Primary pulmonary hypertension. a review
Savajiyani, R.; Benchimol, A.; Desser, K.B.
Arizona Medicine 38(12): 898-901
1981
ISSN/ISBN: 0004-1556 PMID: 7036957 Document Number: 5655
Primary pulmonary hypertension (PPH) is a disease of unidentified cause, characterized by precapillary pulmonary arteriopathy leading to pulmonary hypertension. Observed predominantly in women of child bearing age, PPH results in death within a few months to three years. By definition, all other causes of pulmonary hypertension must be excluded before making the diagnosis of PPH. The natural history of PPH is not clearly established as most patients come to the physician's attention only after the disease is far advanced. Most deaths are sudden and preceded by effort-syncope yet the exact mechanism of demise usually is not determined. A small number of deaths have followed cardiac catheterization, pulmonary angiograms, acute pulmonary embolism, pregnancy, surgery, anesthesia, dissection or rupture of the pulmonary artery and sedation.
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