, mean age 13.9 +/- 2.8 years, 70 boys, mean HbA(1c) 7.8 +/- 1.4%; and 58 healthy controls, mean age 14.1 +/- 3.1 years, 32 boys) were studied. BRS and heart rate variability (HRV) were analysed from a short-time ECG and BP recording using the sequence method (BRS) and the frequency domain method (HRV).There were 111 of 150 patients (74%) and 5 of 58 controls (8.6%) that showed impaired BRS. Mean BRS differed significantly between patients and controls (18.4 +/- 7.2 vs 25.8 +/- 8.2 ms/mm, p0.001). BRS correlated inversely with systolic BP (r = -0.23, p = 0.009) and was related to diabetes duration (r = -0.194, p = 0.027). Analysis of HRV showed greater sympathetic and less parasympathetic influence in patients than in controls (low frequency/high frequency ratio 1.3 +/- 0.8 vs 0.9 +/- 0.6, p0.05); the low frequency/high frequency ratio was inversely correlated with BRS (r = -0.28, p = 0.001).Diabetic children show reduced BRS. In our patient group, the single risk factor for this finding was found to be the disease duration. The degree of BRS impairment was related to the degree of autonomic dysbalance.

Introduction: Heart rate variability (HRV) is reduced in adults and children after cardiac transplantation. Testing of HRV has been used to assess re-innervation of the cardiac graft; its reliability in ruling out acute graft rejection is still under investigation. This study used a short-term test on HRV in 23 heart and heart–lung transplanted children and adolescents and compared the results with 24-h ECG recordings. Patients and methods: Twenty-three subjects (16.3 ± 4.2 yr; 10 females) underwent a 10-min HRV test at two occasions and one 24-h ECG. HRV was calculated according to the time domain method (RR interval, standard deviation of RR interval) and the frequency domain method (total power, LF and HF for assessment of sympathovagal modulation of heart rate). Results: Correlation between the short-term tests and 24-h ECG was high with regard to the frequency domain analysis of HRV. Correlation was less pronounced in the time domain method. Conclusions: In heart and heart–lung-transplanted children and adolescents, due to reduced overall HRV short-term testing may give as reliable data as 24-h ECG. Therefore, especially when power spectral analysis has to be performed as a longitudinal assessment of re-innervation of the cardiac graft, short-term testing may offer an easily applicable and non-invasive diagnostic tool. Further studies are warranted to investigate whether HRV testing may contribute to rule out acute graft rejection.

Die Synkope – ein kurz dauernder, spontan reversibler Anfall von Bewusstlosigkeit – stellt ein haufiges Krankheitsbild im Kindes- und Jugendalter dar. In der diagnostischen Abklarung steht die Unterscheidung zwischen harmloseren, neural vermittelten Ohnmachtsanfallen sowie Synkopen aufgrund organischer Erkrankungen im Mittelpunkt. Mit Hilfe von Anamnese, korperlicher Untersuchung sowie Ruhe-EKG ist sie in vielen Fallen moglich. Weitergehende Untersuchungen sind v. a. bei vermuteter organischer Ursache (neurologisch, kardial, psychiatrisch, endokrinologisch/metabolisch bedingte Synkope) indiziert. Wahrend bei der neural vermittelten Synkope konservative Masnahmen und ggf. eine medikamentose Therapie zum Erfolg fuhren konnen, steht bei organisch bedingten Anfallen die Behandlung des Grundleidens im Vordergrund. Die Prognose neural vermittelter Synkopen ist exzellent; unter den organisch bedingten Synkopen sind v. a. die kardialen Synkopen wegen ihrer hohen Mortalitat hervorzuheben.

Background: Adults born small for gestational age (SGA) are at increased risk for the metabolic syndrome and cardiovascular disease. Hypothesis: Impaired short-term blood pressure regulation may contribute to the development of hypertension in patients born SGA. Methods: In all, 43 patients born SGA (18 female, age 19.4 ± 0.3 years) were evaluated by beat-to-beat blood pressure and heart rate registration during rest and mental and orthostatic stress. The study group was divided into Group 1 with normal resting blood pressure (n = 32) and Group 2 with slightly elevated blood pressure (n = 11). Baroreceptor sensitivity (BRS) was calculated. Fasting insulin as well as lipid levels were correlated with hemodynamic parameters. Results: Eleven of the 43 study patients (25%) had a slightly elevated resting systolic blood pressure (SBP) rising during mental and orthostatic stress. Body mass index (BMI) and fasting insulin levels correlated strongly with SBP in Group 2. Baroreceptor sensitivity was lower in Group 2 at rest (p

Being overweight in childhood causes several cardiovascular risk factors which in turn contribute to accelerated atherosclerosis. Being overweight itself represents a risk factor, but also contributes to an increased prevalence of arterial hypertension, dyslipidemia and impaired glucose tolerance. Thus, cardiovascular prevention should be included in the management of obese children. Most of all, therapy of adiposity should be performed, as weight reduction and increased fitness represent protective factors. Moreover, a detailed cardiovascular workup and therapy of secondary vascular disease must also be performed. Subclinical changes at the level of the endothelium may be diagnosed using modern imaging techniques such as the measurement of the intima-media thickness of the carotid artery. In general, the overweight child should be considered as a future patient with vascular disease! The following article focuses on the prevalence, diagnostics and therapeutic options in the cardiovascular management of overweight children.

Background: Inflammation and glucocorticoid therapy are major factors influencing growth and bone maturation in patients with juvenile idiopathic arthritis (JIA). In addition to alterations in total bone mineral density and bone geometry, longitudinal data confirm that the main contributors to errant bone maturation in patients with JIA are reductions in muscle mass and force. Growth hormone (GH) therapy, which has shown efficacy in controlling disease, may also positively influence body composition. For several years, GH therapy has been used to treat growth retardation in patients with JIA receiving glucocorticoids. GH therapy normalizes growth velocity, increases height, bone mineral density and bone mass and changes bone geometry. Despite ongoing glucocorticoid therapy, muscle mass and bone size substantially increase with GH therapy. Increased bone size suggests improved bone stability, which may reduce fracture risk. Along with the increase in muscle mass, patients experience stabilized or slightly decreased fat mass during GH therapy. Conclusions: All these effects suggest an anabolic effect of GH therapy on bone and body composition.

In the first two yr of life blood-group incompatible (ABO-incompatible) heart transplantation can be performed leading to immune tolerance to donor blood group. Antibody titers should be below 1:4. VAD use is correlated with sensitization toward blood-group antigens. A boy was diagnosed with dilated cardiomyopathy at nine months of age and listed for 0-compatible transplantation. Progressive heart failure required implantation of a left VAD. His listing was extended for ABO-incompatible transplantation despite antibody titers of 1:32 anti-A and 1:8 anti-B. After 26 days on VAD, he was transplanted with a B donor heart. No hyperacute or acute rejection occurred in 12 months post-transplant. Anti-B antibodies rose to a maximum of 1:2. No use of rituximab or plasmapheresis was required. There are no signs of graft vasculopathy. This indicates that inclusion criteria for ABO-incompatible transplantation may be extended to immediate cases. This is the first case with a healthy immune system to show signs of tolerance development after ABO-incompatible heart transplantation with increased prior antibody titers and without specific treatment.