Copyright © 1994 by the European Society of Cardiology.
© 1994 The European Society of Cardiology
Supraventricular arrhythmia as the cause of sudden death in hypertrophic cardiomyopathy
Division of Cardiology Hospital de Navarra, 31008-Pamplona, Navarra, Spain
Received 20 April 1993; revised 5 August 1993; .
Correspondence: Irene Madanaga, MD. Servicio de Cardiologia, Hospital de Navarra, Irunlarrea 3, 31008 Pamplona, Navarra, Spain
Abstract
Electrophysiological studies with simultaneous echocardiographic control and invasive measurement of intravascular pressures were carried out in a 13-year-old boy with hypertrophic cardiomyopathy who was hospitalized after an episode of aborted sudden death.
Ventricular stimulation did not induce ventricular tachycardia, but atrial stimulation induced atrial fibrillation, atrial flutter and non-sustained ventricular tachycardia. Atrial stimulation (S1) at 200 beats. min1 (1015s) also induced significant repolarization abnormalities in the 510 post-stimulation beats. Akinesia of the ventricular septum and posterior wall without opening of the mitral valve was documented by echocardiography. A complete anterior systolic motion, not observed under basal conditions, was detected in the first post-stimulation beat. Atrial stimulation at rates over 120 beats. min1 caused a drop in systolic blood pressure, a rise in pulmonary artery pressure, and a decrease in cardiac output.
Despite therapy with propranolol and amiodarone, the patient died suddenly.
Key Words: Hypertrophic cardiomyopathy sudden death supraventricular arrhythmia electrophysiological studies echocardiography ventricular fibrillation
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