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 polycy...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.展开更多
基金The 1.3.5.Project for Disciplines of Excellence,No.2018HXFH046West China Hospital,Sichuan University and the National Natural Science Foundation of China,No.81971806.
文摘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.
