Dextrocardia with corrected transposition in an adult - sequential segmental analysis by transthoracic echocardiography and cardiac CT

Corrected transposition alongwith dextrocardia and situs inversus is a very rare entity. The hallmark of corrected transposition is discordance at atrio-ventricular and ventriculo-arterial level and that is defined as “double discordance”. This can occur as an isolated anomaly but more commonly has associated defects; most common being ventricular septal defect followed by tricuspid valve abnormalities. Other associated defects are pulmonary stenosis, systemic and pulmonary venous anomalies, univentricular physiology, ventricular dysfunction (morphological right ventricle facing systemic circulation) and association of conduction abnormalities. 

Dextrocardia with situs inversus, also known as mirror-image dextrocardia, accounts for nearly 40% of all dextrocardia cases and is characterized by heart chambers located exactly opposite to their normal positions. Congenitally corrected transposition of great arteries (CCTGA) may co-occur in nearly 8% of dextrocardia with situs inversus.

Echocardiography and Cardiac CT play a pivotal role in defining the anatomy of such a complex association and planning the management.We report a case of a 29-year-old male who presented to our hospital with dyspnea on effort [New York Heart Association (NYHA) Functional Classification class II]. Transthoracic echocardiographic study disclosed dextrocardia, situs inversus, CCTGA, pulmonary valvular stenosis with left sided aortic arch. Cardiac CT additionally detected atrial septal defect (ASD) and sub-pulmonary stenosis.