Published online Apr 16, 2021. doi: 10.12998/wjcc.v9.i11.2634
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
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.
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.
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.