Unusual presentation of congenitally corrected transposition of great arteries (CCTGAs) in a middle aged male

Abstract

Congenitally corrected transposition of great arteries (CCTGAs) is a rare congenital heart disease. The literature review reveals that asymptomatic CCTGA is even rarer in elderly male. We report a case of a 54-year-old male, with no past significant medical history, who presented with angina type of chest pain. On examination, he had bradycardia, lower limb hypertension and grade 3 pansystolic murmur in the mitral area. Investigations revealed complete heart block in electrocardiograph, chest radiograph showed cardiomegaly, transthoracic echocardiography showed an apically located systemic atrio-ventricular (AV) valve, parallel arrangement of the great arteries, AV and ventriculoarterial discordance, hypertrophied right ventricle and significant systemic AV valve insufficiency with ejection fraction of 40%. These findings were consistent with congenitally corrected transposition of the great arteries with systemic AV valve insufficiency. Holter monitoring showed complete heart block, no significant pauses and one event of ill-sustained ventricular tachycardia (ventricular triplet) with the patient being asymptomatic during the study period. Need of internal cardioverter-defibrillator or pacemaker was deferred. The remaining hospital stay was uneventful. At the time of discharge, the patient was stable.