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.

Anesthetic management of a child with double outlet right ventricle and severe polycythemia: A case report

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.

Biventricular repair for endocardial cushion defects with double outlet right ventricle

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

2q37.3 Deletion with Complex Heart Defects Suggesting Interruption of Early Ventricular Looping

A maternally inherited 828 kb microdeletion of 2q37.3 manifested in a 3-week-old premature boy as left juxtaposition of the atrial appendages associated with tricuspid atresia,double outlet infundibulum,subvalvar pulmonary atresia,large secundum atrial septal defect,and right aortic arch with mirror-image branching,consistent with developmental arrest early in heart looping.To the best of our knowledge,no previous 2q37 deletion syndrome has been reported with such a severe cardiac dysmorphology.Hence,this case adds to the cardiac phenotypes identified in 2q37 deletion syndrome.

Interventricular Septal Hematoma after Congenital Cardiac Defects Repair at a Single Institution

Background: Interventricular septal hematoma is a rare complication after congenital cardiac repair. The managementvaries according to the literature. We present our experience with this rare complication. Methods:Echocardiography database were reviewed with the term ‘‘hematoma’’ or “hypoechoic mass” for patients whounderwent congenital heart surgery from January 2018 to December 2021 at our institution to identify potentialinterventricular septal hematoma cases. Relevant data of the patients identified were collected. Focus was put onthe presentation, management, outcomes according to patent medical charts and serial echocardiographic reportdata. Results: In total, there were 5 patients included. The mean age and weight at surgery were 5.5 ± 3.6 monthsand 5.5 ± 1.4 kg, respectively. Four patients were diagnosed with ventricular septal defect and the other one beingdouble outlet of the right ventricle. While all patients had intraoperative transesophageal echocardiography, 80%(4 of 5) of Interventricular septal hematoma were revealed intraoperatively. Only one patient received hematomadrainage intraoperatively while the other 3 identified in the operating room were only closely observed. One afterventricular septal defect repair presented continuous dysfunction of the left ventricle at the last follow-up, whilethe others were doing well. All hematomas resolved completely with a mean time to interventricular septal hematomaresolution of 35.8 ± 16.9 days. Conclusion: Infants seem to be at a higher risk for Interventricular septalhematoma following congenital heart surgery. While the majority of interventricular septal hematoma has abenign postoperative course, some may result in ventricular dysfunction. Management strategies may be chosenon a case-by-case basis.

Concordant Atrioventricular Connection to L-Looped Ventricles with the Left Ventricle on Top of the Right Ventricle in Situs Solitus:A Case Report with 3D Modelling and Printing

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.

Surgical Treatment of Double Outlet Right Ventricle Complicated by Pulmonary Hypertension

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.

Surgical treatment of double outlet ventricle: report on 72 cases

Double outlet ventricle (DOV) is defined as a congenital heart disease with: ( 1) both, great arteries arising completely, or almost completely (greater than or equal to 90%), from a single ventricle ( usually, right ventricle); ( 2) an aortic valve having no fibre connection with mitral valve; and (3) the only outlet of the other ventricle is a ventricular septal defect (VSD). The incidence of DOV accounts for 1% of all congenital heart disease.(1) Clinically there are three common types: Fallot type or double outlet right ventricle (DORV) with subaortic VSD with pulmonary stenosis; Eisenmenger type or DORV with subaortic VSD without pulmonary stenosis and Taussig-Bing type or DORV with subpulmonary VSD. Operative techniques are different according to the position of the VSD and great arteries and the presence or absence of ventricular outflow tract obstruction. The operative difficulty increases in the presence of coronary artery malformation. Recently, our research centre has treated 72 cases of patients suffering from double outlet ventricle. This article is the report of the operative methods and the outcomes.