An Echocardiographic Screening Program Helps to Identify Pulmonary Hypertension in Extremely Low Birthweight Infants with and without Bronchopulmonary Dysplasia: A Single-Center Experience.

Background: Pulmonary hypertension (PH) affects 1 in 6 infants with a birthweight <1,000 g (extremely low birthweight; ELBW) and is frequently associated with bronchopulmonary dysplasia (BPD). If untreated, the mortality rates of the disease are high.

Objectives: The aim of this study was to characterize risk factors for PH in ELBW infants and to describe the timing of onset of the disease by setting up a screening program.

Disease control via intensified lipoprotein apheresis in three siblings with familial hypercholesterolemia.

Background: Familial hypercholesterolemia (FH), the prevalent monogenic form of hypercholesterolemia, carries the risk of premature coronary heart disease. Lipoprotein-apheresis is established in children with severe dyslipidemia. We present 3 siblings with a negative/negative residual low-density lipoprotein (LDL) receptor mutation (p.

Towards a proposal for a universal diagnostic definition of protein-losing enteropathy in Fontan patients: a systematic review.

Objective: A standardised diagnostic definition of protein-losing enteropathy (PLE) in Fontan patients serves both patient care and research. The present study determined whether a diagnostic definition of PLE was routinely used in published clinical Fontan studies, and to identify potentially relevant diagnostic criteria for composing a uniform PLE definition.

Methods: A systematic review was conducted in adherence to the PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analyses) recommendations.

Low serum 25-hydroxyvitamin D levels and secondary hyperparathyroidism in Fontan patients.

Background: Limited data exist on the vitamin D status in Fontan patients. We determined the prevalence and potential risk factors of vitamin D deficiency in this patient subset. Methods and results Data were collected from 27 Fontan patients (55.

Controversies in arrhythmias and arrhythmic syndromes of active children and young adults.

Important advances in the diagnosis and therapy of various arrhythmic disorders have been made in the last two decades. These, in turn, have necessitated a re-examination of current practice guidelines, with a view to deciding on optimal management of young patients with suspected or proven arrhythmia syndromes and in assessing the risk of adverse arrhythmic events during sport participation. There has also been a concomitant emphasis on identifying individuals at risk by nationwide screening programs using the ECG and excluding them from competitive sport.

Inhomogeneous Longitudinal Cardiac Rotation and Impaired Left Ventricular Longitudinal Strain in Children and Young Adults with End-Stage Renal Failure Undergoing Hemodialysis.

Background: Cardiac dysfunction frequently complicates the clinical course of patients with end-stage renal failure (ESRF). Recently, we observed abnormal longitudinal cardiac rotation (LR) among patients with ESRF. In this study, we sought to quantify LR mechanics in patients undergoing hemodialysis (HD).

The pathophysiologic aspects and clinical implications of electrocardiographic parameters of ventricular conduction delay in repaired tetralogy of Fallot.

The 12-lead surface electrocardiogram is a valuable and feasible clinical tool in the management of patients following tetralogy of Fallot (TOF) repair. The importance of QRS duration in TOF patients has long been acknowledged. A prolonged QRS complex has been associated with increased risk for subsequent life-threatening ventricular arrhythmia and sudden cardiac death.

Fast diastolic swinging motion of the mitral valve as a clinical marker of familial hypertrophic cardiomyopathy in genetically affected young children without left ventricular hypertrophy: a new role for noninvasive imaging?

Structural mitral valve (MV) abnormalities are common in patients with hypertrophic cardiomyopathy (HCM). This is the first report demonstrating MV abnormalities in very young children as the sole overt clinical feature of a known HCM-causing sarcomere protein gene mutation. Due to MV leaflet elongation, we also noticed a typical fast diastolic swinging motion of the MV in our patients.

Stenting the arterial duct in neonates and infants with congenital heart disease and duct-dependent pulmonary blood flow: a multicenter experience of an evolving therapy over 18 years.

Objectives: The primary aim of this multi-institutional study was to describe our 18-year experience of ductal stenting (DS) in infants with a duct-dependent pulmonary circulation. The secondary aim sought to identify a subgroup of patients who may benefit the most using this evolving technique.

Background: No study has examined the extraordinary evolution of this promising therapy over the last two decades.

Use of an active fixation lead and a subpectoral pacemaker pocket may not avoid Twiddler's syndrome.

Manipulation of a pacemaker with consequent malfunction of the device has been called Twiddler's syndrome. Use of active-fixation leads and subpectoral pacemaker pockets has been considered to help in avoiding this problem. We describe a child in whom twiddling was not prevented despite implantation of a lumenless atrial lead and insertion of the pacemaker generator in a subpectoral pocket.

Use of propofol in pediatric intensive care units: a national survey in Germany.

Objective: Propofol is not licensed for sedation in pediatric intensive care medicine mainly due to the risk of propofol infusion syndrome. Nevertheless, it is applied by many pediatric intensive care units. The aim of this national survey was to asses the current use of propofol in pediatric intensive care units in Germany.

Dyssynchronous ventricular activation in asymptomatic wolff-Parkinson-white syndrome: a risk factor for development of dilated cardiomyopathy.

A subset of children and adults with Wolff-Parkinson-White (WPW) syndrome develop dilated cardiomyopathy (DCM). Although DCM may occur in symptomatic WPW patients with sustained tachyarrhythmias, emerging evidence suggests that significant left ventricular dysfunction may arise in WPW in the absence of incessant tachyarrhythmias. An invariable electrophysiological feature in this non-tachyarrhythmia type of DCM is the presence of a right-sided septal or paraseptal accessory pathway.

Dilated cardiomyopathy in children with ventricular preexcitation: the location of the accessory pathway is predictive of this association.

Background: Ventricular preexcitation may be associated with dilated cardiomyopathy, even in the absence of recurrent and incessant tachycardia.

Methods: This report describes the clinical and electrophysiologic characteristics of 10 consecutive children (6 males), with median age of 8 years (range, 1-17 years), who presented with dilated cardiomyopathy and overt ventricular preexcitation on the 12-lead electrocardiogram. Incessant tachycardia as the cause of dilated cardiomyopathy could be excluded.

Transient neonatal myelosuppression after fetal exposure to maternal chemotherapy. Case report and review of the literature.

Transient neonatal myelosuppression (TNM) is a rare but potentially life-threatening adverse effect of fetal exposure to maternal chemotherapy during pregnancy. We report a case of TNM in a preterm infant born to a mother diagnosed with acute lymphoblastic leukemia during pregnancy. The mother received chemotherapy during the second and third trimester.

Incidence and risk factors of prolonged mechanical ventilation in neuromuscular scoliosis surgery.

Patients with neuromuscular scoliosis (NMS) are frequently considered at high risk for postoperative complications based on their underlying disease and comorbidities. Postoperative complications include prolonged mechanical ventilation (MV), defined longer than 72 h, at the paediatric intensive care unit. The objectives of this retrospective study were to assess the incidence of prolonged MV in patients with NMS following scoliosis surgery and to identify predictive risk factors.

Transient electrocardiographic abnormalities following blunt chest trauma in a child.

Blunt cardiac injury may occur in patients after suffering nonpenetrating trauma of the chest. It encompasses a wide spectrum of cardiac injury with varied severity and clinical presentation. Electrocardiographic abnormalities are frequently encountered.

Pacemaker therapy in isolated congenital complete atrioventricular block.

The aim of this study was to evaluate the effect of pacemaker (PM) therapy in patients with isolated congenital complete atrioventricular block (CCAVB). Patients with CCAVB eventually quality for PM implantation, however, timing remains controversial. Retrospective evaluation of left ventricular end-diastolic diameter (LVEDD), shortening fraction (SF), and cardiothoracic ratio (CTR) in 149 CCAVB patients, before, at, and after PM implantation was carried out.