Key result
Historical review traces primary pulmonary hypertension evolution from clinical pathology to standardized etiologic classifications.
Why the study?
Reports on primary pulmonary hypertension were limited to clinical pathological correlations until epidemiologic studies emerged following an aminorex fumarate epidemic.
Clinical review details disease progression and therapy in primary pulmonary arterial hypertension, highlighting the need for prompt hemodynamic evaluation and targeted vascular treatment.
For the first half of the twentieth century, the published reports on primary pulmonary hypertension (PPH) were confined to clinical pathological correlations. In the 1950s the physiological aspects began to be reported followed by epidemiologic studies triggered by an epidemic of PPH due to the ingestion of an appetite suppressant, aminorex fumarate. The epidemic prompted a series of meetings of the World Health Organization, which led, in turn, to the creation of the U.S. Registry of Primary Pulmonary Hypertension, standardization of nomenclature, and an etiologic classification of all types of pulmonary hypertension.
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Alfred P. Fishman (2004) conducted a review in Primary pulmonary arterial hypertension. A historical review of primary pulmonary hypertension highlights its evolution from clinical pathological correlations to the establishment of registries and standardized etiologic classifications.
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