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STUDY DESIGN A retrospective study. OBJECTIVE The aim was to evaluate the relationships of Cobb angle and pulmonary function tests (PFTs) changes in severe spinal deformity and underwent posterior vertebral column resection (PVCR). SUMMARY OF BACKGROUND DATA No previous study focused on the correlation of deformity correction and PFTs changes in patients with cobb angle >90 degrees. METHODS PFTs values [forced vital capacity (FVC), forced expiratory volume in 1 second (FEV1), and percent-predicted values FVC%, FEV1%] were evaluated preoperative and at 2 years after PVCR. FVC% <80% were defined as restrictive ventilation dysfunction (RVD), the severity of RVD were divided into mild (FEV1% ≥70%), moderate (70% > FEV1% ≥50%) and severe (FEV1% <50%). The relationships among PFTs values improvements and all possible impact factors (mainly correction cobb angle) collected in this study were analyzed. PFTs data were compared among the 3 RVD subgroups (mild vs. moderate vs. severe) and between residual >30 versus <30 degrees. RESULTS A total of 53 cases (28 male/25 female, mean ages 18.9 Y) underwent PVCR in one center from 2004 to 2016 were enrolled cobb angle. When 2 years after PVCR, average PFTs values showed significant improvements. PFTs values changes showed no correlation with correction rate and correction angle. The only significant impact factor in this study for FVC, FVC%, FEV1 improvements was preoperative FVC% and the only impact factor for FEV1% improvement was preoperative FEV1%, the relationships were negative. In accordance with the regression analysis, PFTs values improvements among the 3 RVD subgroups from high to low was severe>moderate>mild. However, patients with residual cobb angle <30 degrees had less PFTs values improvements than patients with residual cobb angle >30 degrees. CONCLUSIONS Two years after PVCR, PFTs values were significantly improved. There is no linear correlation between cobb angle change and PFTs values improvements. Lower preoperative FVC% and FEV1% indicate more PFTs values improvements at 2 years post-PVCR. LEVEL OF EVIDENCE Level III.