Summary
Pulmonary arterial hypertension (PAH) is characterised by increased pressure in the
pulmonary arteries leading to right-sided ventricular failure, and death. Identification
of factors that affect patient survival is important to improve patient management
and outcomes. The first registry to evaluate survival and develop a prognostic model
was the National Institutes of Health (NIH) registry in 1981. Importantly this prognostic
model is based on data collected prior to availability of PAH-targeted therapies and
does not reflect survival rates for treated patients. Since the 1980s, however, four
modern registries of PAH now exist which compensate for the NIH equations shortcomings
and include the French National registry, Pulmonary Hypertension Connection registry,
the Mayo registry, and the Registry to Evaluate Early and Long-Term PAH Disease Management
(REVEAL). The similarities and difference in these registries are highlighted in this
review and although similar in many respects, the four registries vary in patient
population, including the numbers of newly and previously diagnosed patients, as well
as the era of observation, period of survival, and timing of assessment of potential
predictive factors. Despite this, the predictive factors identified in each registry
and described in detail within the body of this manuscript share surprising homology
in that disease aetiology, patient gender and factors reflective of right heart failure
are integral in depicting survival. Future modifications of modern prognostic equations
should be an ongoing goal of the PAH community in order to provide increased accuracy
with identification of novel risk factors and prediction of disease course.
Keywords
Prognosis - pulmonary arterial hypertension - REVEAL - risk calculator - survival
prediction
