Key result
Integrated echocardiography unmasks ccTGA previously misdiagnosed as hypertrophic cardiomyopathy.
Case Report (n=1)
No
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Highlights how congenital ventriculoarterial discordance with a hypertrophied systemic right ventricle can mimic hypertrophic cardiomyopathy in adulthood and present with accessory pathway-mediated incessant tachycardia amenable to radiofrequency ablation.
A 65-year-old woman with a history of diabetes and a prior diagnosis of hypertrophic cardiomyopathy was admitted to the Department of Cardiology for palpitations. Cardiac examination revealed a3/6 holosystolic murmur over theleft parasternal area. Echocardiography showed that the aorta extended from the morphological systemic ventricle (RV), which was identified by the three-leaflet tricuspidvalve (Fig. 1) that was inserted more apically than the mitral valve (Fig. 2).The pulmonary trunk, identified by its bifurcation, arose from the morphological “pulmonic” ventricle (LV) (Fig. 3). The RV was mildly dilated, the septum wall was 18 mm thick, and systolic RV function was mildlyreduced(ejection fraction 40%). There was moderate atrioventricular(tricuspid) valve regurgitation. The LV showed normal function and there was mild atrioventricular (mitral) valve regurgitation. There was mild aortic regurgitation but no pulmonary valvular stenosis. A persistent patency of oval-shaped foramen was found. The aorta was anterior and to the left of the pulmonary trunk, and the two vessels were side by side. Holter monitoring demonstrated incessant tachycardia with only brief periods of sinus rhythm. The patient was submitted to electrophysiological evaluation, and the accessory pathway was successfully ablated using radiofrequency pulses.
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Ieva et al. (2008) conducted a case report in Congenitally corrected transposition of the great arteries (CCTGA) initially misdiagnosed as hypertrophic cardiomyopathy (n=1). Integrated transthoracic and transesophageal echocardiography and radiofrequency catheter ablation was evaluated on Diagnosis of CCTGA and ablation of accessory pathway. Integrated transthoracic and transesophageal echocardiography identified congenitally corrected transposition of the great arteries (18 mm septum, RV EF 40%) in a 65-year-old woman previously misdiagnosed with hypertrophic cardiomyopathy.
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