Prognostic significance of pulmonary hypertension in patients with cystic fibrosis: A systematic review and meta-analysis

BACKGROUND AND OBJECTIVE: Pulmonary hypertension (PH) is frequently found in advanced parenchymal lung diseases like cystic fibrosis (CF), but the role played by PH in the clinical outcome of CF patients remains unclear. The aim of this study is to determine the influence of PH on survival in the CF population by meta-analysis. METHODS: Publications addressing the associations between PH and overall survival (OS) or other clinical characteristics in CF patients were selected from electronic databases. Odds ratios (ORs) or mean differences (MDs) were used to estimate the association between PH and the clinical characteristics. The hazard ratios (HRs) with 95% confidence interval (CI) were abstracted or calculated to evaluate the association between PH and CF survival outcome. Subgroup analyses were also conducted. RESULTS: Seven studies including 2141 CF patients who met the inclusion criteria were included in our meta-analysis. With respect to clinical features, PH was significantly associated with lower PaO(2) (P < .001), higher PaCO(2) (P = .02), lower forced expiratory volume in 1 second percent (P < .001) and lower forced vital capacity percent (P < .001). However, PH had no significant impact on CF patients’ OS (HR = 1.29, 95% CI 0.81 to 2.06, P = .283). Furthermore, subgroup analyses also showed no evidence of prognostic role of PH in CF patients (all P values >.05). CONCLUSIONS: Our findings suggest that the presence of PH was strongly correlated with worse blood-gas parameters and worse lung function, but surprisingly had no significant prognostic value on survival among CF patients. Further large-scale and prospective studies are needed to confirm these findings.