通过吸入曲普瑞替尼和肝移植缓解严重的肺门高压症

IF 0.9 4区 医学 Texas Heart Institute Journal Pub Date : 2024-03-14 DOI:10.14503/THIJ-23-8209
Alex J Chang, Choon Hwa Goh
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引用次数: 0

摘要

肺动脉高压是一种罕见的疾病,预后不良。及时处理对获得肝移植资格至关重要,而肝移植是一种潜在的治疗方案。本报告介绍了一例严重的门静脉肺动脉高压患者,该患者在接受了他达拉非、马西坦坦和吸入曲普瑞替尼的保守治疗方案后,病情得到缓解,最终成功进行了肝移植。移植后,肺动脉高压没有复发,患者也脱离了大部分肺动脉高压治疗。本病例报告首次提供了证据,证明吸入曲普瑞替尼可安全有效地替代持续静脉注射前列环素治疗门静脉肺动脉高压。
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Resolution of Severe Portopulmonary Hypertension With Inhaled Treprostinil and Liver Transplantation.

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.

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来源期刊
Texas Heart Institute Journal
Texas Heart Institute Journal CARDIAC & CARDIOVASCULAR SYSTEMS-
自引率
11.10%
发文量
131
期刊介绍: For more than 45 years, the Texas Heart Institute Journal has been published by the Texas Heart Institute as part of its medical education program. Our bimonthly peer-reviewed journal enjoys a global audience of physicians, scientists, and healthcare professionals who are contributing to the prevention, diagnosis, and treatment of cardiovascular disease. The Journal was printed under the name of Cardiovascular Diseases from 1974 through 1981 (ISSN 0093-3546). The name was changed to Texas Heart Institute Journal in 1982 and was printed through 2013 (ISSN 0730-2347). In 2014, the Journal moved to online-only publication. It is indexed by Index Medicus/MEDLINE and by other indexing and abstracting services worldwide. Our full archive is available at PubMed Central. The Journal invites authors to submit these article types for review: -Clinical Investigations- Laboratory Investigations- Reviews- Techniques- Coronary Anomalies- History of Medicine- Case Reports/Case Series (Submission Fee: $70.00 USD)- Images in Cardiovascular Medicine (Submission Fee: $35.00 USD)- Guest Editorials- Peabody’s Corner- Letters to the Editor
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