Severe Pulmonary Hypertension Associated With Emphysema : A New Phenotype?

Mild to moderate precapillary pulmonary hypertension (PH) is a common complication of COPD and has typically been related to severe airflow limitation associated with chronic hypoxemia. Previous studies focusing specifically on patients with emphysema found that worsening PH was associated with progression of airflow obstruction. In the present report, we describe a new phenotype of COPD with severe precapillary PH in patients presenting with progressive dyspnea, normal spirometry, severely reduced diffusion capacity of the lung for carbon monoxide, and high-resolution CT scans of the chest showing diffuse centrilobular emphysema.