An article titled “The Genesis and Legacy of the NHLBI Patient Registry for the Characterization of Primary Pulmonary Hypertension”, published recently in Pulmonary Circulation, traces the historical development of primary pulmonary hypertension (PPH) research from its first pathological description by Ernest Romberg in 1891 through the establishment of the landmark US National Heart, Lung, and Blood Institute (NHLBI) Patient Registry.
The condition remained rare and poorly understood until the late 1960s, when Swiss physicians Hans Peter Gurtner and colleagues observed a dramatic 20-fold increase in primary pulmonary hypertension cases. This epidemic was traced to the appetite suppressant aminorex fumarate, which had been marketed not only in Switzerland but also in Austria and the Federal Republic of Germany, where similar increases in primary pulmonary hypertension incidence were documented. Remarkably, 76% of primary pulmonary hypertension patients in these countries during 1967-1973 had taken aminorex, and when the drug was withdrawn in 1968, primary pulmonary hypertension rates returned to baseline levels by 1973.
The aminorex crisis prompted the World Health Organization to convene a meeting in Geneva in 1973 to coordinate international research efforts on primary pulmonary hypertension. This meeting, attended by 22 experts, established the foundation for systematic study of the disease. The World Health Organization meeting directly led to the creation of the National Heart, Lung, and Blood Institute Patient Registry for the Characterization of Primary Pulmonary Hypertension, which operated from 1981-1985.
This Registry represented a groundbreaking collaborative effort involving 32 medical centers that enrolled 187 carefully screened patients. A unique feature was the pathology core led by Giuseppe Pietra, which standardized the histopathological analysis of lung specimens – despite the high risk of lung biopsy procedures. The registry’s findings, published in the Annals of Internal Medicine in 1987 and 1991, established the clinical and hemodynamic criteria that became the foundation for all subsequent primary pulmonary hypertension therapeutic development and continue to serve as comparators for modern pulmonary hypertension treatments.
Read more at this link on Pulmonary Circulation
See also our article on the history of pulmonary hypertension at this link and Prof. Adam Torbicki’s review of key milestones here
Citation
Palevsky, H. I. (2025). The Genesis and Legacy of the NHLBI Patient Registry for the Characterization of Primary Pulmonary Hypertension (NIH-PPH Registry). Pulmonary Circulation, 15(3), e70148. https://doi.org/10.1002/pul2.70148
