Pulmonary Hypertension in a Patient with Hereditary Hemorrhagic Telangiectasia
t Hereditary Hemorrhagic Telangiectasia (HHT), also known as Osler-Weber-Rendu Disease, is an autosomal dominant genetic disorder that is characterized by the abnormal development of blood vessels. While the pathophysiology underlying the development of pulmonary hypertension (PH) in patients with HHT is not fully understood, it is believed to occur by one of two mechanisms: increases in pulmonary vascular resistance or cardiac output. In the following report, we describe an interesting case of a 26-year-old woman with HHT whose right heart catheterization initially demonstrated PH with elements of both pre- and post-capillary PH. Once the pre-capillary PH component was treated, however, an underlying high-normal cardiac-output state was unmasked. [Full article available at http://rimed.org/rimedicaljournal-2017-08.asp].