A case report of upgrading to cardiac resynchronization therapy in a patient with congenitally corrected transposition of great arteries and dextrocardia.

Background: Congenitally corrected transposition of the great arteries (CCTGA) is a rare congenital heart anomaly. Physiological correction may be associated with a long pre-symptomatic period in many patients and delayed accidental diagnosis. Additional related congenital malformations may increase the complexity of cardiac interventions.

Case Summary: A 59-year-old man with known dextrocardia, situs viscerum inversus, and CCTGA was scheduled for upgrading of a dual-chamber pacemaker to cardiac resynchronization therapy to treat heart failure related to a progressive systolic dysfunction of the systemic right ventricle (RV). Because of the specific anatomy of this patient, the therapeutic procedure was complicated by the cannulation of the Marshall vein. Nevertheless, the left ventricular lead was successfully implanted into the coronary sinus lateral branch. At the 3-month follow-up, the patient remarkably reported a significant functional improvement, despite no favourable reverse remodelling of the systemic RV.

Discussion: Upgrade of a pacemaker to biventricular pacing was feasible in this patient, who had CCTGA and dextrocardia, which resulted in symptomatic improvement at follow-up. Pre-implant contrast cardiac computed tomography angiography was essential for visualizing the venous-specific anatomy in this patient, who suffered from congenital heart disease. Conduction system pacing represents a potential alternative for the patient to prevent or treat pacing-related heart failure.