青少年肺动脉高压伴 II 型阿伯内特畸形:病例报告

Q3 Social Sciences
I. Miklashevich, E. A. Potrokhova, D. A. Morozov, Yu. S. Isaeva
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引用次数: 0

摘要

文章提供了一例肺动脉高压(PAH)伴有先天性肝外门腔分流术(CEPS)或阿伯内蒂畸形的病例,患者为一名 17 岁女性。CEPS长期未被确诊,幼年时表现为严重脑病。6 岁时并发 PAH,9 岁时并发肝局灶性结节增生。由于没有及时进行手术治疗,也没有对 CEPS 进行适当的治疗,导致 PAH 不断恶化。14 岁时才开始使用的 PAH 联合双重疗法效果不佳。尽管该患者在 17 岁时接受了三联疗法的联合治疗,其临床和功能可能有所改善,但该患者仍然符合不良事件高风险标准,包括死亡率。在开始使用三联疗法治疗 PAH 两个月后,由于未能达到可降低手术和围手术期风险的目标血液动力学特征,患者试图通过手术治疗 CEPS,结果导致患者在术后早期死亡。本病例报告的描述显示了诊断罕见形式的门静脉肺动脉高压的困难,以及在早期阶段对严重的、可能治愈的 PAH 进行药物治疗的现代可能性。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Pulmonary arterial hypertension associated with type II Abernethy malformation in an adolescent: a case report
The article provides a case of pulmonary arterial hypertension (PAH) associated with congenital extrahepatic portocaval shunt (CEPS), or Abernethy malformation, in a 17-year-old female patient. CEPS, which remained undiagnosed for a long time, manifested with severe encephalopathy at an early age. By the age of 6 years, it was complicated by PAH, and by the age of 9 years — by hepatic focal nodular hyperplasia. In the absence of timely surgical treatment and adequate therapy of CEPS, PAH progressed. Combined dual therapy for PAH, prescribed only at age 14, was ineffective. Despite the potential for clinical and functional improvement documented with combination triple therapy at 17 years of age, the patient continued to meet criteria for a high risk of adverse events, including mortality. Two months after the initiation of triple therapy for PAH, not achieving the target hemodynamic characteristics that would reduce operative and perioperative risks lead to an attempt of surgical treatment of CEPS, which led to the patient death in the early postoperative period. The description of this case report shows the difficulties of diagnosing a rare form of portopulmonary hypertension, the modern possibilities of drug therapy for a severe, potentially curable PAH in the early stages.
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来源期刊
Cardiovascular Therapy and Prevention
Cardiovascular Therapy and Prevention Medicine-Cardiology and Cardiovascular Medicine
CiteScore
1.70
自引率
0.00%
发文量
155
审稿时长
6-12 weeks
期刊介绍: The most important objectives of the journal are: the generalization of scientific and practical achievements in the field of cardiology, increasing scientific and practical skills of cardiologists. The scientific concept of publication does the publication of modern achievements in the field of epidemiology, prevention and treatment of cardiovascular diseases, the results of research, national and international clinical trials. For publication in the journal are invited both domestic and foreign scientists and clinicians working in the field of cardiology, as well as doctors of other specialties. The magazine covers various issues in cardiology and related specialties. Each issue is prepared by Executive editor of the issue, a respected specialist in the field of epidemiology, prevention and treatment of cardiovascular diseases. The main focus of the publication — scientific articles on original research, the pharmacotherapy of cardiovascular disease, new diagnostic methods. All members of the group of authors should meet all four criteria of authorship set forth in the ICMJE recommendations: 1) concept and design development or data analysis and interpretation, and 2) manuscript justification or verification of critical intellectual content, and 3) final approval for publication of the manuscript, and 4) consent to be responsible for all aspects of the work, and assume that issues relating to the thoroughness and diligent execution of any part of the study submitted are duly investigated and resolved. Great importance the editors attached to the preparation of scientific papers by groups of authors at a high level, literacy, authors, and their ownership information, availability of research results not only to colleagues in Russia, but also abroad.
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