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
A 17-year-old adolescent with non-operated double inlet left-ventricle and severe stenosed parachute mitral valve is reported. He was admitted with repetitive syncope related to intermittent atrial fibrillation. Life-...A 17-year-old adolescent with non-operated double inlet left-ventricle and severe stenosed parachute mitral valve is reported. He was admitted with repetitive syncope related to intermittent atrial fibrillation. Life-threatening syncope combined with pre- and post-capillary pulmonary hypertension together with his single ventricle pathophysiology led to the decision for left atrial decompression by percutaneous static atrial septum ballooning after transseptal needle perforation. Aiming to create a restrictive atrial septum defect, unloading of the left atrium without disturbing the balanced hemodynamics was directed for a long-term palliation or as a basis for a further surgical follow-up approach.展开更多
We report a very rare case of successful intracardiac correction in a patient with heterotaxy syndrome.The cardiac malformations included dextrocardia,double outlet right ventricle,pulmonary stenosis,interrupted infer...We report a very rare case of successful intracardiac correction in a patient with heterotaxy syndrome.The cardiac malformations included dextrocardia,double outlet right ventricle,pulmonary stenosis,interrupted inferior vena cava,hemiazygos continuation and total anomalous pulmonary venous return.One-stage correction was performed.The atrial procedure consisted of intra-and extraatrial rerouting of the anomalous systemic and pulmonary venous return.The hepatic veins were detached and diverted to the left atrium via an extracardiac conduit.The correction of the double outlet right ventricle was accomplished by intraventricular redirection of the blood flow from the left ventricle to the aorta.The right ventricular outflow was ultimately remodeled using a valved conduit.For better perception of the complex morphology,a three-dimensional model was designed,using CT scan images.This proved to be very useful for surgical planning,especially with regard to the intraatrial reconstruction of the systemic and pulmonary venous rerouting.展开更多
Objective-Double outlet right ventricle,which often associated with total anomalous pulmonary venous connection and complete endocardial cushion defects,has been considered a risk factor for biventricular repair proce...Objective-Double outlet right ventricle,which often associated with total anomalous pulmonary venous connection and complete endocardial cushion defects,has been considered a risk factor for biventricular repair procedure. To reviewed cases treated by biventricular repair for endocardial cushion defects with double outlet right ventricle. Methods From July to November of 2009,6展开更多
In response to the divergent understanding of double inlet cyclone performance in the literature,the effect of inlet volute wrap angle on the performance and flow field of double inlet cyclone separator was studied by...In response to the divergent understanding of double inlet cyclone performance in the literature,the effect of inlet volute wrap angle on the performance and flow field of double inlet cyclone separator was studied by Computational Fluid Dynamic(CFD)method.The results showed that the inlet volute wrap angle can affect the comparison results of the single and double inlet gas cyclones with the same total inlet cross-sectional area and velocity.0°and 90°volute double inlet improved the efficiency mainly by separating particles below 10μm,while 180°volute double inlet had no separation advantage for any particles,so the symmetrical double inlet does not always improve the efficiency,and the appropriate inlet volute wrap angle should be selected according to the actual situation,otherwise,the expected performance requirements of the symmetrical double inlet cyclone cannot be achieved.Compared with the flow field,it is found that the inlet volute wrap angle changed the tangential velocity of the sym-metrical double inlet cyclone separator,thus changing the performance.展开更多
We report the case of a rare complex cardiac anomaly involving situs solitus,concordant atrioventricular connection with left-hand ventricular topology,and L-looped ventricles.The ventricles had a superior-inferior re...We report the case of a rare complex cardiac anomaly involving situs solitus,concordant atrioventricular connection with left-hand ventricular topology,and L-looped ventricles.The ventricles had a superior-inferior relationship with an inferiorly located right ventricle,which had a double outlet with far posteriorly located great arteries.The left atrium was elongated,with juxta-positioned atrial appendages on the right side.The 3D-printed model using a computed tomography scan taken on the fourth day of birth demonstrated the anatomy clearly and helped us decide on the surgical management.展开更多
A 23-year-old, lean, scoliotic female presented to our hospital with a history of shortness of breath and cyanosis on exertion. Her 2D echocardiography revealed single left ventricle with both atrio-ventricular valves...A 23-year-old, lean, scoliotic female presented to our hospital with a history of shortness of breath and cyanosis on exertion. Her 2D echocardiography revealed single left ventricle with both atrio-ventricular valves opening in it. She had normally related great arteries, with severe pulmonary artery hypertension, without pulmonary stenosis. Her blood tests indicated that she was reactive to human immunodeficiency virus (HIV-1). The patients died within 2 months despite treatments with anti-retroviral therapy and decongestive therapy.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.
文摘A 17-year-old adolescent with non-operated double inlet left-ventricle and severe stenosed parachute mitral valve is reported. He was admitted with repetitive syncope related to intermittent atrial fibrillation. Life-threatening syncope combined with pre- and post-capillary pulmonary hypertension together with his single ventricle pathophysiology led to the decision for left atrial decompression by percutaneous static atrial septum ballooning after transseptal needle perforation. Aiming to create a restrictive atrial septum defect, unloading of the left atrium without disturbing the balanced hemodynamics was directed for a long-term palliation or as a basis for a further surgical follow-up approach.
文摘We report a very rare case of successful intracardiac correction in a patient with heterotaxy syndrome.The cardiac malformations included dextrocardia,double outlet right ventricle,pulmonary stenosis,interrupted inferior vena cava,hemiazygos continuation and total anomalous pulmonary venous return.One-stage correction was performed.The atrial procedure consisted of intra-and extraatrial rerouting of the anomalous systemic and pulmonary venous return.The hepatic veins were detached and diverted to the left atrium via an extracardiac conduit.The correction of the double outlet right ventricle was accomplished by intraventricular redirection of the blood flow from the left ventricle to the aorta.The right ventricular outflow was ultimately remodeled using a valved conduit.For better perception of the complex morphology,a three-dimensional model was designed,using CT scan images.This proved to be very useful for surgical planning,especially with regard to the intraatrial reconstruction of the systemic and pulmonary venous rerouting.
文摘Objective-Double outlet right ventricle,which often associated with total anomalous pulmonary venous connection and complete endocardial cushion defects,has been considered a risk factor for biventricular repair procedure. To reviewed cases treated by biventricular repair for endocardial cushion defects with double outlet right ventricle. Methods From July to November of 2009,6
基金supported by the National Natural Science Foundation of China(grant No.21978322,21276274).
文摘In response to the divergent understanding of double inlet cyclone performance in the literature,the effect of inlet volute wrap angle on the performance and flow field of double inlet cyclone separator was studied by Computational Fluid Dynamic(CFD)method.The results showed that the inlet volute wrap angle can affect the comparison results of the single and double inlet gas cyclones with the same total inlet cross-sectional area and velocity.0°and 90°volute double inlet improved the efficiency mainly by separating particles below 10μm,while 180°volute double inlet had no separation advantage for any particles,so the symmetrical double inlet does not always improve the efficiency,and the appropriate inlet volute wrap angle should be selected according to the actual situation,otherwise,the expected performance requirements of the symmetrical double inlet cyclone cannot be achieved.Compared with the flow field,it is found that the inlet volute wrap angle changed the tangential velocity of the sym-metrical double inlet cyclone separator,thus changing the performance.
文摘We report the case of a rare complex cardiac anomaly involving situs solitus,concordant atrioventricular connection with left-hand ventricular topology,and L-looped ventricles.The ventricles had a superior-inferior relationship with an inferiorly located right ventricle,which had a double outlet with far posteriorly located great arteries.The left atrium was elongated,with juxta-positioned atrial appendages on the right side.The 3D-printed model using a computed tomography scan taken on the fourth day of birth demonstrated the anatomy clearly and helped us decide on the surgical management.
文摘A 23-year-old, lean, scoliotic female presented to our hospital with a history of shortness of breath and cyanosis on exertion. Her 2D echocardiography revealed single left ventricle with both atrio-ventricular valves opening in it. She had normally related great arteries, with severe pulmonary artery hypertension, without pulmonary stenosis. Her blood tests indicated that she was reactive to human immunodeficiency virus (HIV-1). The patients died within 2 months despite treatments with anti-retroviral therapy and decongestive therapy.
