Report of an atypical case with arrhythmogenic right ventricular dysplasia or Uhls anomaly

Luis M Morales Pérez; Omar R González Greck; Ana M Jerez Castro; Eliézer San Román García; Aníbal González Trujillo

2012, Number 4

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CorSalud 2012; 4 (4)

Report of an atypical case with arrhythmogenic right ventricular dysplasia or Uhl's anomaly

Morales PLM, González GOR, Jerez CAM, San Román GE, González TA

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Language: Spanish
References: 30
Page: 300-306
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ABSTRACT

Arrhythmogenic right ventricular dysplasia is a cardiomyopathy characterized bymalignant ventricular arrhythmias and progressive structural abnormalities, affecting primarily the right ventricle. It appears due to a partial or massive progressive replacement of the myocardium by fibroadipose or adipose tissue. Uhl's disease may be an extreme and widespread manifestation of arrhythmogenic right ventricular dysplasia, a rare congenital disorder with absence of right ventricular myocardium, so that its walls are paper thin. The case of a 56 year old male patient who had loss of consciousness and underwent clinical and echocardiographic diagnosis is presented. The clinical features, diagnosis and action to take against this potentially fatal heart disease in patients with syncope, ventricular tachycardia or cardiac arrest are discussed.

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