Pulmonary Hypertension-COPD May Have Poorer Prognosis vs Idiopathic PAH

Pulm Hypertension Angiograph
Pulm Hypertension Angiograph
Patients with pulmonary hypertension and COPD appear to have a poorer prognosis than those with idiopathic pulmonary arterial hypertension.

Patients with pulmonary hypertension and chronic obstructive pulmonary disease (PH-COPD) appear to have a poorer prognosis than those with idiopathic pulmonary arterial hypertension (IPAH). In the current study, researchers analyzed data from COMPERA — an ongoing, investigator-initiated, noninterventional, prospective European-based registry that enrolls patients with all forms of PH (ClinicalTrials.gov Identifier: NCT01347216). Results of this analysis were published in the journal CHEST.

Investigators sought to describe the clinical characteristics and outcomes among a large population of patients with PH-COPD who were treated with targeted therapy to compare outcomes of these individuals with outcomes in patients with IPAH; explore factors predictive of survival in patients with PH-COPD; compare patients with moderate and severe PH-COPD, based on the latest recommendations from the 6th World Symposium on Pulmonary Hypertension; and describe patient response to PH-targeted therapy. The primary study outcome was transplant-free survival. Patients with more than 5 years of follow-up were censored after 60 months. Survival was established by patient visits to the center or, alternatively, by telephone calls to the patients, their relatives, or their local physicians.

A total of 489 patients with IPAH, 307 with severe PH-COPD, and 68 with moderate PH-COPD were included. Overall, the participants with PH-COPD were predominantly men, older, and treated primarily with phosphodiesterase-5 inhibitors. Although patients with IPAH and PH-COPD had similar hemodynamic impairment, those with PH-COPD had worse 6-minute walking distance (6MWD) and more advanced World Health Organization functional class (WHO FC).  

Results showed that transplant-free survival rates at 1, 3, and 5 years were significantly higher in the IPAH group than in the PH-COPD arm (94%, 74%, and 57%, respectively, in the IPAH group vs 86%, 55%, and 38%, respectively, in the PH-COPD group; P =.001). Risk factors for poor outcomes among participants with PH-COPD included male sex, low 6MWD, and high pulmonary vascular resistance. In participants with severe PH-COPD, improvements of at least 30 m in 6MWD or improvements in WHO FC following initiation of medical therapy were associated with better outcomes.

The researchers concluded that these findings demonstrated that patients with PH-COPD exhibited more functional impairment than those with IPAH. These results suggest that some patients with PH-COPD may benefit from the treatment of their PH. Prospective, randomized, controlled clinical studies are warranted to explore this hypothesis further.

Disclosure: COMPERA is supported by Acceleron, Actelion Pharmaceuticals, Bayer, OMT, and GSK. Please see the original reference for a full list of authors’ disclosures.


Vizza CD, Hoeper MM, Huscher D, et al. Pulmonary hypertension in patients with chronic obstructive lung disease: results from COMPERA. CHEST. Published online February 10, 2021. doi:10.1016/j.chest.2021.02.012