Benefits of Targeted Pharmacologic Therapy in PAH and Distal CTEPH

In patients with pulmonary arterial hypertension (PAH) or distal chronic thromboembolic pulmonary hypertension (CTEPH), the use of pharmacologic treatment was associated with a stabilization or improvement in risk factor profiles and an increased percentage of achieved therapeutic goals, according to the results of a real-life cohort study conducted at University Hospital Zurich in Switzerland and published in Respiration.

The investigators sought to evaluate whether the risk factor goals are attained and combination therapy is used in patients with PAH or distal CTEPH. Achievement of therapeutic goals was defined as New York Heart Association (NYHA) class ≤II, N-terminal pro-brain natriuretic peptide (NT-proBNP) <300 ng/L, and 6-minute walking distance (6MWD) >440 meters.

A total of 108 patients with PAH (age, 59±18 years; 62% women) and 38 patients with distal CTEPH (age, 69±14 years; 55% women) were included in the study. Participants had been diagnosed with PAH/distal CTEPH an average of 66±48 months prior to being recruited for the study.

Of the patients with PAH who attained therapeutic goals, 52% achieved NYHA class ≤II, 61% achieved NT-proBNP <300 ng/L, and 63% achieved 6MWD >440 meters. The percentages of patients with distal CTEPH who attained therapeutic goals were as follows: 53% achieved NYHA class ≤II, 52% achieved NTproBNP <300 ng/L, and 58% achieved 6MWD >440 meters.

Overall, 33% of participants with PAH and 31% of participants with distal CTEPH fulfilled three of the therapeutic goals; 29% of participants with PAH and 35% of patients with distal CTEPH fulfilled two of the therapeutic goals. With respect to therapy, 43% of patients with PAH were receiving double combination therapy and 10% were receiving triple combination therapy. In contrast, 16% of patients with distal CTEPH were receiving double combination therapy and 3% were receiving triple combination therapy.

The investigators concluded that in patients with PAH or distal CTEPH, the use of targeted pharmacologic therapy was associated with achievement of therapeutic goals. Patients with PAH are more likely to receive double combination therapy whereas patients with distal CTEPH are treated primarily with a single agent. It remains to be determined whether more aggressive combination treatment in this population would further improve goals in patients at lower risk.

Reference

Bartenstein P, Saxer S, Appenzeller P, Lichtblau M, Schwarz EI, Ulrich S. Risk factor profiles achieved with medical therapy in prevalent patients with pulmonary arterial and distal chronic thromboembolic pulmonary hypertension [published online April 11, 2018]. Respiration.
doi:10.1159/000488000