[Guided anti-arrhythmia therapy by programmed ventricular stimulation. A case of arrhythmogenic dysplasia of the right ventricle].
Right ventricle dysplasia (RVD) is a rare clinical entity, described in young people, and a known cause of ventricular arrhythmias and sudden death. A genetic transmission has been suggested from the study of familial cases. The disease is characterized by a dilated right ventricle, cardiac infiltration with fatty tissue and focal fibrosis. Lymphoplasmocyte infiltrates are documented. The diagnosis is based on electrocardiography, echocardiography, magnetic resonance imaging and right ventricular endomyocardial biopsy. The authors report a case of a young woman with symptomatic ventricular tachycardia (VT) in whom the diagnosis of RVD was established. Programmed ventricular stimulation was used to reproduce the clinical VT and to test the efficacy of the anti-arrhythmic drug therapy. The diagnosis criteria and therapeutic options are discussed.
Oliveira
M
M
Serviço de Cardiologia, Hospital de Santa Marta, Lisboa.
da Silva
N
N
Antunes
E
E
Pinto
E
E
Cotrim
C
C
Pitta
L
L
Cacela
D
D
Gracias
R
R
Antunes
A M
AM
D000328:Adult; D000889:Anti-Arrhythmia Agents / Q000627:therapeutic use; D001706:Biopsy; D002304:Cardiac Pacing, Artificial / Q000379:methods*; D009202:Cardiomyopathies / Q000150:complications / Q000175:diagnosis / Q000628:therapy*; D004699:Endocardium / Q000473:pathology; D005260:Female; D006352:Heart Ventricles / Q000002:abnormalities; D006801:Humans; D011405:Propafenone / Q000627:therapeutic use; D017180:Tachycardia, Ventricular / Q000175:diagnosis / Q000209:etiology / Q000628:therapy*; D018497:Ventricular Dysfunction, Right / Q000175:diagnosis / Q000209:etiology / Q000628:therapy*
D000889:Anti-Arrhythmia Agents; D011405:Propafenone