BACKGROUND Superior vena cava syndrome (SVCS) is a rare complication of pacemaker implantation. Numerous methods have been employed to treat this condition, ranging from anticoagulation and thrombolysis to surgical interventions and stenting. However, thus far only small case series have been reported and there is no currently accepted standard of care. METHODS Our group preformed a PubMed literature search to identify cases of symptomatic SVCS that developed following implantation of permanent pacemakers or implanatable cardioverter defibrillators and were treated with one of five different modalities: anticoagulation, thrombolysis, venoplasty, stenting, and surgical reconstuction. Duration of follow-up and incidence of recurrence of symptoms were the main end-points. RESULTS One hundred and four eligible cases from 74 different publications were identified, in which SVCS presented at a median of 48 (range 0-396) months after device implantation. We found that over the last 40 years, conservative treatments have been replaced by surgical reconstruction, and most recently by stenting, as the most common therapeutic modality employed. Anticoagulation, thrombolysis, and venoplasty alone were all associated with high recurrence rates. Surgery and stenting were more successful: recurrence rates were 12% and 5% over a median follow-up of 16 (range: 2-179) and 9.5 (range: 2-60) months, respectively. CONCLUSIONS Currently, transvenous stenting is the most common treatment used for pacemaker-related SVCS, usually with conservation of the implanted leads. Both surgery and stenting appear to be effective treatments, with low incidences of recurrent SVCS over the first 12 months, but there is unfortunately a paucity of data on long-term outcomes. (PACE 2010; 420-425).