Distinct Forms of Pulmonary Hypertension Complicate Hereditary Hemorrhagic Telangiectasia

Pulmonary hypertension (PH) associated with hereditary hemorrhagic telangiectasia (HHT) occurs most commonly as a consequence of high-output heart failure related to excessive shunting through visceral vascular malformations. A smaller number of HHT patients develop a form of heritable pulmonary arterial hypertension, characterized by an elevated pulmonary vascular resistance with normal left heart filling pressures. In this review, we will discuss the clinical manifestations of HHT in general, the pathophysiology behind the different forms of PH associated with HHT, and the clinical care of the HHT patient with PH.