The prevalence of pulmonary hypertension in patients with end stage cystic fibrosis

Introduction: Lung transplantation (LTx) is a widely accepted treatment for end stage cystic fibrosis (CF). The prevalence of secondary pulmonary hypertension (PH) in CF patients is estimated with ultrasound to be 21−49%. But the gold standard, heart catheterization, is used in few studies because of the invasive nature. The existence of PH increases the perioperative risk. Therefore, we measured mean pulmonary artery pressure by right heart catheterization, in CF patients screened for LTx. Methods and Materials: CF patients evaluated for LTx between 2001 and 2008 were included. We excluded patients with incomplete or conflicting data. Right heart catheterization was performed according to our protocol. PH was defined as mean pulmonary artery pressure greater than 25mmHg at rest with a pulmonary capillary wedge pressure of 15mmHg or less. The forced expiratory volume in one second was determined at moment of catheterization. Results: 73 CF patients were screened for LTx and 68 were included (of whom 93% accepted for LTx). Median age was 31 (range 16−57) years at moment of screening. During the study period, 35 patients were transplanted (56%) after a median waiting time of 10 months (range 0−49). 16 CF patients (24%) were diagnosed with PH by heart catheterization. No significant differences were observed between the groups with or without PH regarding to age, gender and number of enlisted patients. Lung function was significantly lower in the group with PH (p=0.003). No correlation between lung function and mean pulmonary artery pressure was found (p=0.09). Conclusions: This study demonstrated a prevalence of 24% for pulmonary hypertension in CF patients screened for LTx.