Feasibility, safety and diagnostic impact of endomyocardial biopsies for the diagnosis of myocardial disease in children and adolescents.

Aims: Endomyocardial biopsies (EMBs) are performed infrequently in children owing to significant past complication rates and controversial discussions about the therapeutic value of results. The objective of this study was to investigate the safety and feasibility of EMBs for suspected myocardial disease in relation to their clinical value.

Methods And Results: We performed a retrospective multicentre review of the Working Group for Interventional Cardiology of the German Society for Paediatric Cardiology.

The Pulmonary Artery in Pediatric Patients with Marfan Syndrome: An Underestimated Aspect of the Disease.

Aortic root dilatation and its complications are known to be the most important and life limiting features in patients with Marfan syndrome (MFS). Since monitoring of patients, preventive medical and surgical treatments are available nowadays, other MFS pathologies are becoming more relevant for the outcome of the disease. Main pulmonary artery (MPA) dilatation is a cardiac finding, which has not been fully investigated in children.

Toward evidence-based diagnosis of myocarditis in children and adolescents: Rationale, design, and first baseline data of MYKKE, a multicenter registry and study platform.

Unlabelled: The aim of this registry is to provide data on age-related clinical features of suspected myocarditis and to create a study platform allowing for deriving diagnostic criteria and, at a later stage, testing therapeutic interventions in patients with myocarditis.

Study Design And Results: After an initial 6-month pilot phase, MYKKE was opened in June 2014 as a prospective multicenter registry for patients from pediatric heart centers, university hospitals, and community hospitals with pediatric cardiology wards in Germany. Inclusion criteria consisted of age<18 years and hospitalization for suspected myocarditis as leading diagnosis at the discretion of the treating physician.

Cardiovascular biomarkers in paired maternal and umbilical cord blood samples at term and near term delivery.

Background: Cardiovascular biomarkers might help to identify fetuses or pregnancies at risk.

Aim: To examine the umbilical cord neonatal and maternal levels of cardiovascular biomarkers at the time of delivery, and to correlate maternal and fetal biomarker levels to each other, to gestational age and to delivery mode.

Study Design: In a prospective, observational, cross-sectional, single-center study biomarkers were measured in paired maternal and umbilical venous cord blood samples.

Health-related quality of life is unimpaired in children and adolescents with Marfan syndrome despite its distinctive phenotype.

Aim: Marfan syndrome (MFS) is a progressive, life-threatening genetic disorder of the connective tissue, which causes impaired quality of life (QoL) in adults. This study investigated the quality of life in children and adolescents, taking into account their gender, age and how MFS affected their organs.

Methods: This prospective nonrandomised single-centre study included 46 patients with verified MFS with a mean age of 10.

Carcinoid Heart Valve Disease: Transcatheter Pulmonary Valve-In-Valve Implantation in Failing Biological Xenografts.

Background And Aim Of The Study: Carcinoid heart valve disease (CHVD) occurs as the cardiac manifestation of carcinoid syndrome (also known as Hedinger's syndrome), which develops secondary to neuroendocrine tumor activity. CHVD almost exclusively affects right-sided heart valves, since circulating serotonin is metabolized by pulmonary endothelial cells, thus sparing left-sided valves. Replacement of the tricuspid and pulmonary valve is a well-established and feasible therapeutic option for these patients.

Kid-Short Marfan Score (Kid-SMS) Is a Useful Diagnostic Tool for Stratifying the Pre-Test Probability of Marfan Syndrome in Childhood.

Due to age dependent organ manifestation, diagnosis of Marfan syndrome (MFS) is a challenge, especially in childhood. It is important to identify children at risk of MFS as soon as possible to direct those to appropriate treatment but also to avoid stigmatization due to false diagnosis. We published the Kid-Short Marfan Score (Kid-SMS) in 2012 to stratify the pre-test probability of MFS in childhood.

Impedance cardiography (electrical velocimetry) and transthoracic echocardiography for non-invasive cardiac output monitoring in pediatric intensive care patients: a prospective single-center observational study.

Introduction: Electrical velocimetry (EV) is a type of impedance cardiography, and is a non-invasive and continuously applicable method of cardiac output monitoring. Transthoracic echocardiography (TTE) is non-invasive but discontinuous.

Methods: We compared EV with TTE in pediatric intensive care patients in a prospective single-center observational study.

Management of Pentalogy of Cantrell with complete ectopia cordis and Double Outlet Right Ventricle.

Pentalogy of Cantrell (PoC) is a rare congenital midline defect. We present a case and its treatment of PoC with complete ectopia cordis and congenital heart disease. Postnatally the congenital heart defect was surgically corrected and the ectopic heart was covered by musculous mobilized flap.

Retrospective analysis of the effect of angiotensin II receptor blocker versus β-blocker on aortic root growth in paediatric patients with Marfan syndrome.

Objectives: Cardiovascular pathology, including aortic root dilation at the level of sinus of Valsalva (SV), is one of the major causes of morbidity in paediatric patients with Marfan syndrome (MFS). β-Blocker (BB) is well established to slow aortic dilation in MFS. Less is known about the effectiveness of angiotensin II receptor blocker (ARB) on aortic dilation in paediatric patients with MFS.

Health-related quality of life compared with cardiopulmonary exercise testing at the midterm follow-up visit after tetralogy of Fallot repair: a study of the German competence network for congenital heart defects.

This nationwide study aimed to evaluate health-related quality of life (QoL) experienced by children after tetralogy of Fallot repair and to compare self-reported physical ability with objective exercise performance. This prospective nonrandomized, government-funded multicenter study enrolled 168 patients (70 girls; ages 8-16 years) after tetralogy of Fallot repair at eight German heart centers. Health-related QoL was analyzed by the self-reported KINDL-R quality-of-life questionnaire.

Prevalence of Anemia in Children with Congestive Heart Failure due to Dilated Cardiomyopathy.

Introduction. Anemia is prevalent in adult heart failure patients and appears to be an independent risk factor for morbidity and mortality. The purpose of this work is to determine the prevalence of anemia in children with heart failure from dilated cardiomyopathy (DCM) and to evaluate its influence on morbidity and mortality.

Impact of age and gender on cardiac pathology in children and adolescents with Marfan syndrome.

Cardiac pathologies are the major aspect in the treatment strategies for Marfan syndrome (MFS). In this progressive disease, less is known about manifestations and progression of cardiovascular symptoms in children. To define a certain decision regarding therapeutic options, knowledge concerning the onset of cardiovascular findings is essential.

The Kid-Short Marfan Score (Kid-SMS) - an easy executable risk score for suspected paediatric patients with Marfan syndrome.

Aim: Due to age-dependent manifestations, diagnosis of Marfan syndrome (MFS) in children and adolescents is sophisticated. Although revised Ghent criteria is a major step forward, its utility in children is still restricted due to expensive and technically advanced diagnostics. As early diagnosis submits long-term benefits concerning prognosis, the need of an appropriate diagnostic tool for risk stratification of suspected paediatric patients with Marfan is justified.

First experience with a new drug-eluting balloon for the treatment of congenital pulmonary vein stenosis in a neonate.

Paclitaxel-eluting balloons are a new and innovative method in the treatment of in-stent stenosis and small vessel disease in adult cardiac pathology. The treatment of congenital pulmonary vein stenosis is difficult to manage, and results in a high mortality rate due to residual or recurrent stenosis. We report the first case of treatment for neonatal pulmonary vein restenosis with a paclitaxel-eluting balloon.