Hao SY, Muhetaer Y, Zheng X, Long YL, Song JQ, Zhong M. Rapid improvement in postpartum pulmonary hypertension associated with hereditary hemorrhagic telangiectasia: A case report and review of literature. World J Clin Cases 2025; 13(11): 98128 [DOI: 10.12998/wjcc.v13.i11.98128]
Corresponding Author of This Article
Ming Zhong, PhD, Department of Critical Care Medicine, Zhongshan Hospital Affiliated to Fudan University, No. 180 Fenglin Road, Shanghai 200032, China. zhongming2022@163.com
Research Domain of This Article
Cardiac & Cardiovascular Systems
Article-Type of This Article
Case Report
Open-Access Policy of This Article
This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/
Sheng-Yu Hao, Yaxiaerjiang Muhetaer, Xin Zheng, Jie-Qiong Song, Ming Zhong, Department of Critical Care Medicine, Zhongshan Hospital Affiliated to Fudan University, Shanghai 200032, China
Yu-Liang Long, Department of Cardiology, Zhongshan Hospital Affiliated to Fudan University, Shanghai 200032, China
Co-first authors: Sheng-Yu Hao and Yaxiaerjiang Muhetaer.
Co-corresponding authors: Jie-Qiong Song and Ming Zhong.
Author contributions: Hao SY, Muhetaer Y, and Zheng X collected the patient’s information, summarized the literature data, and wrote the manuscript; Hao SY and Muhetaer Y contributed equally to this article, they are the co-first authors of this manuscript; Long YL followed up with the patient and collected the patient’s information; Song JQ and Zhong M were the major contributors in revising the manuscript, they contributed equally to this article, they are the co-corresponding authors of this manuscript; and all authors have read and approved the final manuscript.
Supported by the Shanghai Sailing Program, No. 21YF1440300 and No. 22YF1407700; and the National Natural Science Foundation of China, No. 82200061.
Informed consent statement: Informed written consent was obtained from the patient for publication of this report and any accompanying images.
Conflict-of-interest statement: All the authors report no relevant conflicts of interest for this article.
CARE Checklist (2016) statement: The authors have read the CARE Checklist (2016), and the manuscript was prepared and revised according to the CARE Checklist (2016).
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: https://creativecommons.org/Licenses/by-nc/4.0/
Corresponding author: Ming Zhong, PhD, Department of Critical Care Medicine, Zhongshan Hospital Affiliated to Fudan University, No. 180 Fenglin Road, Shanghai 200032, China. zhongming2022@163.com
Received: June 18, 2024 Revised: November 18, 2024 Accepted: December 10, 2024 Published online: April 16, 2025 Processing time: 190 Days and 18.4 Hours
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.
Core Tip: This case report examines pulmonary vasodilators in a postpartum patient with hereditary hemorrhagic telangiectasia and pulmonary arterial hypertension carrying an activin receptor-like kinase 1 mutation. Despite treatments with diuretics, sildenafil, and nitric oxide, the efficacy was limited. Inhaled iloprost, however, rapidly relieved symptoms and improved hemodynamics. The study underscores the importance of genotype in treatment responses and highlights the need for personalized management of hereditary hemorrhagic telangiectasia-related pulmonary arterial hypertension, especially during pregnancy and postpartum. Further research is needed to explore underlying mechanisms and drug interactions.
