Epidemiology of pulmonary hypertension: From quaternary referral centre to the community

Abstract

Pulmonary arterial hypertension (PAH) in its classic form is a rare progressive disease affecting predominantly young women. The first clinical classification system for pulmonary hypertension (PH) was put forth in Evian, France in 1998 in order to categorize alternate forms of pulmonary vascular disease, including PH as the result of chronic heart, lung, and systemic conditions. Over the past several decades, even the classic idiopathic (previously primary) PAH description has evolved as we have come to understand epidemiology in the modern treatment era. In all forms, PH and PAH cause significant morbidity and mortality from progressive right heart failure. A number of observational studies are now available, spanning PH population-based cohorts and PAH center-based registries. Reviewing the epidemiology of all subtypes of PH may elucidate commonalities along the spectrum of pulmonary vascular disease and identify critical areas for future study.