THE VALUE OF TRANSTHORACIC ECHOCARDIOGRAPHY AND CARDIAC CT IN THE DIAGNOSIS AND PREOPERATIVE PLANNING OF PULMONARY ARTERY ATRESIA WITH VENTRICULAR SEPTAL DEFECT IN CHILDREN; [РОЛЬ ТРАНСТОРАКАЛЬНОЙ ЭХО-КГ И КТ СЕРДЦА В ДИАГНОСТИКЕ И ПРЕДОПЕРАЦИОННОМ ПЛАНИРОВАНИИ АТРЕЗИИ ЛЕГОЧНОЙ АРТЕРИИ С ДЕФЕКТОМ МЕЖЖЕЛУДОЧКОВОЙ ПЕРЕГОРОДКИ У ДЕТЕЙ]

Purpose. To assess and compare transthoracic echocardiography (TTE) cardiac CT in the diagnosis and preoperative planning in children with pulmonary atresia with ventricular septal defect. Materials and methods. The study comprised 38 patients, mean age 1, 5 y.o., all of them underwent TTE, 36 - cardiac CT, 33 - angiocardiography. On TTE, cardiac CT and angiocardiography assessment of pulmonary artery degree hypoplasia was performed. The ability of methods to determine the type of atresia, stenosis of the pulmonary artery branches, and aorto-pulmonary collateral arteries was analyzed. Results. The accuracy of TTE and cardiac CT in the diagnosis of pulmonary atresia with ventricular septal defect was 94.7 % and 100%, respectively. CT of the heart had 100% accuracy (AUC 1.00) in determining the type of pulmonary atresia with ventricular septal defect, assessing the functional state of the pulmonary artery, and in detecting large aortopulmonary collateral arteries. Cardiac CT proved to be a more effective than TTE (p<0.05). Cardiac CT had one erroneous result in large aortopulmonary collateral arteries stenosis detection due to technical limitations in a newborn child with a high heart rate: the accuracy of the method was 96.4% (AUC 0.967) and it was significantly higher than of TTE (accuracy 75%, AUC 0.756). Assessing the possibilities of cardiac CT in the correct determination of the large aortopulmonary collateral arteries segment supplying, the diagnostic efficiency of the method was as follows: sensitivity 85.2%, specificity 98.3%, accuracy 97% (AUC 0.931). TTE did not determine the junction of the large aortopulmonary collateral arteries with native pulmonary artery in any of the cases. Conclusion. TTE is a screening method and has a high diagnostic value in the diagnosis of pulmonary atresia with ventricular septal defect, but due to limitations in assessing the detailed anatomy of the pulmonary arteries (accuracy rates vary from 80% to 86%) and collateral pulmonary blood flow (accuracy less than 85%), it cannot be considered as the method of choice in preoperative planning. Compared to TTE cardiac CT is a more effective technique both in diagnosing pulmonary atresia with ventricular septal defect in children and in preoperative planning (the accuracy of the method varies from 96 to 100%), and in patients with pulmonary atresia with ventricular septal defect types 1 and 2 can be an alternative to angiocardiography tool in case of planning open palliative interventions. In case of planning endovascular correction of pulmonary atresia with ventricular septal defect 1 and 2 types cardiac CT is a duplicating angiocardiography technique. In some cases, CT of the heart has limitations in assessing the distal parts of the large aortopulmonary collateral arteries at the level of the pulmonary segment - an important preoperative goal in treatment children with pulmonary atresia with ventricular septal defect types 3-4. For this reason, in this group of patients, angiocardiography remains the method of choice for preoperative planning for the correction of pulmonary atresia with ventricular septal defect. © 2023 Russian Electronic Journal of Radiology. All rights reserved.