© 2005 The European Society of Cardiology. Published by Elsevier Ltd. All rights reserved.
IMAGES IN ELECTROPHYSIOLOGY/PACING
Multiple exercise induced syncopal episodes in a young woman due to arrhythmogenic right ventricular dysplasia
Ziekenhuis Oost-Limburg Genk, Cardiology Schiepse Bos 6, 3600 Genk, Belgium
Manuscript submitted 25 May 2004. Accepted after revision 22 September 2004.
*Corresponding author. Tel.: +32 89 327 100. Email address: wmulle0{at}hotmail.com (W. Mullens).
Abstract
A case of a young woman with multiple exercise induced syncopal episodes due to arrhythmogenic right ventricular dysplasia is described. The report emphasizes the importance of exercise induced syncope and the management is described.
Key Words: arrhythmogenic right ventricular dysplasia (ARVD), AICD, ventricular tachycardia, syncope
Case report
An 18-year-old woman was referred for multiple exercise induced syncopal episodes occurring since the age of 16. She had been playing competitive tennis (twice/week) since the age of 8. Physical examination was within normal limits. An electrocardiogram at rest showed sinus rhythm with inverted T-waves in V1–V4 and ventricular extrasystoles with a left bundle branch block pattern (LBBB). Multiple polymorphic non-sustained episodes of ventricular tachycadia (nsVT) were recorded on 24-hour Holter and on a bicycle exercise test nsVT with LBBB pattern occurred at 140 Watts (Figs. 1–Fig. 5).
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Transthoracic echocardiographic examination documented a dilated right ventricular cavity with extensive regional wall thinning and regional hypokinesia consistent with arrhythmogenic right ventricular dysplasia (ARVD). There was no involvement of the left ventricle. She was treated with sotalol and participation in competitive sports was stopped. An automatic defibrillator was implanted because of syncope during limited exercise three weeks later. One month after implantation an appropriate shock was delivered because of the occurrence of VT during limited exercise.
The patchy replacement of the RV myocardium by fatty and fibrous tissue provides a substrate for reentrant ventricular arrhythmias. Approximately 50 percent of patients with ARVD present with symptomatic ventricular arrhythmias manifest by palpitations, dizziness, and/or syncope. Patients with recurrent syncope at a young age, extensive right ventricular involvement, VT unresponsive to medical therapy, polymorphic VT with sympathetic stimulation, ECG abnormalities and survivors of sudden death should be considered for defibrillator implantation because of an increased risk of sudden cardiac death [1,
2]. If this can be prevented life expectancy may be normal [3].
This case report underlies the importance of cardiac investigation in young persons with syncope during exercise.
References
[1] Corrado D, Leoni L, Link MS, et al. Implantable cardioverter-defibrillator therapy for prevention of sudden death in patients with arrhythmogenic right ventricular cardiomyopathy/dysplasia. Circulation 2003; 108: 3084–3091.
[2] Daliento L, Turrini P, Nava A, et al. Arrhythmogenic right ventricular cardiomyopathy in young versus adult patients: similarities and differences. J Am Coll Cardiol 1995; 25: 655–664.[Abstract]
[3] Corrado D, Basso C, Thiene G, et al. Spectrum of clinicopathologic manifestations of arrhythmogenic right ventricular cardiomyopathy/dysplasia: a multicenter study. J Am Coll Cardiol 1997; 30: 1512–1520.[Abstract]
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