Objective: To determine the prevalence of pulmonary hypertension (PH) in patients with cystic fibrosis (CF), to compare clinical characteristics, radiographic scores, pulmonary function, and echocardiographic parameters in patients with and without PH, and to correlate echocardiographic findings with clinical characteristics, radiographic scores, and pulmonary function. Methods: This was a prospective, cross-sectional study involving clinically stable patients (aged 16 or older) enrolled in an adult CF program. The patients were submitted to clinical evaluation, Doppler echocardiography, pulmonary function tests, and chest X-rays. Results: Tricuspid regurgitant jet velocity (TRV) was obtained in 37 of the 40 patients studied. The prevalence of PH was 49% with a TRV cut-off of 2.5 m/s (18 patients) and 30% with a TRV cut-off of 2.8 m/s (11 patients). Peripheral oxygen saturation (SpO2) at rest, clinical score, forced expiratory volume in one second (FEV1), and forced vital capacity (FVC) were significantly lower in the group with PH. The TRV was found to correlate significantly with SpO2 at rest (p < 0.001), clinical score (p < 0.001), radiographic score (p = 0.030), FEV1 in liters (p < 0.001) and in % of predicted (p < 0.001), and FCV in liters (p = 0.008) and in % of predicted (p = 0.001). The single best predictor of TRV was SpO2 at rest (p < 0.001). Conclusion: The high prevalence of PH in the CF patients studied suggests that PH should be considered in the evaluation and follow-up treatment of such patients. The best predictor of PH was SpO2 at rest.

Keywords: Cystic fibrosis; Tricuspid valve/physiopathology; Hypertension, pulmonary; Echocardiography, Doppler.