What causes pulmonary hypertension in Osler-Weber-Rendu disease (OWRD) (hereditary hemorrhagic telangiectasia [HHT])?

Updated: Oct 06, 2020
  • Author: Klaus-Dieter Lessnau, MD, FCCP; Chief Editor: Vincent Lopez Rowe, MD  more...
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Answer

Despite shunt formation across the pulmonary tree from AVMs, HHT is also associated with pulmonary hypertension. Lung tissue of HHT patients with pulmonary hypertension appears histologically similar to that of patients with primary pulmonary hypertension. This manifestation of HHT is associated with ALK1 gene mutations. [60]


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