Department of Medicine, Kaiser Permanente San Francisco Medical Center, San Francisco, California.
Department of Cardiology, Kaiser Permanente San Francisco Medical Center, San Francisco, California.
Tex Heart Inst J. 2024 Mar 14;51(1). doi: 10.14503/THIJ-23-8209.
Portopulmonary hypertension is a rare condition with a poor prognosis. Prompt management is essential for liver transplantation eligibility, a potentially curative option. This report presents a case of severe portopulmonary hypertension that resolved with a conservative therapeutic regimen of tadalafil, macitentan, and inhaled treprostinil, which ultimately enabled successful liver transplantation. There was no recurrence of pulmonary hypertension after transplantation, and the patient was weaned off most pulmonary arterial hypertension therapies. This case report is the first to provide evidence that inhaled treprostinil is a safe and effective alternative to continuous intravenous prostacyclins in portopulmonary hypertension.
门脉高压性肺高血压是一种预后不良的罕见疾病。及时治疗对于肝移植资格至关重要，肝移植是一种潜在的治愈选择。本报告介绍了一例严重的门脉高压性肺高血压病例，该病例通过保守治疗方案（他达拉非、马西替坦和吸入用曲前列尼尔）得到缓解，最终成功进行了肝移植。移植后没有出现肺高血压复发的情况，且患者停用了大多数肺动脉高压治疗药物。本病例报告首次提供了证据，证明吸入用曲前列尼尔是门脉高压性肺高血压中替代持续静脉内前列环素的安全有效选择。
