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
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
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
Background： Double outlet right ventricle （DORV） is a group of complex congenital heart abnormalities. Preoperative pulmonary hypertension （PH） is considered an important risk factor for early death during the su...Background： Double outlet right ventricle （DORV） is a group of complex congenital heart abnormalities. Preoperative pulmonary hypertension （PH） is considered an important risk factor for early death during the surgical treatment of DORV. The aim of this study was to report our experience on surgical treatment of DORV complicated by PH. Methods： From June 2004 to November 2016, 61 patients （36 males and 25 females） aged 2 weeks to 26 years （median： 0.67 years and interquartile range： 0.42-1.67 years） with DORV （two great arteries overriding at least 50%） complicated by PH underwent surgical treatment in our center. All patients were categorized according to surgical age and lesion type, respectively. Pulmonary artery systolic pressure （PASP）, pulmonary artery diastolic pressure （PADP）, and mean pulmonary artery pressure （mPAP） were measured directly before cardiopulmonary bypass （CPB） was established and after CPB was removed. An intracardiac channel procedure was performed in 37 patients, arterial switch procedure in 19 patients, Rastelli procedure in three patient, Senning procedure in one patients, and Mustard procedure in one patient. The Student＇s t-test and Chi-squared test were performed to evaluate clinical outcomes of the surgical timing and operation choice. Results： Fifty-five patients had uneventful recovery. PASP fell from 55.3 ± 11.2 mmHg to 34.7±11.6 mmHg （t = 14.05, P 〈 0.001）, PADP fell from 29.7 ± 12.5 mmHgto 18.6± 7.9 mmHg （t = 7.39,P〈 0.001）, and mPAP fell from 40.3 ± 10.6 mmHgto25.7 ± 8.3 mmHg （t= 11.85, P 〈 0.001）. Six （9.8%） patients died owing to complications including low cardiac output syndrome in two patients, respiratory failure in two, pulmonary hemorrhage in one, and sudden death in one patient. Pulmonary artery pressure （PAP） dropped significantly in infant and child patients. Mortality of both infants （13.9%） and adults （33.3%） was high. Conclusions： PAP of patients with DORV complicated by PH can be expected to fall significantly after surgery. An arterial switch procedure can achieve excellent results in patients with transposition of the great arteries type. Higher incidence of complications may occur in patients with ventricular septal defect （VSD） type before 1 year of age. For those with remote VSD type, VSD enlargement and right ventricle outflow tract reconstruction are usually required with acceptable results. The degree of aortic overriding does not influence surgical outcome.展开更多
ne of premature twins was noticed with thoracoabdominal ectopia cordis and abdominal-walldelect. Purthermore, the mlant was presented with symptoms of tachypnea and moderate cyanosis. Physical examination showed the g...ne of premature twins was noticed with thoracoabdominal ectopia cordis and abdominal-walldelect. Purthermore, the mlant was presented with symptoms of tachypnea and moderate cyanosis. Physical examination showed the giant and pulsating omphalocele measured 40 mm × 50 mm over the anterior abdominal wall. Room air oxygen saturation was 82%. Chest X-ray showed absence of the normal contour of heart in the chest, descensus of the heart to the epigastrium （FigureI A）. Echocardiography visualized abdominal ectopia cordis and double-outlet right ventricle with pulmonary stenosis, the ventricular septal defect and atrial septal defect. Computed tomography （CT） angiography demonstrated a ventral thoracoabdominal wall defect with展开更多
目的回顾性分析47例右心室双出口（double outlet right ventricle，DORV）病例的病理解剖特点及其手术方式，探讨DORV的个案化手术设计治疗。方法自1997年1月至2006年1月对47例DORV患者行手术治疗，其中男性31例，女性16例；年龄2-26...目的回顾性分析47例右心室双出口（double outlet right ventricle，DORV）病例的病理解剖特点及其手术方式，探讨DORV的个案化手术设计治疗。方法自1997年1月至2006年1月对47例DORV患者行手术治疗，其中男性31例，女性16例；年龄2-26（13．6±12．1）岁。诊断为单纯DORV者36例，DORV合并镜面右位心、内脏转位5例，1例合并下腔静脉闭锁、左上腔静脉畸形引流及无顶冠状静脉窦畸形，2例Taussing—-ing心脏畸形，2例伴有完全型房室隔缺损（其中1例伴有大血管转位），46例患者均伴有肺动脉狭窄（PS），临床表现为紫绀和杵状指。另外1例17岁男性患者术前仅由超声心动图诊断为右位心、室间隔缺损（VSD），无紫绀，术中探查为DORV且无PS，肺动脉压不高。手术方式：（1）心内隧道修补VSD、心外补片加宽流出道和肺动脉36例；（2）内管道连接VSD与主动脉，同时作补片加宽右室流出道或切断（开）肺动脉，封闭其近端、远端与右室切口之间用外管道连接（Rastelli手术）8例；（3）全腔静脉-肺动脉连接术2例；（4）Glenn（格林）手术1例。结果平均带气管导管时间（30．6±18．40）h，平均住ICU时间（2．8±1．5）d，平均住院日（25．4±18．O）d。术后出现并发症6例，其中二次插管1例，胸水2例，腹水2例，气胸1例，低心排1例。手术后早期死亡3例，死亡率6．4％。远期随访28例，行超声心动图检查均无残余梗阻和残余分流。结论明确DORV病理解剖关系，根据不同病变采取不同手术方式予以矫治，是取得良好治疗效果的保证。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
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Background： Double outlet right ventricle （DORV） is a group of complex congenital heart abnormalities. Preoperative pulmonary hypertension （PH） is considered an important risk factor for early death during the surgical treatment of DORV. The aim of this study was to report our experience on surgical treatment of DORV complicated by PH. Methods： From June 2004 to November 2016, 61 patients （36 males and 25 females） aged 2 weeks to 26 years （median： 0.67 years and interquartile range： 0.42-1.67 years） with DORV （two great arteries overriding at least 50%） complicated by PH underwent surgical treatment in our center. All patients were categorized according to surgical age and lesion type, respectively. Pulmonary artery systolic pressure （PASP）, pulmonary artery diastolic pressure （PADP）, and mean pulmonary artery pressure （mPAP） were measured directly before cardiopulmonary bypass （CPB） was established and after CPB was removed. An intracardiac channel procedure was performed in 37 patients, arterial switch procedure in 19 patients, Rastelli procedure in three patient, Senning procedure in one patients, and Mustard procedure in one patient. The Student＇s t-test and Chi-squared test were performed to evaluate clinical outcomes of the surgical timing and operation choice. Results： Fifty-five patients had uneventful recovery. PASP fell from 55.3 ± 11.2 mmHg to 34.7±11.6 mmHg （t = 14.05, P 〈 0.001）, PADP fell from 29.7 ± 12.5 mmHgto 18.6± 7.9 mmHg （t = 7.39,P〈 0.001）, and mPAP fell from 40.3 ± 10.6 mmHgto25.7 ± 8.3 mmHg （t= 11.85, P 〈 0.001）. Six （9.8%） patients died owing to complications including low cardiac output syndrome in two patients, respiratory failure in two, pulmonary hemorrhage in one, and sudden death in one patient. Pulmonary artery pressure （PAP） dropped significantly in infant and child patients. Mortality of both infants （13.9%） and adults （33.3%） was high. Conclusions： PAP of patients with DORV complicated by PH can be expected to fall significantly after surgery. An arterial switch procedure can achieve excellent results in patients with transposition of the great arteries type. Higher incidence of complications may occur in patients with ventricular septal defect （VSD） type before 1 year of age. For those with remote VSD type, VSD enlargement and right ventricle outflow tract reconstruction are usually required with acceptable results. The degree of aortic overriding does not influence surgical outcome.
文摘ne of premature twins was noticed with thoracoabdominal ectopia cordis and abdominal-walldelect. Purthermore, the mlant was presented with symptoms of tachypnea and moderate cyanosis. Physical examination showed the giant and pulsating omphalocele measured 40 mm × 50 mm over the anterior abdominal wall. Room air oxygen saturation was 82%. Chest X-ray showed absence of the normal contour of heart in the chest, descensus of the heart to the epigastrium （FigureI A）. Echocardiography visualized abdominal ectopia cordis and double-outlet right ventricle with pulmonary stenosis, the ventricular septal defect and atrial septal defect. Computed tomography （CT） angiography demonstrated a ventral thoracoabdominal wall defect with
文摘目的回顾性分析47例右心室双出口（double outlet right ventricle，DORV）病例的病理解剖特点及其手术方式，探讨DORV的个案化手术设计治疗。方法自1997年1月至2006年1月对47例DORV患者行手术治疗，其中男性31例，女性16例；年龄2-26（13．6±12．1）岁。诊断为单纯DORV者36例，DORV合并镜面右位心、内脏转位5例，1例合并下腔静脉闭锁、左上腔静脉畸形引流及无顶冠状静脉窦畸形，2例Taussing—-ing心脏畸形，2例伴有完全型房室隔缺损（其中1例伴有大血管转位），46例患者均伴有肺动脉狭窄（PS），临床表现为紫绀和杵状指。另外1例17岁男性患者术前仅由超声心动图诊断为右位心、室间隔缺损（VSD），无紫绀，术中探查为DORV且无PS，肺动脉压不高。手术方式：（1）心内隧道修补VSD、心外补片加宽流出道和肺动脉36例；（2）内管道连接VSD与主动脉，同时作补片加宽右室流出道或切断（开）肺动脉，封闭其近端、远端与右室切口之间用外管道连接（Rastelli手术）8例；（3）全腔静脉-肺动脉连接术2例；（4）Glenn（格林）手术1例。结果平均带气管导管时间（30．6±18．40）h，平均住ICU时间（2．8±1．5）d，平均住院日（25．4±18．O）d。术后出现并发症6例，其中二次插管1例，胸水2例，腹水2例，气胸1例，低心排1例。手术后早期死亡3例，死亡率6．4％。远期随访28例，行超声心动图检查均无残余梗阻和残余分流。结论明确DORV病理解剖关系，根据不同病变采取不同手术方式予以矫治，是取得良好治疗效果的保证。
