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.

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.

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.

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).

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.