C65-04 Single-center Pilot: Does Reveal 2.0 Fit Methamphetamine-pulmonary Arterial Hypertension

Abstract Rationale REVEAL 2.0—derived from a large U.S. multicenter PAH (pulmonary arterial hypertension) cohort (∼3,500 patients, 55 centers)—is widely used to stratify risk and guide therapy. Methamphetamine-associated PAH (meth-PAH) is increasingly being recognized in the United States, yet drug/toxin–associated PAH comprised only ∼10% of REVEAL and methamphetamine exposure was not separately characterized, leaving meth-PAH under-represented. Notably, Pulmonary Hypertension Association Registry data suggests that meth-PAH presents with more adverse hemodynamics, and case series have reported poorer overall outcomes. Accordingly, whether REVEAL is applicable and clinically useful for meth-PAH—particularly for treatment selection and prediction of early mortality—remains uncertain. Methods We performed a single-center retrospective review of meth-PAH patients from 2012–2023. Consistent with REVEAL, the date of diagnosis was the diagnostic right heart catheterization (RHC). Inputs (6-minute walk distance, pulmonary function tests, echocardiography, BNP/NT-pro-BNP, and routine labs) were abstracted from ±90 days of RH; if multiple values were available, we used the closest (ties: worst value). Expected 1-year deaths were calculated as the sum across strata of (Known at 1 year × REVEAL 1-year risk: Low 1.9%, Intermediate 6.5%, High 25.8%). We report standardized mortality ratios (SMR = Observed/Expected) with exact Poisson 95% CIs (for O = 0, upper ≈ 3/E) and two-sided exact p-values. Two patients had BNP measured outside ±90 days (∼5 months) and were retained in the primary analysis; a sensitivity analysis excluded these cases. Results Sixteen patients met criteria (Low 5, Intermediate 4, High 7). Observed 1-year deaths were 0, whereas REVEAL predicted 2.161 deaths in total (Low 0.095, Intermediate 0.260, High 1.806) -i.e., we did not observe the deaths expected by REVEAL. Overall SMR was 0.00 (95% CI, 0.00–1.39; p = 0.230). In a sensitivity analysis excluding two cases with BNP obtained 90 days from RHC, totals and inferences were unchanged (E = 2.161; O = 0; SMR=0.00; 95% CI, 0.00–1.39; p = 0.230). Among patients who died (n = 7), deaths occurred at High: 16.2, 28.3, 74.0 months; Intermediate: 48.0 months; Low: 48.7, 81.1, 134.7 months after RHC—indicating events accrued beyond the first year in all groups. Conclusions In this pilot meth-PAH cohort, no deaths occurred within 1 year despite 2.161 expected by REVEAL (SMR 0.00; 95% CI, 0.00–1.39), indicating no signal of excess early mortality and lower-than-expected early risk. However, precision is limited by sample size, and the estimate is not statistically different from expected. These hypothesis generating findings support multi-center accrual to determine whether REVEAL 2.0 risk stratification can be applied to meth-PAH. This abstract is funded by: none