Complications of liver vascular malformations in hereditary hemorrhagic telangiectasia during pregnancy

Hereditary hemorrhagic telangiectasia (HHT), also known as Osler-Weber-Rendu syndrome, is a dominantly inherited disorder of angiogenesis characterized by mucocutaneous telangiectases and visceral arteriovenous malformations. HHT is the most common cause of hepatic arteriovenous malformations (HAVMs) which can induce severe complications during pregnancy. We report a case series of five pregnancies in four women with HHT, where severe complications of HAVMs were recorded. Short-term maternal outcome was good. In the long-term, one woman underwent liver transplantation ten years after her last pregnancy. About fetal outcome, one post-partum infant death occurred. These cases, together with a review of the literature, underscore the importance of timely diagnosis and management of HAVMs in pregnant women with HHT and provide useful insights to optimize maternal and fetal outcomes.