Patient registries in pulmonary arterial hypertension: The role of survival equations and risk calculators

Abstract

Over the last 30 years, numerous patient registries in pulmonary arterial hypertension (PAH) have been conducted primarily in the United states and Western Europe. The registries provide extensive information about this rare and deadly disease. In addition, survival estimates and risk factors associated with mortality have been spotlighted. Several predictive equations have been generated from these large registries, such as the National Institutes of Health, French National, Pulmonary Hypertension Connection, and REVEAL. These equations have been designed to forecast survival of PAH cohorts and have been useful tools in clinical research. Adding to this experience, the REVEAL survival equation and risk calculator have been shown to reliably predict short-term survival of individual PAH patients. But, several factors, including regional differences in PAH sub-populations, access to care and a rapidly evolving therapeutic approach, limit the usefulness of these predictive equations in routine clinical practice.