Evolving Guidelines for PAH Treatment

Professional guidelines say that when pulmonary arterial hypertension (PAH) is diagnosed, right heart catheterization should be performed, but a quarter of the time, it isn’t—so investigators set out to discover why.

Most patients with pulmonary arterial hypertension (PAH) suffered from poor sleep quality, and dyspnea was a significant predictor of sleep quality, independent of disease severity, according to this study.

When patients were re-classified based on the adjusted thresholds of N-terminal pro-brain natriuretic peptide and 6-minute walk distance and the inclusion of TAPSE rather than right atria area, classification of patients improved significantly.

The study findings underscore the importance of early initiation of macitentan and tadalafil among patients who have pulmonary arterial hypertension (PAH), and represent a shift in understanding of prognosis based on diagnosis timing.

The comparative effectiveness study found that the addition of selexipag (Uptravi) to double oral therapy (DOT) reduced the risk of patients being hospitalized or having their disease progress.

Using Mendelian randomization analysis, researchers found evidence that 2 small groups of immunophenotypes are linked to either greater or lesser odds of developing pulmonary arterial hypertension (PAH).