Persistent left superior vena cava coexisting with the absence of right superior vena cava is an uncommon anomaly, and sometimes requires permanent pacemaker implantation due to the relatively high incidence of conduction disturbances and arrhythmias. This anomaly makes the implantation of pacemaker leads more difficult; therefore, accurate preintervention diagnosis of this anomaly is valuable for the interventionalist. A patient in which the diagnosis of persistent left superior vena cava coexisting with absent right superior vena cava was made echocardiographically before permanent pacemaker implantation is presented. Acetylsalicylic acid was prescribed after pacemaker implantation to prevent a potentially fatal complication of coronary sinus thrombosis, and no complication occurred during the four-year follow-up. Some clues for the noninvasive diagnosis of this anomaly and techniques of pacemaker implantation are also described briefly.

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