Situs inversus totalis with congenitally corrected transposition of the great arteries: insights from cardiac MRI

INTRODUCTION: Situs inversus totalis with congenitally corrected transposition of the great arteries represents a relatively rare congenital condition. CASE DESCRIPTION: The current report describes the case of a 56 year old patient with an atrio-ventricular and ventricular-arterial discordance of the heart chambers without surgical correction, incidentally detected during hepatocellular carcinoma evaluation. The systemic venous blood arrived via the right atrium and a mitral valve in the morphologically left but pulmonary arterial ventricle that gave rise to a pulmonary trunk. The pulmonary venous blood passed the left atrium and the tricuspid valve into a morphologically right but systemic ventricle that gave rise to the aorta. DISCUSSION AND EVALUATION: The switched anatomy was incidentally detected on echocardiography. The patient was referred to cardiac magnetic resonance imaging (CMR) including flow measurements, volumetry and late enhancement. CMR results showed a mildly impaired function and the switched anatomy. During a follow-up period of 2 years the patient was suffering from only mild heart failure and dyspnea. CONCLUSIONS: Heart failure symptoms and arrhythmias can appear with increasing age in patients with congenitally corrected transposition. Early CMR allows accurate diagnosis and timely introduction of adequate therapy thereby avoiding disease progression.