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Case Reports
. 2014 Dec;35(12):1510-2.

Ventricular tachycardia an atypical presentation of Ebstein's disease

Gabriel E Baztarrica  1 , Gastón G Sereno, Sebastían A Villecco, Rafael Porcile
Affiliations
Case Reports

Ventricular tachycardia an atypical presentation of Ebstein's disease

Gabriel E Baztarrica et al. Saudi Med J. 2014 Dec.

Abstract

Ebstein's anomaly (EA) is a rare congenital malformation, characterized by an apical displacement and dysplasia of the septal leaflet of the tricuspid valve with a right ventricular atrialization compromising its function. This malformation includes electrical conduction abnormalities, and very rarely ventricular arrhythmias. We report the case of a 22-year-old male, presenting with dizziness and palpitations. The electrocardiogram showed a sustained monomorphic ventricular tachycardia (SMVT). The presentation of the disease with ventricular tachycardia associated with hemodynamic instability is extremely rare. We placed a surgical implantable cardiac defibrillator when the diagnosis of EA, SMVT, and right ventricular systolic impairment was confirmed.

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Figures

Figure 1
Figure 1
Twelve lead electrocardiogram showing the sustained monomorphic ventricular tachycardia.
Figure 2
Figure 2
Images showing the A) transthoracic echocardiogram of the low implantation of the TV and RV atrialization was visualized in a 4-chamber view. B) Vectographic image using MR showed extensive RA dilatation (53 × 103 mm) with atrialization of the RV inflow tract and showed the TV with abnormal apical implantation of the septal leaflet (31 mm). MR - magnetic resonance, RA - right atrium, TV - tricuspid valve, LA - left atrium, RV - right ventricle, LV - left ventricle.
See this image and copyright information in PMC

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