Rapid improvement in postpartum pulmonary hypertension associated with hereditary hemorrhagic telangiectasia: A case report and review of literature.

IF 1 4区医学 Q3 MEDICINE, GENERAL & INTERNAL

World Journal of Clinical Cases Pub Date : 2025-04-16 DOI:10.12998/wjcc.v13.i11.98128

Sheng-Yu Hao, Yaxiaerjiang Muhetaer, Xin Zheng, Yu-Liang Long, Jie-Qiong Song, Ming Zhong

{"title":"Rapid improvement in postpartum pulmonary hypertension associated with hereditary hemorrhagic telangiectasia: A case report and review of literature.","authors":"Sheng-Yu Hao, Yaxiaerjiang Muhetaer, Xin Zheng, Yu-Liang Long, Jie-Qiong Song, Ming Zhong","doi":"10.12998/wjcc.v13.i11.98128","DOIUrl":null,"url":null,"abstract":"Background: Postpartum pulmonary arterial hypertension (PAH) complicated with hereditary hemorrhagic telangiectasia (HHT) is a rare condition. Diagnosing and treating PAH in patients with HHT can be challenging. To the best of our knowledge, no previous reports have investigated the efficacy of pulmonary vasodilators in improving hemodynamics in postpartum patients with this disease.Case summary: In this paper, we report a postpartum case of HHT combined with PAH, presenting with worsening dyspnea. Genetic testing revealed that the patient carried a heterozygous variant of activin receptor-like kinase 1. The patient received various treatments, including diuretics, anticoagulants, sildenafil, macitentan, inhalation of nitric oxide, and iloprost. Changes in PaO2/FiO2, pulmonary artery systolic pressure as assessed by echocardiography, and N-terminus pro-brain natriuretic peptide levels suggested that, except for iloprost inhalation, the other treatments appeared to have limited efficacy.Conclusion: To our knowledge, this is the first report on efficacy of pulmonary vasodilators in postpartum patients with HHT and PAH.","PeriodicalId":23912,"journal":{"name":"World Journal of Clinical Cases","volume":"13 11","pages":"98128"},"PeriodicalIF":1.0000,"publicationDate":"2025-04-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11718578/pdf/","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"World Journal of Clinical Cases","FirstCategoryId":"3","ListUrlMain":"https://doi.org/10.12998/wjcc.v13.i11.98128","RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q3","JCRName":"MEDICINE, GENERAL & INTERNAL","Score":null,"Total":0}

引用次数: 0

Abstract

Background: Postpartum pulmonary arterial hypertension (PAH) complicated with hereditary hemorrhagic telangiectasia (HHT) is a rare condition. Diagnosing and treating PAH in patients with HHT can be challenging. To the best of our knowledge, no previous reports have investigated the efficacy of pulmonary vasodilators in improving hemodynamics in postpartum patients with this disease.

Case summary: In this paper, we report a postpartum case of HHT combined with PAH, presenting with worsening dyspnea. Genetic testing revealed that the patient carried a heterozygous variant of activin receptor-like kinase 1. The patient received various treatments, including diuretics, anticoagulants, sildenafil, macitentan, inhalation of nitric oxide, and iloprost. Changes in PaO₂/FiO₂, pulmonary artery systolic pressure as assessed by echocardiography, and N-terminus pro-brain natriuretic peptide levels suggested that, except for iloprost inhalation, the other treatments appeared to have limited efficacy.

Conclusion: To our knowledge, this is the first report on efficacy of pulmonary vasodilators in postpartum patients with HHT and PAH.

查看原文本刊更多论文

产后肺动脉高压合并遗传性出血性毛细血管扩张的快速改善：1例报告及文献复习。

背景：产后肺动脉高压（PAH）合并遗传性出血性毛细血管扩张（HHT）是一种罕见的疾病。诊断和治疗HHT患者的PAH可能具有挑战性。据我们所知，以前没有报道调查肺血管扩张剂改善产后此病患者血流动力学的疗效。病例总结：本文报告1例产后HHT合并PAH，表现为呼吸困难加重。基因检测显示患者携带激活素受体样激酶1的杂合变异。患者接受了多种治疗，包括利尿剂、抗凝剂、西地那非、马西坦、吸入一氧化氮和伊洛前列素。超声心动图评估的PaO2/FiO2、肺动脉收缩压和n端脑利钠肽前水平的变化表明，除吸入伊洛前列素外，其他治疗似乎疗效有限。结论：据我们所知，这是第一次报道肺血管扩张剂对产后HHT合并PAH患者的疗效。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

求助全文

约1分钟内获得全文求助全文

来源期刊

World Journal of Clinical Cases Medicine-General Medicine

自引率

0.00%

发文量

3384

期刊介绍： The World Journal of Clinical Cases (WJCC) is a high-quality, peer reviewed, open-access journal. The primary task of WJCC is to rapidly publish high-quality original articles, reviews, editorials, and case reports in the field of clinical cases. In order to promote productive academic communication, the peer review process for the WJCC is transparent; to this end, all published manuscripts are accompanied by the anonymized reviewers’ comments as well as the authors’ responses. The primary aims of the WJCC are to improve diagnostic, therapeutic and preventive modalities and the skills of clinicians and to guide clinical practice in clinical cases.