Unilateral Absence of Pulmonary Artery Complicated With Severe Pulmonary Hypertension

Unilateral absence of pulmonary artery (UAPA) is a rare disorder that can present as an isolated lesion or in association with other congenital heart defects. An important complication of UAPA is the development of pulmonary hypertension (PH). We discuss an adult patient with severe PH in the setting of congenital UAPA and highlight the hemodynamic response to combination therapy that included parenteral treprostinil. To our knowledge, this is the first case report using parenteral treprostinil as part of triple combination therapy to treat PH in setting of UAPA. There are various treatment modalities for severe PH complicating isolated UAPA. This case highlights an additional option, parenteral treprostinil, for physicians to consider in highly selected cases.