To our knowledge, there are no specific and validated measures of quality of life (QoL) or degree of disability for pulmonary arterial hypertension (PAH). A review of the literature shows that, with the exception of one recently designed specifically for pulmonary hypertension, QoL questionnaires used in PAH studies are generic measures. These are selected because of shared symptoms that do not necessarily correlate well with functional or physiologic measures and have not been validated for applicability in PAH.
In this review, we present the available QoL tools for pulmonary artery hypertension and describe the need for more specific instruments that consider the physical and emotional implications of the diseases associated with PAH and the impact of various treatment options. We also discuss the impact of PAH on work ability and the need for provisions to address medical disability status and Social Security benefit status.