Evaluation of pulmonary arteries and source of pulmonary blood supply in children with pulmonary atresia and ventricular septal defect using CT angiography
Main Article Content
Abstract
Objective: To evaluate pulmonary arteries and determine the sources of pulmonary blood supply in children with pulmonary atresia and ventricular septal defect (PA-VSD) using computed tomography angiography (CTA).
Methods: This retrospective cross-sectional study was conducted at Pediatric Cardiology Clinic, Hayatabad Medical Complex, Peshawar, Pakistan, from January 2009 to December 2019. Pediatric patients (≤18 years) with confirmed PA-VSD who underwent CTA for preoperative anatomical assessment were included. CTA was used to evaluate pulmonary artery morphology, patent ductus arteriosus (PDA), and major aortopulmonary collateral arteries (MAPCAs). Based on CTA findings, patients were classified according to the source of pulmonary blood supply into Type A (native pulmonary arteries supplied via PDA), Type B (native pulmonary arteries supplied by both PDA and MAPCAs), and Type C (absence of native pulmonary arteries with MAPCAs only). Statistical analysis was performed using SPSS version-22.
Results: Ninety-one patients were included, with a mean age of 3.2±2.8 years; 58 (63.7%) were males. Cyanosis was the most common presenting symptom (94.5%). Type B PA-VSD was the most frequent anatomical pattern (46.2%), followed by Type C (27.5%) and Type A (26.4%). Twenty-four patients (26.4%) had oxygen saturations below 60%, 50 patients (54.9%) had oxygen saturations between 60% and 80%, and 17 patients (18.7%) had oxygen saturations above 80% (p=0.018). CTA demonstrated higher detection rates than echocardiography for MAPCAs (83.3% vs 47.4%, p<0.001) and pulmonary artery stenosis (25.6% vs 10.3%, p=0.012).
Conclusion: CTA provides comprehensive anatomical delineation of pulmonary arteries and collateral circulation in PA-VSD and is valuable for accurate preoperative evaluation.
Article Details

This work is licensed under a Creative Commons Attribution 4.0 International License.
Work published in KMUJ is licensed under a
Creative Commons Attribution 4.0 License
Authors are permitted and encouraged to post their work online
(e.g., in institutional repositories or on their website) prior to and during the submission process, as it can lead to productive exchanges, as well as earlier and greater citation of published work.
References
1. Lertsakulpiriya K, Vijarnsorn C, Chanthong P, Chungsomprasong P, Kanjanauthai S, Durongpisitkul K, et al. Current era outcomes of pulmonary atresia with ventricular septal defect: a single center cohort in Thailand. Sci Rep 2020;10(1):5165. https://doi.org/10.1038/s41598-020-61879-2
2. Tchervenkov CI, Roy NJTaots. Congenital heart surgery nomenclature and database project: pulmonary atresia-ventricular septal defect. Ann Thorac Surg 2000;69(3):97-105. https://doi.org/10.1016/S0003-4975(99)01285-0
3. Kim M, Woo JJ, An JK, Cho YKJJJoR. Anomalous origin of the right pulmonary artery from the ascending aorta associated with patent ductus arteriosus: focusing on computed tomography findings. Jpn J Radiol 2015;33(3):164-8. https://doi.org/10.1007/s11604-015-0391-8
4. Ekure EN, Kalu N, Sokunbi OJ, Kruszka P, Olusegun-Joseph AD, Ikebudu D, et al. Clinical epidemiology of congenital heart disease in Nigerian children, 2012-2017. Birth Defects Res 2018;110(16):1233-40. https://doi.org/10.1002/bdr2.1361
5. Peng J, Wang Q, Meng Z, Wang J, Zhou Y, Zhou S, et al. A loss-of-function mutation p.T256M in NDRG4 is implicated in the pathogenesis of pulmonary atresia with ventricular septal defect (PA/VSD) and tetralogy of Fallot (TOF). FEBS Open Bio 2021;11(2):375-85. https://doi.org/10.1002/2211-5463.13044
6. Zhou J, Zhou Q, Peng Q, Zhang R, Tang W, Zeng S. Fetal pulmonary atresia with ventricular septal defect: Features, associations, and outcome in fetuses with different pulmonary circulation supply types. Prenat Diag 2019;39(12):1047-53. https://doi.org/10.1002/pd.5538
7. Tchervenkov CI, Tang R, Peek GJ, Bleiweis MS, Jacobs JP. Pulmonary atresia and ventricular septal defect: definitions, nomenclature, and classification. World J Pediatr Congenit Heart Surg 2025;16(2):173-6. https://doi.org/10.1177/21501351241311878
8. Barron DJ, Kutty RS, Stickley J, Stümper O, Botha P, Khan NE, et al. Unifocalization cannot rely exclusively on native pulmonary arteries: the importance of recruitment of major aortopulmonary collaterals in 249 cases†. Eur J Cardio-thoracic Surg 2019;56(4):679-87. https://doi.org/10.1093/ejcts/ezz070
9. Vesel S, Rollings S, Jones A, Callaghan N, Simpson J, Sharland GK. Prenatally diagnosed pulmonary atresia with ventricular septal defect: echocardiography, genetics, associated anomalies and outcome. Heart 2006;92(10):1501-5. https://doi.org/10.1136/hrt.2005.083295
10. Gurson SC. Pulmonary atresia with ventricular septal defect without major aorto-pulmonary collateral arteries: echocardiography and the role of computed tomography and magnetic resonance imaging. World J Pediatr Congenit Heart Surg 2025;16(2):183-90. https://doi.org/10.1177/21501351241289128
11. Kumari V, Shaikh AS, Zakai SB, Kumar N, Bangash SK, Patel N. Incidence of aortic regurgitation in association with type of ventricular septal defects and its immediate and intermediate outcome after surgical closure. Cureus 2019;11(7):e5102. https://doi.org/10.7759/cureus.5102
12. Lizano Santamaria RW, Gillespie MJ, Dori Y, Rome JJ, Glatz AC. Palliative balloon pulmonary valvuloplasty for infants with unrestrictive ventricular septal defect or single ventricle associated with severe pulmonary stenosis. Catheter Cardiovasc Interv 2015;86(5):829-33. https://doi.org/10.1002/ccd.26083
13. Zikarg YT, Yirdaw CT, Aragie TG. Prevalence of congenital septal defects among congenital heart defect patients in East Africa: a systematic review and meta-analysis. PLoS One 2021;16(4):e0250006. https://doi.org/10.1371/journal.pone.0250006
14. Zou MH, Ma L, Cui YQ, Wang HZ, Li WL, Li J, et al. Outcomes after repair of pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries: a tailored approach in a developing setting. Front Cardiovasc Med 2021;8:665038. https://doi.org/10.3389/fcvm.2021.665038
15. Soquet J, Barron DJ, d'Udekem Y. A review of the management of pulmonary atresia, ventricular septal defect, and major aortopulmonary collateral arteries. Ann Thorac Surg 2019;108(2):601-12. https://doi.org/10.1016/j.athoracsur.2019.01.046
16. Kaskinen AK, Helve O, Andersson S, Kirjavainen T, Martelius L, Mattila IP, et al. Chronic hypoxemia in children with congenital heart defect impairs airway epithelial sodium transport. Pediatr Crit Care Med 2016;17(1):45-52. https://doi.org/10.1097/PCC.0000000000000568
17. Mainwaring RD, Desai M, Punn R, Felmly LM, Hanley FL. Retroaortic innominate vein in association with pulmonary atresia/major aortopulmonary collaterals. Ann Thorac Surg Short Rep 2023;1(3):436-40. https://doi.org/10.1016/j.atssr.2023.04.002
18. Güzelbağ AN, Baş S, Toprak MHH, Kangel D, Çoban Ş, Sağlam S, et al. Transforming cardiac imaging: can CT angiography replace interventional angiography in tetralogy of fallot? J Clin Med 2025;14(5):1493. https://doi.org/10.3390/jcm14051493
19. Wipf A, Christmann M, Navarini-Meury S, Dave H, Quandt D, Knirsch W, et al. Aortopulmonary collaterals in neonates with d-transposition of the great arteries - clinical significance early after arterial switch operation. Int J Cardiol 2018;258:237-42. https://doi.org/10.1016/j.ijcard.2018.01.132
20. Kumar N, Hussain N, Kumar J, Essandoh MK, Bhatt AM, Awad H, et al. Evaluating the impact of pulmonary artery obstruction after lung transplant surgery: a systematic review and meta-analysis. Transplantation 2021;105(4):711-22. https://doi.org/10.1097/TP.0000000000003407
21. Haydin S, Genç SB, Ozturk E, Yıldız O, Gunes M, Tanidir IC, et al. Surgical strategies and results for repair of pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals: experience of a single tertiary center. Braz J Cardiovasc Surg 2020;35(4):445-51. https://doi.org/10.21470/1678-9741-2019-0055
22. Yadav A, Bhargava S, Buxi TBS, Sirvi K. Collateral or fistula? Coronary artery as the primary source of pulmonary blood flow in a patient with pulmonary atresia and ventricular septal defect. Indian J Radiol Imag 2018;28(4):433-5. https://doi.org/10.4103/ijri.IJRI_489_17
23. Sridhar A, Subramanyan R, Cherian KM. Coronary artery to pulmonary artery communications in pulmonary atresia with ventricular septal defect. Indian Heart J 2013;65(5):636-8. https://doi.org/10.1016/j.ihj.2013.08.017