Primary pulmonary hypertension in adults

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Wood1 first defined primary pulmonary hypertension as a clinical entity in 1950, but considerable controversy exists as to the natural history, etiology, and pathogenesis of the disease. Primary pulmonary hypertension is a progressive disease more commonly seen in women, with death occurring 2 to 10 years after diagnosis. The diagnosis is often one of exclusion. Numerous case reports and small clinical series have been published,2–6 but there is no large series that describes the clinical and detailed angiographic findings in these patients with long-term follow-up. The purpose of our paper is to report a large clinical series (38 patients) with emphasis on clinical, hemodynamic, and angiographic findings. All but one of our patients were contacted to determine follow-up status. To our knowledge, this is the largest clinical series reported with long-term follow-up.


Primary pulmonary hypertension was defined as an elevation in mean pulmonary artery pressure greater than 30 mm Hg with exclusion of known secondary causes of pulmonary hypertension. Thirty-eight cases of primary pulmonary hypertension were seen at the Cleveland Clinic from 1962 to 1978. The diagnosis was established by cardiac catheterization in 37 patients and in one patient was confirmed at autopsy. Written or telephone communication or both on the clinical course and treatment was obtained from referring physicians, the patients, or relatives of the patients. Electrocardiograms were available on all patients. Echocardiograms were performed on 13 of the 38 patients. Eight patients had detailed evaluation for collagen disease, and lung immunofluorescence studies were done on one patient . . .



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