Association of congenital, diffuse electrical disease in children with normal heart: sick sinus syndrome, intraventricular conduction block, and monomorphic ventricular tachycardia.

Iturralde-Torres, Pedro; Nava-Townsend, Santiago; Gómez-Flores, Jorge; Medeiros Domingo, Argelia; Colín-Lizalde, Luis; Hermosillo, Antonio G.; Victoria, Diana; Márquez, Manlio F. (2008). Association of congenital, diffuse electrical disease in children with normal heart: sick sinus syndrome, intraventricular conduction block, and monomorphic ventricular tachycardia. Journal of cardiovascular electrophysiology, 19(5), pp. 550-555. Wiley-Blackwell 10.1111/j.1540-8167.2007.01006.x

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INTRODUCTION Rhythm disturbances in children with structurally normal hearts are usually associated with abnormalities in cardiac ion channels. The phenotypic expression of these abnormalities ("channelopathies") includes: long and short QT syndromes, Brugada syndrome, congenital sick sinus syndrome, catecholaminergic polymorphic ventricular tachycardia, Lènegre-Lev disease, and/or different degrees of cardiac conduction disease. METHODS The study group consisted of three male patients with sick sinus syndrome, intraventricular conduction disease, and monomorphic sustained ventricular tachycardia. Clinical data and results of electrocardiography, Holter monitoring, electrophysiology, and echocardiography are described. RESULTS In all patients, the ECG during sinus rhythm showed right bundle branch block and long QT intervals. First-degree AV block was documented in two subjects, and J point elevation in one. A pacemaker was implanted in all cases due to symptomatic bradycardia (sick sinus syndrome). Atrial tachyarryhthmias were observed in two patients. The common characteristic ventricular arrhythmia was a monomorphic sustained ventricular tachycardia, inducible with ventricular stimulation and sensitive to lidocaine. In one patient, radiofrequency catheter ablation was successfully performed. No structural abnormalities were found in echocardiography in the study group. CONCLUSION Common clinical and ECG features suggest a common pathophysiology in this group of patients with congenital severe electrical disease.

Item Type:

Journal Article (Original Article)

Division/Institute:

04 Faculty of Medicine > Department of Cardiovascular Disorders (DHGE) > Clinic of Cardiology

UniBE Contributor:

Medeiros Domingo, Argelia

Subjects:

600 Technology > 610 Medicine & health

ISSN:

1045-3873

Publisher:

Wiley-Blackwell

Language:

English

Submitter:

Argelia Medeiros Domingo

Date Deposited:

18 Jun 2014 11:42

Last Modified:

18 Jun 2014 11:42

Publisher DOI:

10.1111/j.1540-8167.2007.01006.x

PubMed ID:

17971134

URI:

https://boris.unibe.ch/id/eprint/42285

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