Pulmonary hypertension is a mild comorbidity in end-stage cystic fibrosis patients.

Background: This study investigated the prevalence of pulmonary hypertension (PH) in cystic fibrosis (CF) patients awaiting lung transplantation (LTx) and its influence on survival. We also explored the feasibility of using echocardiography as a first assessment for diagnosing PH.

Methods: The study included 93 CF patients (46 women [50%]) evaluated for LTx between 2001 and 2010.