Anesthetic Management of a Child From the Qinghai-tibet Plateau With a Double Outlet Right Ventricle and Severe Polycythemia: A Case Report

Abstract

Background Acquired secondary polycythemia is common in patients with a double outlet right ventricle, and results from tissue hypoxia. Secondary erythrocytosis can cause coagulation deficiencies, and increases the risks of thrombosis and infarction. Perioperative management in double outlet right ventricle patients with severe erythrocytosis is intractable, and only a few cases have been reported. Case presentation: Herein, we report the anesthetic management of a 10-year-old female patient with a double outlet right ventricle. 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 to decrease the risk of thromboembolism, and correction of coagulopathy to prevent hemorrhage. Conclusions Anesthetic management is challenging in rare cyanotic congenital heart disease patients with severe polycythemia. For prevention of thrombosis and hemorrhage, it is important to adopt perioperative hemodilution and correction of coagulopathy.