[Pulmonary arterial hypertension in patients with hereditary hemorrhagic telangiectasia (Osler-Rendu disease)]. / Legochnaia arterial'naia gipertenziia u bol'nykh nasledstvennoi gemorragicheskoi teleangiektaziei (bolezn'iu Randiu-Oslera).

Pulmonary arterial pressure was studied using a noninvasive method in 23 patients with hereditary hemorrhagic telangiectasia (HHT) without clinical and x-ray signs of arterial-venous shunting in the lungs. It was established that pulmonary arterial hypertension was typical of HHT patients. A significant decrease in the volume and frequency of hemorrhages in 12 of 17 HHT patients was combined with a statistically significant decrease in pulmonary arterial pressure as a result of HBO therapy. It was assumed that pulmonary microcirculatory disorders played an important role in HHT pathogenesis and were expressed in metabolic derangement of a number of biologically active substances in the lungs resulting in the development of telangiectasia.