Clinical Outcomes and Prognostic Factors in a Cohort of Adults With Cystic Fibrosis: A 7-Year Follow-Up Study.

BACKGROUND: Due to the heterogeneity of cystic fibrosis (CF), the longer survival observed in cohorts of adult subjects, and inter-population variations, there is a clear need to seek further information about clinical outcomes and prognostic factors in different cohorts of subjects with CF. Our objectives were to evaluate clinical outcomes and prognostic factors in a cohort of adult subjects with CF after a 7-y follow-up period and investigate longitudinal changes in clinical scores, spirometry, 6-min walk test performance, and pulmonary artery systolic pressure as assessed by Doppler echocardiography.METHODS: A cohort of clinically stable subjects (≥16 y old) who were enrolled in an adult CF program in 2004–2005 underwent clinical evaluation. Outcome was classified as good (survival) or poor (survival with lung transplantation or death). In 2011–2012, survivors were re-examined.RESULTS: Of 40 subjects with CF evaluated in 2004–2005, 32 (80%) survived, 2 (5%) survived with lung transplantation, and 6 (15%) died. Logistic regression analysis showed that a low percent-of-predicted FEV1 was associated with poor outcome. An FEV1 cut-off value of ≤30% and pulmonary artery systolic pressure of ≥42 mm Hg predicted poor outcome with high sensitivity, specificity, and positive and negative predictive values. Deterioration was observed in clinical scores ( P = .03), FVC ( P = .02), FEV1 ( P u003c .001), distance walked in the 6-min walk test ( P = .002), baseline SpO2 ( P u003c .001), and final SpO2 ( P u003c .001).CONCLUSIONS: After 7 y of follow-up, 20% of subjects with CF had a poor outcome. Pulmonary artery systolic pressure of ≥42 mm Hg and FEV1 of ≤30% were the most significant prognostic predictors of poor outcome. Clinical and functional deterioration was observed in survivors.