Endothelial damage in long-term survivors of childhood cancer.

Purpose: To evaluate the presence of vascular damage in long-term childhood cancer survivors (CCS) and sibling controls, and to evaluate the association between vascular damage parameters and cancer treatment and influence of cardiovascular risk factors.

Patients And Methods: Vascular assessment was performed in 277 adult CCSs (median age at diagnosis, 9 years; range, 0 to 20 years; median current age, 28 years; range, 18 to 48 years) treated with potentially cardiovascular toxic anticancer treatment (ie, anthracyclines, platinum, and/or radiotherapy [RT]). Measurements included carotid- and femoral-wall intima-media thickness (IMT), flow-mediated vasodilatation of the brachial artery by ultrasound, assessment of endothelial and inflammatory marker proteins (including tissue-type plasminogen activator [t-PA], plasminogen activator inhibitor type 1 [PAI-I]), and cardiovascular risk factors.

Systolic and diastolic dysfunction in long-term adult survivors of childhood cancer.

Aim: To assess systolic and diastolic function in adult childhood-cancer survivors (CCS) after treatment entailing potential cardiovascular toxicity.

Methods: The study cohort consisted of 277 adult CCS (median age 28 [range 18-48]years), who had been treated with anthracyclines, platinum, and/or radiotherapy between 1976 and 1999, along with 130 healthy sibling controls. The assessments included echocardiography, baroreflex sensitivity measurement, and plasma N-terminal pro-brain natriuretic peptide (NT-proBNP).

Low-dose anthracyclines in childhood Acute Lymphoblastic Leukemia (ALL): no cardiac deterioration more than 20 years post-treatment.

Introduction: In children with cancer a well-known risk factor for cardiotoxicity is a high cumulative dose of anthracyclines, but little is known about cardiac function in low-dose anthracycline-treated survivors. Also, it is unclear if a safe anthracycline-dose exists at all.

Patients And Methods: Cardiac function was assessed in 23 long-term ALL-survivors with a median follow-up of 22 years (range 19.

Natural history of ventricular premature contractions in children with a structurally normal heart: does origin matter?

Aims: Premature ventricular contractions (PVCs) are thought to be innocent in children with normal hearts, especially if they disappear during exercise. The aim of our study was to study the natural history of PVCs in childhood and whether there is a difference between PVCs originating from the right [premature ventricular contraction with left bundle branch block (PVC-LBBB)] or the left ventricle [premature ventricular contraction with right bundle branch block (PVC-RBBB)].

Methods And Results: We evaluated children with frequent PVCs and anatomically normal hearts (n= 59; 35M/24F) by 12-lead ECG, echocardiography, Holter recording, and an exercise test.

Drug management of fetal tachyarrhythmias: are we ready for a systematic and evidence-based approach?

Fetal tachyarrhythmias are a life-threatening condition complicating a small proportion of normal pregnancies. Despite major advances in the (intrauterine) pharmacologic treatment of these arrhythmias over the last years major uncertainties remain. Among these are controversies in the choice of agents in relation to arrhythmia type, and timing and duration of treatment.

Longitudinal echocardiographic follow-up in children with congenital complete atrioventricular block.

Background: Due to a low heart rate (HR) in children with congenital complete atrioventricular block (CCAVB), a larger stroke volume of the left ventricle (LV) may be expected. If so, end-diastolic (LVEDD) and end-systolic (LVESD) diameters may be enlarged and even dilated cardiomyopathy (DCM) may occur. The aim of this study was to answer the question if children with CCAVB develop LV dilatation.

Expanding spectrum of human RYR2-related disease: new electrocardiographic, structural, and genetic features.

Background: Catecholaminergic polymorphic ventricular tachycardia is a disease characterized by ventricular arrhythmias elicited exclusively under adrenergic stress. Additional features include baseline bradycardia and, in some patients, right ventricular fatty displacement. The clinical spectrum is expanded by the 2 families described here.

Sinus node function in children with congenital complete atrioventricular block.

Aims: Children with congenital complete atrioventricular block (CCAVB) often need pacemaker therapy. In these children, it may be preferable to use single-lead VDD pacing, but for VDD pacing a normal sinus node function is required. Our aim was to study sinus node function in children with CCAVB.

Long-term cardiac follow-up in survivors of a malignant bone tumour.

Background: Longitudinal studies of cardiac function in long-term childhood cancer survivors are scarce and frequently concern a median follow-up shorter than 13 years.

Patients And Methods: Cardiac assessment was performed in 22 doxorubicin-treated long-term survivors of a malignant bone tumour at median 22 years (range 15-27.5) post-treatment.

Developmental aspects of long QT syndrome type 3 and Brugada syndrome on the basis of a single SCN5A mutation in childhood.

Objectives: The aim was to investigate at what age electrocardiographic characteristics of long QT syndrome type 3 (LQT3) and Brugada syndrome (BS), based on a single SNC5A mutation, appear.

Background: The QT interval (QT) in LQT3 is prolonged during bradycardia. It is not clear yet if this is obvious in young children with a relative fast heart rate (HR).

Implantable cardioverter defibrillator implantation in children in The Netherlands.

Unlabelled: To evaluate the indications, underlying cardiac disorders, efficacy and complications involved with implantable cardioverter-defibrillators (ICDs) in paediatric patients in The Netherlands, the records of all patients aged 18 years or younger who underwent ICD placement were reviewed retrospectively. Between January 1995 and September 2002, 23 patients (11 males, 12 females; median age 12 years, range 6 months to 16 years) underwent ICD implantation. The ICD was implanted for aborted sudden cardiac death ( n = 14), syncope ( n = 5) or for primary prevention of sudden cardiac death ( n = 4).

Predictors of sudden cardiac death after Mustard or Senning repair for transposition of the great arteries.

Objectives: The goal of this research was to identify predictors for sudden death (SD) in patients with transposition of the great arteries (TGA) who have undergone atrial inflow repair.

Background: Sudden death is the most common cause of late death after atrial inflow repair of TGA. Little is known about the predictors of SD.

Ventricular fibrillation without overt cardiomyopathy as first presentation of organic cation transporter 2-deficiency in adolescence.

This case report describes ventricular fibrillation without overt cardiomyopathy as the presenting symptom of primary carnitine deficiency due to organic cation transporter 2 (OCTN2)-deficiency in a 15-year-old girl. Normally this disease presents early in life with hypoketotic hypoglycemia, muscle weakness, and/or cardiomyopathy. The patient fully recovered after carnitine supplementation.

Pharmacological treatment of young children with permanent junctional reciprocating tachycardia.

Our objective was to assess the efficacy of pharmacological treatment in reducing the incidence of permanent junctional reciprocating tachycardia in young children, or to bring the mean heart rate over 24 h to a normal level. We included 21 children with a median age of 0.05 year seen with permanent junctional reciprocating tachycardia over the period 1990 through 2001.

A large family characterised by nocturnal sudden death.

Background: We recently identified a novel mutation in large family characterised by premature nocturnal sudden death. In the present paper we provide an overview of the findings in this family.

Methods: From 1958 onwards, when the first patient presented, we collected clinical data on as many family members as possible.

Cardiac status in bone tumor survivors up to nearly 19 years after treatment with doxorubicin: a longitudinal study.

Background: Longitudinal assessment of cardiac toxicity in anthracycline-treated long-term bone tumor survivors.

Procedures: Cardiac status was assessed in 29 patients 14.1 (range 7-18.

A sodium-channel mutation causes isolated cardiac conduction disease.

Cardiac conduction disorders slow the heart rhythm and cause disability in millions of people worldwide. Inherited mutations in SCN5A, the gene encoding the human cardiac sodium (Na+) channel, have been associated with rapid heart rhythms that occur suddenly and are life-threatening; however, a chief function of the Na+ channel is to initiate cardiac impulse conduction. Here we provide the first functional characterization of an SCN5A mutation that causes a sustained, isolated conduction defect with pathological slowing of the cardiac rhythm.

3-methylcrotonyl-CoA carboxylase deficiency in an infant with cardiomyopathy, in her brother with developmental delay and in their asymptomatic father.

Unlabelled: Three affected members of one family, each with a different clinical presentation of isolated biotin-resistant 3-methylcrotonyl-CoA carboxylase (MCC) deficiency are described. The index patient presented at 7 weeks of age with feeding difficulties, sweating and tachypnoea. Echocardiography showed a severely dilated left ventricle with minimal contractility.

Pharmacologic management of arrhythmias.

The role of antiarrhythmic drugs in the management of children with arrhythmias has changed due to the rapid development of radiofrequency ablation. Moreover, the release of new drugs and a better insight into the electrophysiologic mechanisms of arrhythmias have changed former patterns of drug management. However, because of lack of controlled trials, arrhythmia management in pediatrics is still mainly based on clinical studies and individual experience.

Low-dose daunorubicin in induction treatment of childhood acute lymphoblastic leukemia: no long-term cardiac damage in a randomized study of the Dutch Childhood Leukemia Study Group.

Background: To investigate late cardiotoxicity in childhood acute lymphoblastic leukemia (ALL) survivors after induction treatment with or without daunorubicin (DNR; 25 mg/m(2), i.v., weekly, x4, cumulative dose 100 mg/m(2)).

A single Na(+) channel mutation causing both long-QT and Brugada syndromes.

Mutations in SCN5A, the gene encoding the cardiac Na(+) channel, have been identified in 2 distinct diseases associated with sudden death: one form of the long-QT syndrome (LQT(3)) and the Brugada syndrome. We have screened SCN5A in a large 8-generation kindred characterized by a high incidence of nocturnal sudden death, and QT-interval prolongation and the "Brugada ECG" occurring in the same subjects. An insertion of 3 nucleotides (TGA) at position 5537, predicted to cause an insertion of aspartic acid (1795insD) in the C-terminal domain of the protein, was linked to the phenotype and was identified in all electrocardiographically affected family members.

Junctional ectopic tachycardia evolving into complete heart block.

Transition from congenital junctional ectopic tachycardia to complete AV block was observed in an 8 month old girl, over a 36 hour period, during initial hospital admission. Two years later she had evidence of a rapidly increasing left ventricular end diastolic diameter, associated with lowest heart rates during sleep of < 30 beats/min. A transvenous permanent pacemaker was therefore implanted.