REVEAL score best predictor of mortality in patients with pulmonary arterial hypertension

Survival rates for pulmonary arterial hypertension (PAH) in this REVEAL* study from a Southeast Asian population are similar with those from other national registries. Moreover, this study validates the REVEAL score as a good predictor of mortality.

A total of 148 patients were included (mean age 50.8±15.9 years; 77 percent female). Racial distribution was consistent with the population census.

Congenital heart disease-associated PAH (35.8 percent), idiopathic PAH (29.7 percent) and connective tissue disease-associated PAH (24.3 percent) were the most common aetiologies. Nearly half of the patients presented with World Health Organization Functional Class (FC) II (48.6 percent), followed by FC III (28.8 percent).

More than half of the patients (54.1 percent) were taking phosphodiesterase type 5 (PDE5) inhibitor monotherapy. Survival rate at the end of the first year was 85.8 percent, while those at 3, 5, 7 and 10 years were 70.9 percent, 66.9 percent, 61.5 percent and 55.4 percent, respectively.

The REVEAL score was the best predictor of mortality, with a score >6 identified as a cutoff. Other predictors were as follows: mean right atrial pressure, heart rate, aetiology, age and N-terminal probrain natriuretic peptide.

In this study, the authors enrolled patients with PAH referred to a pulmonary hypertension specialty centre from January 2003 to December 2016. They also analysed baseline characteristics and survival. A multilevel structural equation model was applied based on a forward predictor selection procedure to identify predictors associated with mortality.

“PAH is a rare and fatal disease. Data from Asia are lacking compared with the West,” the authors noted.

*Registry to Evaluate Early And Long‐term PAH Disease Management