ABSTRACT
Background: Situs inversus totalis (SIT) is a rare condition, where all the organs in the body are mirrored. Atrial fibrillation occurs in patients with SIT. We describe the case of pulmonary vein isolation (PVI) in SIT. Case summary: A patient with atrial fibrillation was referred to our hospital due to palpitations. Diagnosis of atrial fibrillation was made by electrocardiogram. The patient reported to have a SIT that was confirmed. Meticulous preparation was done including a three-dimensional model and radiofrequency PVI was performed successfully. No recurrence of atrial fibrillation was detected until last follow-up 2 years after PVI. Discussion: Pulmonary vein isolation in SIT can be performed successfully and with excellent long-term result.