Abstract
Two sisters with severe pulmonary hypertension and Osler's disease are described. Heart disease also occurred in other family members affected with hereditary hemorrhagic telangiectasia. Multiple abnormalities of large systemic vessels, severe intrahepatic arteriovenous fistulas and systemic-pulmonary arterial anastomoses were demonstrated in one of the patients, and we conclude that analogous manifestations may have been present in her sister and presumably in other members of the family too. The patients are discussed in relation to similar cases reported earlier and to various types of pulmonary hypertension.
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