The Genesis and Legacy of the NHLBI Patient Registry for the Characterization of Primary Pulmonary Hypertension (NIH-PPH Registry)
https://doi.org/10.1002/pul2.70148
Abstract
The pathology of primary pulmonary hypertension was first described by Ernest Romberg in 1891 when he referred to histopathologic findings as pulmonary arterial sclerosis [1]. In the 1920s, Ayerza's Disease was described in a series of patients from Argentina with the proposed etiology being syphilis; this was subsequently disproven [2].
Following the development of right heart catheterization in the 1940s, David Dresdale and colleagues reported hemodynamic data on individuals with unexplained right heart failure and in 1951 coined the descriptor “primary pulmonary hypertension (PPH)” [3]. They and Paul Wood further described the clinical characteristics of the condition in the 1950s. PPH was found to be a rare condition, affecting primarily younger women, with an estimated incidence of 1 to 2 cases per million population per year in industrialized countries.