One-Stage Correction with Intra- and Extraatrial Rerouting of Anomalous Systemic and Pulmonary Venous Return and Intraventricular Repair of Double Outlet Right Ventricle in a Patient with Heterotaxy Syndrome

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.

Pressure-adjustable shunt in treatment of hydrocephalus associated with congenital intracranial anomalous venous return

Objective To investigate the value of Codman Hakim pressure adjustable shunt in the treatment of hydrocephalus associated with congenital intracranial anomalous venous return. Methods 2 patients whose diagnosis were confirmed by CT,MRI,MRV and DSA and programmable ventriculoperitoneal shunting was given.

Monitoring Changes in Hepatic Venous Velocities Flow after a Fluid Challenge Can Identify Shock Patients Who Lack Fluid Responsiveness

Background： Evaluating the hemodynamic status and predicting fluid responsiveness are important in critical ultrasound assessment of shock patients. Transthoracic echocardiography with noninvasive diagnostic parameters allows the assessment of volume responsiveness. This study aimed to assess tile hemodynamic changes in the liver and systemic hemodynamic changes during fluid challenge and during passive leg raising （PLR） by measuring hepatic venous flow （HVF） velocity. Methods： This is an open-label study in a tertiary teaching hospital. Shock patients with hypoperfusion who required fluid challenge were selected for the study. Patients 〈l 8 years old and those with contraindications to PLR were excluded from the study. Baseline values were measured, PLR tests were performed, and 500 ml of saline was infused over 30 rain. Parameters associated with cardiac output （CO） in the left ventricular outflow tract were measured using the Doppler method. In addition, HVF velocity and right ventricular function parameters were determined. Results： Middle hepatic venous （MHV） S-wave velocity was positively correlated in all patients with CO at baseline （r= 0.706, P〈 0.01 ） and after volume expansion （r= 0.524, P = 0.003）. CO was also significantly correlated with MHV S-wave velocity in responders （r = 0.608, P 〈 0.01）. During PLR, however, hepatic venous S-wave velocity did not correlate with CO. For the parameter AMHV D （increase in change in MHV D-wave velocity after volume expansion）, defined as （MHV D - MHV Dtaseline）/MHV DBaseli.,e x 100%, 〉21% indicated no fluid responsiveness, with a sensitivity of 100%, a specificity of 71.2%, and an area under the receiver operating characteristic curve of 0.918. Conclusions： During fluid expansion, hepatic venous S-wave velocity can be used to monitor CO, whether or not it is increasing. AMHV D 〉21% indicated a lack of fluid responsiveness, thus helping to decide when to stop infusions.