Abstract

Introduction: Total anomalous pulmonary venous return (TAPVR) is a congenital heart defect in which there is no direct connection between pulmonary veins and the left atrium. Hospital mortality in patients with TAPVR, depending on the anatomical type of the defect, ranges from 14% to 35%. Without surgical correction, children with obstructive TAPVR die in the neonatal period. The mortality rate of children with nonobstructive TAPVR reaches 80% by the first year of life.Objective: To evaluate the capabilities of transthoracic echocardiography (TTE) and cardiac computed tomography (CT) in TAPVR diagnosis and surgical planning of its correction.Materials and methods: The study included 17 children with an initial diagnosis of TAPVR. The age of the patients was 28.5 (11; 91) days. All the infants underwent TTE; 15 patients underwent cardiac CT, and 4 patients, cardiac catheterization with conventional angiography. We evaluated and compared the capabilities of these methods in the diagnosis and determination of the TAPVR type and concomitant anomalies. All data were compared with conventional angiography findings and intraoperative data. The interval between TTE, cardiac CT, and angiography or open surgery was less than 5 days.Results: The accuracy of TTE in diagnosing TAPVR was not significantly inferior to that of cardiac CT (88% vs 100%); erroneous results of TTE were found in 2 patients with heterotaxy syndrome (right isomerism). Both methods showed 100% diagnostic efficiency in determining the TAPVR type. The accuracy of TTE in diagnosing obstructive TAPVR was 81.2%, whereas that of cardiac CT, 94%. There was no statistically significant difference in diagnostic efficiency between CT and TTE in the diagnosis of TAPVR and its obstructive forms.Discussion: Our findings demonstrate the high efficiency of TTE in diagnosing TAPVR, determining its anatomical type, and identifying obstructive forms. This method has limited diagnostic capabilities in determining TAPVR in patients with heterotaxy syndrome and other congenital anomalies of the atria. Cardiac CT has proven to be a highly accurate tool in diagnosing TAPVR and determining obstructive forms and associated heart anomalies.Conclusions: In children with isolated TAPVR and normal arrangement of the organs (situs solitus) with good acoustic window, TTE is the initial and final tool for surgical planning of TAPVR correction. In children with TAPVR and concomitant heart defects, heterotaxy syndrome and other atrial anomalies, TTE should be supplemented by cardiac CT. Cardiac CT in children with TAPVR is an alternative to invasive cardiac catheterization with conventional angiography and should be a preferred diagnostic tool. Conventional angiography should be used in children with TAPVR for assessment of functional, not anatomical characteristics of the defect.

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