Exploring the Potential of a Tailored Executive Functioning Training Program for Children with Congenital Heart Disease: A Brief Report.

The aim of this pilot study was to explore whether executive functioning (EF) problems of children with severe congenital heart disease (CHD) could be improved through a personalized EF-training program. Eleven preschool-aged children (mean age = 62 months, SD = 6) received a standardized test battery. Eight children were eligible for training and six children participated in the training and posttest.

Emotional and behavioral problems in children with dilated cardiomyopathy.

Background: Dilated cardiomyopathy (DCM) in children is an important cause of severe heart failure and carries a poor prognosis. Adults with heart failure are at increased risk of anxiety and depression and such symptoms predict adverse clinical outcomes such as mortality. In children with DCM, studies examining these associations are scarce.

Orodispersible minitablets of enalapril for use in children with heart failure (LENA): Rationale and protocol for a multicentre pharmacokinetic bridging study and follow-up safety study.

Introduction: Treatment of paediatric heart failure is based on paradigms extensively tested in the adult population assuming similar underlying pathophysiological mechanisms. Angiotensin converting enzyme inhibitors (ACEI) like enalapril are one of the cornerstones of treatment and commonly used off-label in children. Dose recommendations have been extrapolated from adult experience, but the relationship between dose and pharmacokinetics (PK) in (young) children is insufficiently studied.

Additional value of three-dimensional rotational angiography in the diagnostic evaluation and percutaneous treatment of children with univentricular hearts.

Aims: Complex single ventricle topography, changes in vessel geometry after surgical steps and subsequent stenoses are difficult to visualise with biplane conventional angiography (CA). This study aimed to investigate the additional value of three-dimensional rotational angiography (3DRA) compared to CA for diagnostic and interventional purposes in children with univentricular hearts.

Methods And Results: Demographic data, clinical data and catheterisation details of both imaging techniques were collected retrospectively.

Normative Values of Aortic Arch Structures in Premature Infants.

Background: Aortic arch abnormalities represent 5% to 8% of all congenital heart disease. Measurements of the aortic arch dimensions on two-dimensional echocardiographic images remain of critical importance in the diagnosis of aortic arch pathology. To define aortic hypoplasia or coarctation, measured dimensions must be compared with normal values.

Surgical Repair of Ventricular Septal Defect; Contemporary Results and Risk Factors for a Complicated Course.

Surgical closure of the ventricular septal defect is the most commonly performed procedure in pediatric cardiac surgery. There are conflicting data on weight at operation as risk factor for a complicated course. We performed a retrospective evaluation of mortality and morbidity in all patients undergoing surgical ventricular septal defect closure at our institution between 2004 and 2012 to identify risk factor for a complicated course.

Treatment of infantile hemangiomas: therapeutic options in regard to side effects and adverse events - a review of the literature.

Introduction: While options for treatment strategies for infantile hemangiomas (IH) are numerous, evidence-based information about agents, optimal dosage, adverse effects, treatment modality, pretreatment and treatment strategies remain limited.

Areas Covered: To evaluate side effects and adverse events of medical treatment in children with infantile hemangioma, a comprehensive review of the literature was performed to provide information for daily practice. In total 254 studies were retrieved from medical databases and comprised 10,022 patients divided into 5 different treatment groups.

Hypertrophic cardiomyopathy in Donohue syndrome.

We report the case of a patient with Donohue syndrome who died of heart failure due to obstructive hypertrophic cardiomyopathy. A literature survey revealed that hypertrophic cardiomyopathy was present in 30% of these patients and was often fatal. Therefore, every patient with Donohue syndrome should be screened for hypertrophic cardiomyopathy.

Main pulmonary artery area limits exercise capacity in patients long-term after arterial switch operation.

Objectives: Despite excellent survival in patients after the arterial switch operation, reintervention is frequently required and exercise capacity is decreased in a substantial number of patients. This study relates right-sided imaging features in patients long-term after the arterial switch operation to exercise capacity and ventilatory efficiency to investigate which lesions are functionally important.

Methods: Patients operated in the UMC Utrecht, the Netherlands (1976-2001) and healthy controls underwent cardiac magnetic resonance imaging and cardiopulmonary exercise testing within 1 week.

The effect of short-term high versus normal protein intake on whole-body protein synthesis and balance in children following cardiac surgery: a randomized double-blind controlled clinical trial.

Background: Infants undergoing cardiac surgery are at risk of a negative protein balance, due to increased proteolysis in response to surgery and the cardiopulmonary bypass circuit, and limited intake. The aim of the study was to quantify the effect on protein kinetics of a short-term high-protein (HP) diet in infants following cardiac surgery.

Methods: In a prospective, double-blinded, randomized trial we compared the effects of a HP (5 g · kg(-1) · d(-1)) versus normal protein (NP, 2 g · kg(-1) · d(-1)) enteral diet on protein kinetics in children <24 months, on day 2 following surgical repair of congenital heart disease.

Is cardiovascular evaluation necessary prior to and during beta-blocker therapy for infantile hemangiomas?: A cohort study.

Background: Although consensus guidelines for pretreatment evaluation and monitoring of propranolol therapy in patients with infantile hemangiomas (IH) have been formulated, little is known about the cardiovascular side effects.

Objectives: We sought to analyze cardiovascular evaluations in patients with IH at baseline and during treatment with an oral beta-blocker.

Methods: Data from 109 patients with IH were retrospectively analyzed.

Advances in cardiac magnetic resonance imaging of congenital heart disease.

Due to advances in cardiac surgery, survival of patients with congenital heart disease has increased considerably during the past decades. Many of these patients require repeated cardiovascular magnetic resonance imaging to assess cardiac anatomy and function. In the past decade, technological advances have enabled faster and more robust cardiovascular magnetic resonance with improved image quality and spatial as well as temporal resolution.

Effective radiation dosage of three-dimensional rotational angiography in children.

Aims: Three-dimensional rotational angiography (3DRA) is a relatively new but promising imaging technique in the paediatric catheterization laboratory. However, data on effective dose (ED) of this technique in children are lacking. The purpose of this study is to provide ED of 3DRA and to correlate this with parameters readily available in daily practice.

Further confirmation of the MED13L haploinsufficiency syndrome.

MED13L haploinsufficiency syndrome has been described in two patients and is characterized by moderate intellectual disability (ID), conotruncal heart defects, facial abnormalities and hypotonia. Missense mutations in MED13L are linked to transposition of the great arteries and non-syndromal intellectual disability. Here we describe two novel patients with de novo MED13L aberrations.

Five years of Kawasaki disease in the Netherlands: a national surveillance study.

Background: The aim of this study was to evaluate the incidence, disease presentation, treatment and cardiac outcome of Kawasaki disease (KD) in The Netherlands.

Methods: The national Dutch Pediatric Surveillance Unit was used to prospectively register new KD cases from 2008 through 2012. Questionnaires were sent to pediatricians to obtain clinical information.

Congenital cardiac, aortic arch, and vascular bed anomalies in PHACE syndrome (from the International PHACE Syndrome Registry).

PHACE syndrome represents the association of large infantile hemangiomas of the head and neck with brain, cerebrovascular, cardiac, ocular, and ventral or midline defects. Cardiac and cerebrovascular anomalies are the most common extracutaneous features of PHACE, and they also constitute the greatest source of potential morbidity. Congenital heart disease in PHACE is incompletely described, and this study was conducted to better characterize its features.

A Patient with Congenital Generalized Lipodystrophy Due To a Novel Mutation in BSCL2: Indications for Secondary Mitochondrial Dysfunction.

Background: Congenital generalized lipodystrophy (CGL) results from mutations in AGPAT2, encoding 1-acyl-glycerol-3-phosphate-acyltransferase 2 (CGL1; MIM 608594), BSCL2, encoding seipin (CGL2; MIM 269700), CAV1, encoding caveolin1 (CGL3; MIM 612526) or PTRF, encoding polymerase I and transcript release factor (CGL4; MIM 613327). This study aims to investigate the genotype/phenotype relationship and search for a possible pathogenic mechanism in a patient with CGL.

Design: Case report.

The diagnostic and therapeutic aspects of loss-of-function cardiac sodium channelopathies in children.

Background: Loss-of-function sodium channelopathies manifest as a spectrum of diseases including Brugada syndrome (BrS) and cardiac conduction disease.

Objective: To analyze the diagnostic and therapeutic aspects of these disorders in children.

Methods: Patients aged ≤ 16 years with genetically confirmed loss-of-function sodium channelopathies (SCN5A mutation), presenting with cardiac symptoms, positive family history, and/or abnormal electrocardiogram (ECG), were included.

Cardiac function in 7-8-year-old offspring of women with type 1 diabetes.

Offspring of type 1 diabetic mothers (ODMs) are at risk of short-term and long-term complications, such as neonatal macrosomia (birth weight >90th percentile), hypertrophic cardiomyopathy, and cardiovascular morbidity in later life. However, no studies have been performed regarding cardiac outcome. In this study, we investigated cardiac dimensions and function in 30 ODMs at 7-8 years of age in relation to neonatal macrosomia and maternal glycemic control during pregnancy and compared these with those in a control group of 30 children of nondiabetic women.

Persistent fetal sinus bradycardia associated with maternal anti-SSA/Ro and anti-SSB/La antibodies.

Objective: To study the clinical course and outcome of fetal sinus bradycardia (SB) due to maternal antibody-induced sinus node dysfunction.

Methods: We reviewed the maternal, prenatal, and postnatal findings of fetuses with SB associated with elevated maternal anti-SSA/Ro and anti-SSB/La antibodies.

Results: Of the 6 cases diagnosed prenatally, 3 had isolated SB persisting after birth and had a good prognosis.

Hypoglycemia as a result of propranolol during treatment of infantile hemangioma: a case report.

Propranolol is a new and promising treatment for hemangiomas of infancy. We report of a patient in whom steroid maintenance therapy is successfully tapered after introduction of propranolol. This patient, however, developed symptomatic hypoglycemic events presumably because of a concurrent deficiency of epinephrine and cortisol as a direct result of both beta-blockage by propranolol and adrenal insufficiency as a result of prednisone use.

Segmental facial hemangiomas and associated structural defects.

PHACE syndrome refers to the association of large segmental facial hemangiomas with 1 or more of the following anomalies: posterior fossa malformations, arterial anomalies, cardiac anomalies, and eye abnormalities. In this review, we present a newborn with a large segmental facial hemangioma and abnormal genesis of the cerebropetal arteries. Furthermore, we give an overview of the anomalies associated with the PHACE syndrome.

Isolated congenital atrioventricular block diagnosed in utero: natural history and outcome.

Background: Isolated congenital atrioventricular block (CAVB) diagnosed in utero is associated with a high morbidity and mortality. Prognosis is especially poor when heart rate drops below 55 beats per minute (bpm) and when fetal hydrops develops. We describe the natural history and outcome of 24 infants with isolated CAVB diagnosed in utero, review the literature, and assess the risk factors that could predict outcome.