Kuzel Timothy G, El-Kersh Karim
University of Arizona Internal Medicine Residency Program, Phoenix, Arizona, USA.
University of Arizona College of Medicine, Phoenix, Arizona, USA.
JACC Case Rep. 2025 Aug 6;30(22):104549. doi: 10.1016/j.jaccas.2025.104549.
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.
TAKE-HOME MESSAGES: 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.
单侧肺动脉缺如（UAPA）是一种罕见疾病，可表现为孤立性病变或与其他先天性心脏缺陷相关。UAPA的一个重要并发症是肺动脉高压（PH）的发生。
我们讨论了一名患有先天性UAPA并伴有严重PH的成年患者，并强调了包括静脉注射曲前列尼尔在内的联合治疗的血流动力学反应。
据我们所知，这是首例将静脉注射曲前列尼尔作为三联联合治疗的一部分用于治疗UAPA相关PH的病例报告。
对于孤立性UAPA合并的严重PH有多种治疗方式。本病例突出了另一种选择——静脉注射曲前列尼尔，供医生在经过严格筛选的病例中考虑。
