The updated hemodynamic definition of pulmonary hypertension (PH) associated to respiratory disease (PHRD) differentiates severe and non-severe phenotypes, but no further risk stratification strategy has been established or validated for group III PH. We aimed to assess the prognostic value of REVEAL Lite 2 scoring in patients with PHRD.
 
Methods: We retrospectively analyzed 72 patients (COPD 32% and EPID 68%) with PHRD (mean pulmonary artery pressure of >20 mm Hg and PVR > 2 UW) who were enrolled in the Canary PH Registry during 2010?2023. A REVEAL Lite 2 scoring was applied at baseline and follow up. Kaplan?Meier and univariate Cox regression analyses were used to evaluate transplant-free survival and hazard ratios, respectively.

Results: 56% male (70+12 years) had a mean pulmonary arterial pressure (PAPm) of 34±8 mmHg and a mean pulmonary vascular resistance (PVR) of 5.9 ± 2.3 UW. They were treated with specific therapy, 50% monotherapy. During follow up 47 events occurred. According to the REVEAL Lite 2 scorings at baseline, 20%, 24% and 56% were classified as low, intermediate, and high risk respectively. After 3-6 months of treatment, 23%, 32%, and 45% were classified as low, intermediate, and high risk, respectively. The 5-year survival of low, intermediate, and high risk at baseline was 87%, 56%, and 25%, respectively (log-rank p = 0.001; hazard ratio of high vs low-risk group: 3.541 [95% CI: 1.428?6.800])). The 5-year survival of low, intermediate, and high risk after treatment was 98%, 56%, and 28%, respectively (log-rank p = 0.03).

Conclusions: The REVEAl Lite 2 scheme was clinically useful and demonstrated prognostic relevance in patients with PH associated with respiratory diseases