Assessment of disease severity
NYHA functional class at baseline or after initiation of epoprostenol treatment, signs of right heart failure, 6-MWD, peak VO2, echocardiographic parameters, hemodynamic parameters and biological tests (hyperuricemia, brain natriuretic peptide, troponin) predict prognosis in idiopathic PAH when assessed at baseline.
Patients presenting PVOD or PAH associated with CTD (frequently associated with venous involvement) have a worse prognosis than patients with idiopathic PAH [53]. Patients with PAH associated with congenital systemic to pulmonary shunts have a more slowly progressive course than idiopathic PAH patients. Few data are available in other conditions such as HIV infection or portal hypertension. In these circumstances, underlying diseases may contribute to the overall outcome. In clinical practice, the prognostic value of a single variable in the individual patient may be less significant than the value of multiple concordant variables.
A score has been proposed (REVEAL Registry Risk Score) to evaluate severity of newly diagnosed PAH patients [196]. This score was based on several parameters including subgroups of PAH, renal insufficiency, age > 60 years, NYHA FC, systolic blood pressure, heart rate, 6-MWD, BNP, pericardial effusions, DLCO, RAP and PVR [196].