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Arrhythmogenic right ventricular dysplasia (ARVD) is a complex arrhythmogenic cardiomyopathy, characterized by a partial or total replacement of the right ventricular myocytes by fatty and fibrous tissue. In this study, we present a case of ARVD in 17 year old girl, who was admitted to the hospital after syncope with ventricular arrhythmia. The echocardiography did not demonstrate structural cardiac abnormalities but the magnetic resonance recently showed thinning of the right ventricular wall. The girl was treated with the lidocaine, amiodarone and next, after radiofrequency catheter ablation she was receiving metoprolol. The girl has remained asymptomatic for four years of follow-up. [ABSTRACT FROM AUTHOR]

Background: Ventricular arrhythmias are the most common consequences of structural and functional heart diseases, but cases with no evident pathology are also observed. A parameter indicating asymptomatic circulatory failure could support decisions related to possible treatment of ventricular arrhythmias., Hypothesis: The study objective was the evaluation of N-terminal prohormone brain natriuretic peptide (NT-proBNP) levels in children with ventricular arrhythmias and an attempt to determine if this parameter may be used for diagnosis and prognosis of ventricular arrhythmias., Material and Methods: The study population was comprised of 36 children age 5 to 17.5 years old with idiopathic ventricular arrhythmias (Group B) graded mild or potentially malignant; 29 patients with mild ventricular arrhythmias were included into Group B1; and 7 patients with potentially malignant cases into Group B2. In all the patients, NT-proBNP assays were performed., Results: The NT-proBNP levels in Groups B, B1, B2 and the control group (Group K) were as follows: 41.5 +/- 15.1 pg/mL, 35.5 +/- 18.5 pg/mL, 66.3 +/- 24.9 pg/mL and 31.5 +/- 15.1 pg/mL, respectively. Between the groups with and without arrhythmias (Group B vs Group K), no statistically significant differences in NT-proBNP levels were found. However, markedly higher NT-proBNP levels were shown in the children with potentially malignant arrhythmias (Group B2) compared to the patients with mild arrhythmias (B1) and the control group (Group K)., Conclusions: The level of NT-proBNP increases with the severity of ventricular arrhythmia. NT-proBNP assays can be helpful for diagnosing and grading the severity of ventricular arrhythmias.

Background: Mitral valve prolapse (MVP) is the most common primary valvular abnormality in a young population. In some individuals, MVP is silent or associated with palpitations, dizziness, chest pain, and abnormal electrocardiogram (ECG) repolarization with or without ventricular arrhythmias., Hypothesis: The aim of the present study was to assess the occurrence of the clinical and electrocardiographic abnormalities in young athletes with silent MVP., Methods: A group of 10 children, who have been sport training intensively, with preparticipation silent MVP were examined for symptoms and/or ECG abnormalities. The diagnosis of MVP was made by echocardiography., Results: Three athletes were asymptomatic at initial presentation. The other 7 athletes presented with symptoms. The QTc intervals > 440 msec were recorded in 2 athletes (1 with syncope). Abnormal ECG repolarization was found in 7 athletes (4 athletes were symptomatic and 3 were asymptomatic). A large variety of T-waves was registered in athletes who presented with symptoms. In asymptomatic athletes, the tall and flat T-waves were recorded., Conclusions: Young athletes with MVP are often predisposed to electrocardiographic abnormalities of ventricular repolarization, which requires annual cardiologic evaluation.

This review discusses the risk of cardiac events and genotype-based management of LQTS. We describe here the genetic background of long QT syndrome and the eleven different genes for ion-channels and a structural anchoring protein associated with that disorder. Clinical Background section discusses the risk of cardiac events associated with different LQTS types. Management and Prevention section describes in turn gene-specific therapy, which was based on the identification of the gene defect and the dysfunction of the associated transmembrane ion channel. In patients affected by LQTS, genetic analysis is useful for risk stratification and for making therapeutic decisions. A recent study reported a quite novel pathogenic mechanism for LQTS and suggested that treatments aimed at scaffolding proteins rather than specific ion channels may be an alternative to antiarrhythmic strategy in the future.

We describe the clinical and genetic characteristics of the family, in which the diagnosis of LQT1 had been made. The electrocardiogram (ECG) characteristics of this patient indicated the likelihood of LQTS1. Polymorphic ventricular extrasystolies and episodes of polymorphic non-sustained ventricular tachycardia were confirmed by Holter ECG monitoring. On the exertional electrocardiogram polymorphic ventricular tachycardia (torsade de pointes) was recorded. Direct sequencing of both DNA strands revealed the absence of mutations or polymorphisms in the KCNQ1, HERG, and SCN5A genes.

Background: About 5-15% of children suffer from hypertension. The influence of maturity and the physical development of newborns on primary hypertension has been recently underlined., Aim: The aim of the study was to estimate the importance of low birth weight (< 10 percentiles--2500 g), low birth length (< 10 percentiles--50 cm), low Apgar score (< 4 points), prematurity (< 37 Hbd) in the developmental of hypertension in children., Methods: The project was conducted retrospectively in a group of 197 children with primary hypertension (aged 15.4 +/- 1.9) and 119 healthy children (aged 12.9 +/- 3.1) as the control group. The statistical analysis was performed with Epilnfo 6.0 procedures., Results: Male sex, overweight and family history of hypertension increased the risk of hypertension in a statistically significant way and the adequate estimated odds ratios were: OR = 3.69 (95% CI: 2.20-6.19), OR = 22.76 (95% CI: 6.61-94.12), OR = 21.87 (95% CI: 6.27-91.1). No influence of low birth weight, low birth length, low Apgar score and prematurity on primary hypertension was observed., Conclusion: The results suggest that the role of low birth weight, low birth length, low Apgar score and prematurity in the development of hypertension in children is arguable. It also suggests the necessity to conduct epidemiologic surveys in more frequent groups of children.

There is some evidence that deaf children are more threatened than the general population by dangerous heart arrhythmias. An example is Jervell-Lange-Nielsen syndrome (one of the forms of long QT syndrome) which is characterised primarily by congenital deafness and prolongation of the QT interval. The aim of this study was to perform preliminary cardiological examinations on 162 deaf children (76 girls and 86 boys, 3-15 years old, mean age 10.5 +/- 2.8 years) who attend the Regional School Centre for Deaf Children in Katowice. The data in our analysis was obtained from case histories (school records and special questionnaires sent to parents), physical examinations with special regard to the cardiovascular system, double blood pressure measurement and 12 lead surface electrocardiogram. In the studied group, 90 children (55.5%) were congenitally deaf. Within this group, 24 children manifested tachycardia while 4 children manifested-bradycardia. A-V block of I degree was found in 3 children. Incomplete right bundle branch block (RBBB) was observed in 44 children while a complete RBBB was noticed in 1 child. QTc interval was prolonged (> 0.44s) in 12 children suffering from congenital form of deafness and in 16 children suffering from all causes of deafness. On the basis of case history, physical examination, and standard 12-lead electrocardiogram, we established the occurrence of risk factors according to the new diagnostic criteria of Schwartz et al. with relative points assigned to the electrocardiographical, clinical and familial findings. These points when summed up represent the risk of sudden death caused by ventricular arrhythmia due to long QT syndrome. In the studied group (according to the Schwartz criteria), 2 (1.2%) children had high probability (> or = 4 points) of long QT syndrome (LQTS), 25 (15.4%) children had intermediate probability of LQTS (2-3 points) and 135 children had low probability of LQTS (< or = 1 point).