Purpose of review: This review discusses the current impact of pulmonary hypertension on the outcome and treatment of cystic fibrosis (CF).
Recent findings: Pulmonary hypertension is commonly encountered in advanced lung diseases such as CF. The prevalence of pulmonary hypertension in CF patients varies based on disease severity and methodology used for diagnosis. Chronic alveolar hypoxia is the most likely cause. The majority of recent studies have shown worse survival in CF patients who develop pulmonary hypertension. The impact of pulmonary hypertension-specific therapies on symptomatology and outcomes in CF patients has not been well studied.
Summary: Pulmonary hypertension is common in patients with CF and it occurs largely because of hypoxemia. The presence of pulmonary hypertension in patients with CF is likely associated with worse outcome; however, it remains unknown whether treatment with pulmonary hypertension-specific therapies would be beneficial.