Please use this identifier to cite or link to this item: https://www.um.edu.mt/library/oar/handle/123456789/3775
Title: Is arrhythmogenic right ventricular cardiomyopathy a paediatric problem too?
Authors: Turrini, P.
Basso, Cristina
Daliento, Luciano
Nava, Alessandra
Thiene, Gaetano
Keywords: Arrhythmogenic right ventricular dysplasia
Heart -- Ventricles
Magnetic resonance imaging
Syncope
Tachycardia
Congenital heart disease
Issue Date: 2001
Publisher: Images in Paediatric Cardiology
Citation: Images in Paediatric Cardiology. 2000, Vol.3(1), p. 18-37
Abstract: Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a heart muscle disease that is often familial, characterized by arrhythmias of right ventricular origin, due to transmural fatty or fibrofatty replacement of atrophic myocardium. ARVC is usually diagnosed in the clinical setting between 20 and 40 years of age. The disease is seldom recognised in infancy or under the age of 10, probably because the clinical expression of the disease is normally postponed to youth and adulthood. This review focuses its attention to the pediatric age, defined as the period of life raging from birth to 18 years. During this span of life, ARVC is not so rare as previously supposed and can be identified by applying the same diagnostic criteria proposed for the adult. Ventricular arrhythmias range from isolated ventricular arrhythmias to sustained ventricular tachycardia and fibrillation. Children and adolescents with ARVC must be carefully evaluated and followed-up especially when a family positive history is present, taking into account the high probability during this life-period that asymptomatic affected patients become symptomatic or that arrhythmias worsen during follow-up. The recent identification of the first defective gene opens new avenues for the early identification of affected subjects even when asymptomatic.
URI: https://www.um.edu.mt/library/oar//handle/123456789/3775
Appears in Collections:IPC, Volume 3, Issue 1
IPC, Volume 3, Issue 1

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