Patients with PAH-COPD May be at Higher Risk than those with Idiopathic PAH

Transplant-free survival was found lower among patients with pulmonary hypertension-COPD than among those with IPAH in a new study published in CHEST.

Prognosis for patients with pulmonary hypertension who also have chronic obstructive pulmonary disease (PH-COPD) appears to be less favorable than for patients with idiopathic pulmonary arterial hypertension (IPAH), according to new research published online in the journal CHEST.

PH is frequently seen in advanced COPD, the authors write, affecting up to one-half of patients who are slated for volume reduction or lung replacement surgery. Several studies have demonstrated an independent prognostic role for PH in the COPD population. The authors also note, however, that while results of research on the effect of targeted PAH therapies in patients with PH-COPD have been “heterogenous,” these therapies are still used in this population.

The aim of the present study, led by Carmine Dario Vizza, MD, from the pulmonary hypertension unit in the department of cardiovascular and respiratory disease, Sapienza University of Rome, Rome, Italy, was multifold. The researchers set out 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
  • Describe patient response to PH-targeted therapy

The primary outcome was transplant-free survival and was compared using Kaplan-Meier estimates and the Breslow test.

Patient data were drawn from COMPERA — an ongoing, investigator-initiated, noninterventional, prospective European-based registry that enrolls patients with all forms of PH.

A total of 489 patients with IPAH, 307 with severe PH-COPD, and 68 with moderate PH-COPD were included.

Overall, the participants with a diagnosis of PH-COPD were predominantly men, older, and treated primarily with phosphodiesterase-5 inhibitors. Although patients with IPAH and PH-COPD demonstrated 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; =.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 30m in 6MWD or improvements in WHO FC following initiation of medical therapy were associated with better outcomes.

Their findings, the researchers write, demonstrate 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.

Citation: 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