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Arrhythmogenic right ventricular cardiomyopathy in young versus adult patients: similarities and differences.

Abstract：

OBJECTIVES:This study was designed to evaluate and compare the patterns of arrhythmogenic right ventricular cardiomyopathy in young people and adults. BACKGROUND:Few data are available on this cardiomyopathy in young people because clinical and morphologic findings considered pathognomonic are normally based on observations in adults. However, a familial occurrence with a probable genetic transmission led to the study of children and to early detection of this disease, in which sudden death has been reported even in young people. METHODS:Seventeen young patients with arrhythmogenic right ventricular cardiomyopathy diagnosed at a mean age +/- SD of 14.9 +/- 4.9 years were studied. Clinical, electrocardiographic, echocardiographic, cineangiographic and biopsy findings were compared with those of 19 adult patients whose condition was diagnosed at a mean age of 38.1 +/- 13.4 years. RESULTS:Syncope occurred in 23.5% of the young patients but in none of the adults (odds ratio of familial sudden death 5.54, p = 0.1). Ventricular couplets (odds ratio 16.0, p = 0.004) and subtricuspid bulging on echocardiography (odds ratio 5.95, p = 0.036) were prevalent in the young group. Cineangiographic data in the two groups were similar, except that more hypokinetic areas were found in adults (odds ratio 4.44, p = 0.05). Morphometric analysis of biopsy sections showed a greater amount of fibrous tissue in the young patients (p = 0.04) and a prevalence of fatty tissue in the adults (odds ratio 12, p = 0.005). During an equivalent follow-up time (mean 7 years), two young patients died suddenly, and two had ventricular fibrillation in the absence of antiarrhythmic therapy. CONCLUSIONS:The pathognomonic criteria for the diagnosis of arrhythmogenic right ventricular cardiomyopathy in adults are also valid for young people. Sudden or aborted death occurred frequently in young untreated patients.

journal_name

J Am Coll Cardiol

journal_title

Journal of the American College of Cardiology

authors

Daliento L,Turrini P,Nava A,Rizzoli G,Angelini A,Buja G,Scognamiglio R,Thiene G

doi

10.1016/0735-1097(94)00433-Q

subject

Has Abstract

pub_date

1995-03-01 00:00:00

pages

655-64

issue

eissn

0735-1097

issn

1558-3597

pii

0735-1097(94)00433-Q

journal_volume

pub_type

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Arrhythmogenic right ventricular cardiomyopathy in young versus adult patients: similarities and differences.