Gao M, Zhang WH, Zhang ZG, Yang N, Tong Q, Chen LP. Cardiac amyloidosis presenting as pulmonary arterial hypertension: A case report. World J Clin Cases 2023; 11(12): 2780-2787 [PMID: 37214585 DOI: 10.12998/wjcc.v11.i12.2780]
Corresponding Author of This Article
Ming Gao, MD, Clinical Assistant Professor (Honorary), Doctor, Department of Cardiology, The First Hospital of Jilin University, No. 1 Xinmin Street, Changchun 130021, Jilin Province, China. gmbeata@jlu.edu.cn
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/
World J Clin Cases. Apr 26, 2023; 11(12): 2780-2787 Published online Apr 26, 2023. doi: 10.12998/wjcc.v11.i12.2780
Cardiac amyloidosis presenting as pulmonary arterial hypertension: A case report
Ming Gao, Wei-Hua Zhang, Zhi-Guo Zhang, Na Yang, Qian Tong, Li-Ping Chen
Ming Gao, Wei-Hua Zhang, Zhi-Guo Zhang, Na Yang, Qian Tong, Department of Cardiology, The First Hospital of Jilin University, Changchun 130021, Jilin Province, China
Li-Ping Chen, Department of Echocardiography, Center of Cardiovascular Disease, The First Hospital of Jilin University, Changchun 130021, Jilin Province, China
Author contributions: Gao M completed the clinical data collection and the manuscript draft; Chen LP and Yang N improved later revision of the article; Zhang WH and Tong Q participated in the treatment decisions; Zhang ZG, Zhang WH and Tong Q revised the manuscript critically to ensure the authenticity and practicability; All authors read and approved the final manuscript.
Informed consent statement: All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee(s) and with the Helsinki Declaration (as revised in 2013). Written informed consent was obtained from the patient.
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 Gao, MD, Clinical Assistant Professor (Honorary), Doctor, Department of Cardiology, The First Hospital of Jilin University, No. 1 Xinmin Street, Changchun 130021, Jilin Province, China. gmbeata@jlu.edu.cn
Received: January 13, 2023 Peer-review started: January 13, 2023 First decision: February 8, 2023 Revised: February 22, 2023 Accepted: March 30, 2023 Article in press: March 30, 2023 Published online: April 26, 2023 Processing time: 102 Days and 10.5 Hours
Abstract
BACKGROUND
Pulmonary hypertension is a rare cardiopulmonary disease, with an insidious onset that usually worsens rapidly. Amyloid light chain (AL) amyloidosis is a rare systemic disease caused by extracellular deposition of pathologic, insoluble, and proteinaceous fibrils in organs and tissues; however, it is difficult to diagnose given its varied and nonspecific symptoms. To date, rare cases of amyloidosis with pulmonary hypertension have been reported. Of note, the optimal treatments for cardiac amyloidosis complicated with pulmonary hypertension remain unclear.
CASE SUMMARY
We report a case of a 51-year-old woman who presented with progressively worsening dyspnea. Transthoracic echocardiography indicated severe pulmonary hypertension. Twenty-seven months after first admission, the patient returned with symptoms of progressive heart failure. A myocardial tissue sample stained with Congo red was positive, and the patient was ultimately diagnosed with AL amyloidosis with cardiac involvement.
CONCLUSION
Although pulmonary hypertension may be idiopathic, it is frequently associated with other conditions. In rare cases, pulmonary hypertension can be a complication of AL amyloidosis, which should be seriously considered in any adult presenting with nonspecific signs or symptoms of cardiac distress.
Core Tip: Symptomatic pulmonary hypertension is only rarely described and, when present, is typically associated with progressive disease, such as elevated filling pressures secondary to cardiac amyloid. In this case, the patient initially presented with pulmonary hypertension, she was found, 2 years later, to have amyloid light chain (AL) amyloidosis with cardiac involvement. We highlight the diagnostic difficulties presented by pulmonary hypertension in a patient with AL amyloidosis, and illustrate the complicated progression of the disease, as well as the poor efficacy of current palliative medicine.
