REVEAL Registry Risk Score 2.0 for Pulmonary Arterial Hypertension (PAH)
Predicts survival in patients with pulmonary arterial hypertension.
Use in patients with recently diagnosed (<3 months ago) WHO Group 1 pulmonary arterial hypertension (PAH).
Management
- Patients with PAH WHO Group 1 should be considered for referral to a comprehensive care center for PAH.
- A multidisciplinary approach should be taken for patients with elevated scores (score >7), including evaluation by a pulmonary hypertension specialist and lung transplant team, along with nutritional assessments, social support, rehabilitation evaluation, and palliative care referral.
- 2022 ESC/ERC Guidelines recommend that REVEAL scores >10 are appropriate for lung transplant listing if they are undergoing therapy consistent with standard of care.
- Depending on goals of care and treatment plan, these patients should also be considered for additional clinical evaluation with a six minute walk test and right heart catheterization.
Critical Actions
- Hemodynamically unstable or acute right ventricular heart failure with known pulmonary hypertension patients should immediately be hospitalized; consider intensive care monitoring.
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It remains unknown whether a risk-based, goal-oriented treatment strategy is superior to the standard treatment algorithm recommended in the 2015 ESC/ERS guidelines, which are based on NYHA functional class alone.
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Risk stratification remains an imperfect science and although REVEAL has respective advantages, it is only a complementary tool for prognostication and guide treatment decisions.