Ventricular septal defect with bidirectional shunting in a patient with congenitally corrected transposition

Abstract

Congenitally corrected transposition of the great arteries (CCTGA) is a rare cardiac malformation characterized by the combination of discordant atrioventricular and ventriculoarterial connections. Most of the cases with CCTGA are diagnosed in childhood because of concomitant cardiac malformation. Relevant concomitant cardiac defects such as ventricular septal defect (VSD), atrial septal defect, tricuspid regurgitation and pulmonary stenosis were reported previously. We report an asymptomatic patient with CCTGA and coexisting VSD with bidirectional shunting. A 22-year-old asymptomatic male in the army was seen in our department during his periodical examination. He had a grade 3/6 mesocardiac systolic murmur on cardiac auscultation. Electrocardiogram (ECG) showed normal sinus rhythm with right bundle branch block. Transthoracic echocardiography showed CCTGA with VSD with left to right shunt (Fig.1, Video 1. See corresponding video/movie images at www.anakarder.com) and moderate tricuspid and aortic regurgitation in apical four-chamber view. The pulmonary valve was moderately stenotic with a peak pressure gradient of 49 mm Hg. For identifying the direction of shunt flow in VSD contrast echocardiographic examination with agitated saline was carried out. Contrast echocardiography demonstrated positive contrast effect in the left ventricular in diastole confirming a right-to-left shunt at the ventricular septum (Video 2. See corresponding video/movie images at www.anakarder.com). According to our knowledge, our case is the first reported CCTGA with VSD with bidirectional shunting in an asymptomatic patient.