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Ventricular arrhythmias in the young: clinical and experimental findings

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Book cover Ventricular Tachycardias

Part of the book series: Developments in Cardiovascular Medicine ((DICM,volume 71))

Abstract

Adults and children have similarities as well as differences in their ventricular arrhythmias. The most similar are the definition of the arrhythmias; although the rate of ventricular tachycardia in an infant may be as high as 500/minute with a QRS duration of 40 msec. Also similar are the techniques of intracardiac electrophysiology study. However, there are major differences in the etiology, prognosis and treatment of ventricular arrhythmias in children. There are four major areas of difference. 1) Idiopathic incessant ventricular tachycardia occurs in nfants less than the age of three years. It does not respond to conventional investigational antiarrhythmic agents. The majority of patients have Purkinje cell tumors causing the tachycardia. These tumors cannot be detected by echocardiography or angiography. Electrophysiologically directed surgery is curative. 2) In children with ventricular tachycardia who are thought to have an otherwise normal heart, the heart is usually not ‘normal’. When anatomic and hemodynamic cardiac catheterization is performed in these patients, in 70% either arrhythmogenic right ventricle or congestive cardiomyopathy is found. If the heart is truly normal, the prognosis is excellent. On the other hand, children with arrhythmogenic right ventricle or congestive cardiomyopathy, especially those who have exercise-related ventricular tachycardia, may die suddenly. 3) The idiopathic long QT syndrome occurs more commonly in children than adults. Sudden death may occur without preceding symptoms and treatment is recommended for even the asymptomatic child who has the diagnosis of prolonged QT syndome (in addition to the long QT interval, the diagnosis of the syndrome requires either a positive family history, bradycardia, deafness, or typical T wave morphology). 4) Patients who have had repair of congenital heart disease die suddenly due to ventricular arrhythmias. Treatment of these arrhythmias based on results of the Holter have resulted in a significant decrease in morality. The drugs that are successful in the treatment of arrhythmias in these patients (phenytoin, mexiletine) are different from the traditionally effective drugs in adults with ischemic heart disease. This observation has led to the hypothesis that the cellular mechanism of the initiating ventricular arrhythmias in patients after repair of congenital heart disease (triggered activity) may be different from the mechanism in those with ischemic heart disease (reentry). This hypothesis is extended to other patients with the common findings of ventriculr hypertrophy, cellular disarray and fibrosis such as aortic stenosis/aortic insufficiency, hypertrophic cardiomyopathy and systemic arterial hypertension. This hypothesis applies to all patients regardless of their age, perphaps pointing out a major similarity in arrhythmia mechanism. Therefore, the differences between children and adults may be less than is immediately apparent.

Dr. Garson is an Established Investigator of the American Heart Association, Dallas, Texas

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© 1987 Martinus Nijhoff Publishers, Dordrecht

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Garson, A. (1987). Ventricular arrhythmias in the young: clinical and experimental findings. In: Aliot, E., Lazzara, R. (eds) Ventricular Tachycardias. Developments in Cardiovascular Medicine, vol 71. Springer, Dordrecht. https://doi.org/10.1007/978-94-009-3323-1_10

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  • DOI: https://doi.org/10.1007/978-94-009-3323-1_10

  • Publisher Name: Springer, Dordrecht

  • Print ISBN: 978-94-010-7992-1

  • Online ISBN: 978-94-009-3323-1

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