Tan LC, Zhang WY, Zuo YD, Chen HY, Jiang CL. Anesthetic management of a child with double outlet right ventricle and severe polycythemia: A case report. World J Clin Cases 2021; 9(11): 2634-2640 [PMID: 33889630 DOI: 10.12998/wjcc.v9.i11.2634]
Corresponding Author of This Article
Chun-Ling Jiang, MD, Director, Doctor, Professor, Department of Anesthesiology, West China Hospital, Sichuan University and The Research Units of West China (2018RU012), Chinese Academy of Medical Sciences, No. 37 Guoxue Street, Wuhou District, Chengdu 610041, Sichuan Province, China. jiangchunling@scu.edu.cn
Research Domain of This Article
Anesthesiology
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/
Ling-Can Tan, Wei-Yi Zhang, Yi-Ding Zuo, Hong-Yang Chen, Chun-Ling Jiang, Department of Anesthesiology, West China Hospital, Sichuan University and The Research Units of West China (2018RU012), Chinese Academy of Medical Sciences, Chengdu 610041, Sichuan Province, China
Author contributions: Tan LC and Zhang WY collected the medical records of the patient; Chen HY and Zuo YD took responsibility for investigation and data curation; Tan LC, Zhang WY, and Jiang CL drafted and revised the manuscript.
Supported byThe 1.3.5. Project for Disciplines of Excellence, No. 2018HXFH046; West China Hospital, Sichuan University and the National Natural Science Foundation of China, No. 81971806.
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 authors declare that they have no conflicts of interest to disclose.
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: http://creativecommons.org/Licenses/by-nc/4.0/
Corresponding author: Chun-Ling Jiang, MD, Director, Doctor, Professor, Department of Anesthesiology, West China Hospital, Sichuan University and The Research Units of West China (2018RU012), Chinese Academy of Medical Sciences, No. 37 Guoxue Street, Wuhou District, Chengdu 610041, Sichuan Province, China. jiangchunling@scu.edu.cn
Received: December 7, 2020 Peer-review started: December 7, 2020 First decision: December 28, 2020 Revised: January 13, 2021 Accepted: February 9, 2021 Article in press: February 9, 2021 Published online: April 16, 2021 Processing time: 115 Days and 21 Hours
Abstract
BACKGROUND
Double outlet right ventricle (DORV) is a rare and complex congenital heart defect, and the surgical repairs vary with type and pathophysiology consequences. Due to prolonged progressive hypoxemia, severe polycythemia is common in patients with DORV, which ultimately leads to coagulation dysfunction and increases the risk of thrombosis and infarction. Consequently, the anesthetic management is challenging and how to manage severe polycythemia and avoid hypoxia-related complications in such patients is of great significance.
CASE SUMMARY
Herein, we report the anesthetic management of a 10-year-old female patient with a DORV. She lived in the low-oxygen Qinghai-Tibet Plateau, and presented with severe polycythemia (hemoglobin, 24.8 g/dL; hematocrit, 75%). She underwent a modified Fontan surgery, which was satisfactory and without any perioperative complications. Our anesthetic management highlights the importance of perioperative hemodilution in decreasing the risk of thromboembolism and the importance of correcting coagulopathy in preventing hemorrhage.
CONCLUSION
Anesthetic management is challenging in rare cyanotic congenital heart disease patients with severe polycythemia. It is important to adopt perioperative hemodilution and correction of coagulopathy in preventing thrombosis and hemorrhage.
Core Tip: We present the successful anesthetic management of a double outlet right ventricle (DORV) patient with severe polycythemia. Anesthetic management is challenging in rare cyanotic congenital heart disease patients with severe polycythemia. A thorough understanding of the physiopathology of DORV and polycythemia is essential for successful anesthesia.
