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Retrospective cohort study.The purpose of this study is to evaluate pulmonary function and quality of life (QOL) in children treated with early spinal fusion for progressive congenital scoliosis.The relationships between radiographic measures, pulmonary function tests (PFT), and QOL were examined.Twenty-one patients with congenital scoliosis treated with early spinal fusion were evaluated using radiographs, PFT, and the Child Health Questionnaire (CHQ) at 12.6 ± 3.5 years. They were 6.9 ± 2.3 years postdefinitive fusion, which occurred at 4.9 ± 3.1 year of age. The cohort was also divided in two groups, “thoracic fusion” (apex above the thoracolumbar T–L junction) and “nonthoracic fusion” (below T–L junction).Forced vital capacity, forced expiratory volume (FEV1), vital capacity (P < 0.0001), and total lung capacity (P = 0.017) were significantly lower compared with healthy children. CHQ scores were significantly lower than in healthy children in physical function (P = 0.036), general health (P = 0.007), physical summary (P = 0.026), and parental impact/emotional (P = 0.01). Correlation analysis showed that the degrees of thoracic curves were negatively correlated with FEV1 (P < 0.05), family activities, role/social physical (P < 0.05), and physical summary (P < 0.01). The degree of kyphosis was negatively correlated with Self-Esteem (P < 0.01). Patients who had thoracic fusions had shorter spinal height (P = 0.049), lower forced vital capacity (P = 0.004), FEV (P = 0.012), vital capacity (P = 0.031), and reported more pain (P = 0.033) than nonthoracic fused.Compared with healthy peers, congenital scoliosis patients treated with early spinal fusion have worse PFT and QOL scores at 6.9 years follow up. Patients with thoracic fusions had shorter spines, worse pulmonary function, and more pain than nonthoracic fused. The results may support alternatives to early spinal fusion such as growing rods, epiphysiodesis, and distraction thoracoplasty. Current efforts are underway to compare outcomes of this study to those of other treatments.