Familial Primary Pulmonary Hypertension. Report of Two Siblings.
Open Access
- 1 January 1991
- journal article
- case report
- Published by Japanese Society of Internal Medicine in Japanese Journal of Medicine
- Vol. 30 (3) , 273-277
- https://doi.org/10.2169/internalmedicine1962.30.273
Abstract
Familial primary pulmonary hypertension was found in two siblings (sister and brother). The woman noted exertional dyspnea at the age of 28 yr. The younger brother noticed exertional dyspnea, cough with bloody sputum and pretibial edema at the age of 38 yr. We diagnosed them as primary pulmonary hypertension as based on hemodynamicand histopathological findings. Regardless of the treatment, both patients died of right-sided heart failure with a short time course. We examined their family members, but there were no other members with primary pulmonary hypertension.Keywords
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