[Arrhythmogenic right ventricular dysplasia. Case report and review of the literature].
Two cases of arrhythmogenic right ventricular dysplasia are described. The most important clinical features of the disease are malignant ventricular arrhythmia and sudden death. It is characterized by the loss of right ventricular musculature and by the fatty and connective tissue infiltration of the right ventricular wall. The diagnosis is based on the typical echocardiographic appearance of right ventricular dilatation, on the presence of negative T waves in leads V1-4 on the resting ECG and on ventricular tachycardia of left bundle branch block pattern. Right heart failure develops only in the late phase of the disease. Genetic defect might be an etiologic factor. In conclusion authors suggest that in case of left bundle branch block ventricular tachycardia or Adams-Stokes syndrome in young adults echocardiography and family screening are necessary.
Kolonics
I
I
Országos Kardiológiai Intézet, Semmelweis OTE Kardiológiai Tanszék, Budapest.
György
M
M
Lengyel
M
M
Hegyi
L
L
D000293:Adolescent; D002037:Bundle-Branch Block / Q000150:complications / Q000175:diagnosis; D015150:Echocardiography, Doppler; D004562:Electrocardiography; D005500:Follow-Up Studies; D006801:Humans; D017380:Hypertrophy, Right Ventricular / Q000150:complications* / Q000175:diagnosis; D008297:Male; D008875:Middle Aged; D013612:Tachycardia, Ectopic Atrial / Q000175:diagnosis / Q000209:etiology*
Arrhythmogen jobb kamrai dysplasia. Esetismertetés és irodalmi áttekintés.