Diagnostic Capabilities of Transthoracic Echocardiography and Cardiac Computed Tomography in Preoperative Planning of Total Anomalous Pulmonary Venous Return Correction

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. 

1. Sharykin AS. Congenital Heart Defects. Guide for Pediatricians, Cardiologists, Neonatologists. BINOM; 2009. (In Russ.).

2. Russian Association of Cardio-Vascular Surgeons. Clinical Guidelines. Total Anomalous Pulmonary Venous Return. Russian Association of Cardio-Vascular Surgeons; 2021. (In Russ.).

3. Stein P. Total anomalous pulmonary venous connection. AORN J. 2007;85(3):509–524. PMID: 17352891. https://doi.org/10.1016/S0001-2092(07)60123-9

4. Kanter KR. Surgical repair of total anomalous pulmonary venous connection. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 2006;40–44. PMID: 16638546. https://doi.org/10.1053/j.pcsu.2006.02.015

5. Files MD, Arya B. Preoperative physiology, imaging, and management of transposition of the great arteries. Semin Cardiothorac Vasc Anesth. 2015;19(3):210–222. PMID: 25900899. https://doi.org/10.1177/1089253215581851

6. Movsesyan RR, Morozov AA, Golubeva MV, Fedorova NV. The role of the routine use of computed tomography angiography for diagnosis of different anatomical type of pulmonary venous drainage in children with total anomalous pulmonary venous connection. Children’s Heart and Vascular Diseases. 2019;16(4):254– 258. https://doi.org/10.24022/1810-0686-2019-16-4-254-258

7. Sokolov AA, Krivoshchekov EV, Smorgon AV, Kovalev IA. Features of echocardiographic examination in patients with completed Fontan operation. Russian Electronic Journal of Radiology. 2021;11(3):95–106. (In Russ.). https://doi.org/10.21569/2222-7415-2021-11-3-95-106

8. Lee ML, Wu MH, Wang JK, Lue HC. Echocardiographic assessment of total anomalous pulmonary venous connections in pediatric patients. J Formos Med Assoc. 2001;100(11):729–735. PMID: 11802530.

9. Ren ST, Wang Y, Zhou JH, et al. Echocardiographic diagnosis of infracardiac total anomalous pulmonary venous connection. Beijing Da Xue Xue Bao Yi Xue Ban. 2017;49(5):883–888. (In Chinese). PMID: 29045974.

10. Oh KH, Choo KS, Lim SJ, et al. Multidetector CT evaluation of total anomalous pulmonary venous connections: comparison with echocardiography. Pediatr Radiol. 2009;39(9):950–954. PMID: 19506848. https://doi.org/10.1007/s00247-009-1309-3

11. Festa P, Ait-Ali L, Cerillo AG, De Marchi D, Murzi B. Magnetic resonance imaging is the diagnostic tool of choice in the preoperative evaluation of patients with partial anomalous pulmonary venous return. Int J Cardiovasc Imaging. 2006;22(5):685–693. PMID: 16547601. https://doi.org/10.1007/s10554-005-9070-7

12. Ali F, Qureshi S, Amanullah M, Atiq M. Accuracy of echocardiography in diagnosing total anomalous pulmonary venous return. Pak J Med Sci. 2018;34(5):1094–1098. PMID: 30344556. PMCID: PMC6191797. https://doi.org/10.12669/pjms.345.15766

13. Atmashkin AA, Kim AI, Grigor’yants TR, Rogova TV, Kurganov RM, Popov AE. 15-year experience of surgical treatment total anomalous pulmonary venous connections. Children’s Heart and Vascular Diseases. 2021;18(3):176–183. (In Russ.). https://doi.org/10.24022/1810-0686-2021-18-3-176-183