Prevalence, risk factors and prognosis of pulmonary hypertension in cystic fibrosis

Objectives: To determine the prevalence, determinants and prognostic value of pulmonary acceleration time (PAT) to assess pulmonary hypertension (PHT) in cystic fibrosis (CF). Methods: Prospective multicenter longitudinal study of CF patients with forced expiratory volume in one second (FEV1) ≤60% predicted evaluated during their yearly check up with echocardiography, spirometry, and nocturnal oximetry. Results: Sixty-seven consecutive patients were included (mean age 17±10 years and mean FEV1 42±12% predicted). Eight patients received a lung transplant during a mean follow-up of 19±6 months. Systolic pulmonary artery pressure (sPap) was measurable using TR peak velocity in 50 (75%) patients, with 10 having values above 35 mmHg. PAT determined in all patients correlated negatively with sPAP (r=0.36, p=0.01). Patients in the lowest PAT tertile ( 35 mmHg. By multivariate analysis, FEV 1 and the nocturnal desaturation index were the main determinants of low PAT. Conclusion: PAT less than 101 ms is a useful prognostic indicator in patients with CF whatever the age and is determined by FEV1 and nocturnal oxygen desaturation.