Case Report
Copyright ©The Author(s) 2021. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Clin Cases. Apr 16, 2021; 9(11): 2634-2640
Published online Apr 16, 2021. doi: 10.12998/wjcc.v9.i11.2634
Anesthetic management of a child with double outlet right ventricle and severe polycythemia: A case report
Ling-Can Tan, Wei-Yi Zhang, Yi-Ding Zuo, Hong-Yang Chen, Chun-Ling Jiang
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 by The 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.

Keywords: Cyanotic congenital heart diseases; Double outlet right ventricle; Modified Fontan surgery; Anesthesia; Erythrocytosis; Case report

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.