Purpose: Pulmonary thromboendarterectomy (PTE) is a treatment option for patients with chronic thromboembolic pulmonary hypertension (CTEPH). The present systematic review was performed to assess the safety and efficacy of PTE for CTEPH. Methods: A systematic review was performed, and six electronic databases were searched for published studies from January 1999 to February 2010. All articles that presented morbidity and mortality data, survival data or preoperative and postoperative pulmonary hemodynamic indices were included. The primary outcome measures extracted were early morbidity and mortality, pulmonary hemodynamic and functional outcome indices prior to and after the operation, and survival data. Results: Of the 654 publications retrieved, 19 relevant papers (total number of 2729 patients) representing the most recent and complete data set from each institute, were included for appraisal and data extraction. No randomized controlled trials or matched comparative studies were identified. Thirty-day mortality ranged from 1.3% to 24% (median 8%). Residual pulmonary hypertension was reported in 11%-35% of patients after PTE. Pulmonary artery pressure and pulmonary vascular resistance significantly decreased after PTE in all studies. Before PTE, 60%-100% of patients were in NYHA functional class III or IV. This percentage decreased to 0%-21% after PTE. Five-year survival ranged from 74% to 89%. Conclusions: The current literature suggests that PTE for patients with CTEPH is associated with acceptable perioperative morbidity and mortality rates and improved hemodynamic indices and survival when viewed against the prognosis associated with historical controls using medical therapy.