Vol. 30 No. 2 (2026)
CONGENITAL HEART DISEASES

The role of early unifocalization in the correction of pulmonary atresia with ventricular septal defect: the retrospective analysis

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  • Ekaterina N. Amansakhatova,
  • Yuriy Yu. Kulyabin,
  • Alexey V. Voitov,
  • Nataliya R. Nichay,
  • Alexey N. Arkhipov,
  • Ilya A. Soynov
Ekaterina N. Amansakhatova
Meshalkin National Medical Research Center; Novosibirsk State University
Yuriy Yu. Kulyabin
Meshalkin National Medical Research Center, Ministry of Health of the Russian Federation
Alexey V. Voitov
Meshalkin National Medical Research Center, Ministry of Health of the Russian Federation
Nataliya R. Nichay
Meshalkin National Medical Research Center, Ministry of Health of the Russian Federation; Novosibirsk State Medical University
Alexey N. Arkhipov
Meshalkin National Medical Research Center, Ministry of Health of the Russian Federation
Ilya A. Soynov
Meshalkin National Medical Research Center, Ministry of Health of the Russian Federation
DOI: https://doi.org/10.21688/1681-3472-2026-2-45-54

Published 2026-08-10

Keywords

  • pulmonary atresia with ventricular septal defect, congenital heart disease; unifocalization of the pulmonary arteries

How to Cite

Amansakhatova, E. N. ., Kulyabin, Y. Y. ., Voitov, A. V. ., Nichay, N. R. ., Arkhipov, A. N. ., & Soynov, I. A. . (2026). The role of early unifocalization in the correction of pulmonary atresia with ventricular septal defect: the retrospective analysis. Patologiya Krovoobrashcheniya I Kardiokhirurgiya, 30(2), 45–54. https://doi.org/10.21688/1681-3472-2026-2-45-54

Copyright (c) 2026 Amansakhatova et al.

Creative Commons License

This work is licensed under a Creative Commons Attribution 4.0 International License.

Abstract

Introduction: The presence of multifocal pulmonary blood flow in pulmonary atresia with ventricular septal defect plays a critical role in determining the strategy for surgical correction. Early unifocalization of the pulmonary arterial bed facilitates achievement of complete repair with physiological right ventricular pressure.

Objective: To evaluate the effectiveness of pulmonary artery reconstruction with unifocalization to achieve radical correction in pulmonary artery atresia with interventricular defect and multifocal pulmonary flow.

Methods: This study involved 25 patients with pulmonary atresia and ventricular septal defect types B and C (according to the classification of C.
Tchervenkov), who underwent unifocalization of the pulmonary bed. In accordance with age at the time of correction, all patients were divided into two groups: Group EU (early unifocalization, n = 11) – patients operated on at the age of up to 1 year, Group LU (late unifocalization, n = 14) – patients operated on at the age of over 1 year. The primary endpoint was successful complete repair with maintenance of right ventricular pressure at less than two-thirds of systemic pressure. The secondary endpoints included the rates of reintervention, freedom from reoperation, incidence of residual pulmonary hypertension, and rate of adverse outcomes.

Results: The age of patients in the groups with early and late unifocalization of the pulmonary bed was 6.0 (0; 11) and 90.5 (13; 132) months. Pulmonary hypertension in the preoperative period was diagnosed in 1 patient (9 %) in the EU group, and in 11 patients (79 %) in the LU group (p = 0.001). Radical correction and right ventricular pressure less than 2/3 of the systemic pressure were achieved more often in patients in group EU than in group LU (73 % vs. 29 %, р = 0.04 and 89 % vs. 38 %, p = 0.03). Repeated transcatheter interventions and surgical redo procedures were required more often in patients from group LU (85 %, р = 0.02, 85 %, р = 0.007). There were no statistically significant differences in early and long-term mortality rates between the groups (p = 1.0).

Conclusion: Early unifocalization of pulmonary blood flow allows achieving
optimal parameters for achieving radical correction of the defect. After radical correction, most patients who underwent unifocalization at an early age retain low pressure in the right ventricle in the long term and are less susceptible to repeated interventions.

Received 28 November 2024. Revised 25 September 2025. Accepted 30 January 2026.

Funding
The research was carried out within the framework of the state assignment of the Ministry of Health of the Russian Federation (No. 124022500251-0).

Conflict of interest

The authors declare no conflict of interest.

Contribution of the authors
Conception and study design: E.N. Amansakhatova, Yu.Yu. Kulyabin, A.V. Voitov, N.R. Nichay, A.N. Arkhipov

Data collection and analysis: E.N. Amansakhatova, A.V. Voitov, N.R. Nichay, A.N. Arkhipov

Statistical analysis: E.N. Amansakhatova, Yu.Yu. Kulyabin, N.R. Nichay, I.A. Soynov

Drafting the article: E.N. Amansakhatova, Yu.Yu. Kulyabin, I.A. Soynov

Critical revision of the article: Yu.Yu. Kulyabin, N.R. Nichay, A.N. Arkhipov, I.A. Soynov

Final approval of the version to be published: all authors

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References

  1. Ikai A. Surgical strategies for pulmonary atresia with ventricular septal defect associated with major aortopulmonary collateral arteries. Gen Thorac Cardiovasc Surg. 2018;66(7):390-397. PMID: 29802565. https://doi.org/10.1007/s11748-018-0948-4
  2. Kumar S.R. Collateral benefit in pulmonary atresia with ventricular septal defect? J Thorac Cardiovasc Surg. 2018;156(3):1205-1206. PMID: 29753507. https://doi.org/10.1016/j.jtcvs.2018.04.030
  3. Korun O., Yurdakök O., Dedemoğlu M., Yücel İ.K., Çelebi A., Kudsioğlu Ş.T., Sasmazel A., Aydemir N.A. Midline one-stage complete unifocalization early outcomes from a single center. Anatol J Cardiol. 2019;22(3):125-131. PMID: 31475955; PMCID: PMC6735431. https://doi.org/10.14744/AnatolJCardiol.2019.58235
  4. van de Woestijne P., Mokhles M., van Beynum I., de Jong P., Wilschut J., Bogers A. Staged correction of pulmonary atresia, ventricular septal defect, and collateral arteries. J Card Surg. 2022;37(4):960-966. PMID: 35142386; PMCID: PMC9303625. https://doi.org/10.1111/jocs.16299
  5. Malankar D.P., Mali S., Garekar S., Sheth R. Single-Stage Unifocalization and Intracardiac Repair Using Two Tube Grafts. Ann Thorac Surg. 2022;113(4):e299-e302. PMID: 34283957. https://doi.org/10.1016/j.athoracsur.2021.07.007
  6. Lertsakulpiriya K., Vijarnsorn C., Chanthong P., Chungsomprasong P., Kanjanauthai S., Durongpisitkul K., Soongswang J., Subtaweesin T., Sriyoschati S. Current era outcomes of pulmonary atresia with ventricular septal defect: A single center cohort in Thailand. Sci Rep. 2020;10(1):5165. PMID: 32198468; PMCID: PMC7083910. https://doi.org/10.1038/s41598-020-61879-2
  7. Zou M.H., Ma L., Cui Y.Q., Wang H.Z., Li W.L., Li J., Chen X.X. 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. PMID: 33937364; PMCID: PMC8079636. https://doi.org/10.3389/fcvm.2021.665038
  8. Soquet J., Barron D.J., d’Udekem Y. A Review of the Management of Pulmonary Atresia, Ventricular Septal Defect, and Major Aortopulmonary Collateral Arteries. Ann Thorac Surg. PMID: 30831109. 2019;108(2):601-612. https://doi.org/10.1016/j.athoracsur.2019.01.046
  9. Haydin S., Genç S.B., Ozturk E., Yıldız O., Gunes M., Tanidir I.C., Guzeltas A. 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):445-451. PMID: 32864922; PMCID: PMC7454616. https://doi.org/10.21470/1678-9741-2019-0055
  10. Elhedai H., Mohamed M., Mohammed S.S.S., Mustafa K.H.H., Seedahmed M.H.A., Mohamedahmed A.Y.Y. Comparison of staged repair versus single-stage complete repair for pulmonary atresia with ventricular septal defect: A systematic review and meta-analysis. Indian J Thorac Cardiovasc Surg. 2022;38(1):5-16. PMID: 34898872; PMCID: PMC8630289. https://doi.org/10.1007/s12055-021-01296-w
  11. Mainwaring R.D., Patrick W.L., Hanley F.L. Surgical Management of Pulmonary Atresia With Ventricular Septal Defect and Major Aortopulmonary Collateral Arteries: Part I – Anatomy, Physiology, and Palliative Procedures. Oper Tech Thorac Cardiovasc Surg. 2019;24(1):38-55. https://doi.org/10.1053/j.optechstcvs.2019.07.003
  12. 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 Diagn. 2019;39(12):1047-1053. PMID: 31351012. https://doi.org/10.1002/pd.5538
  13. Nakamura Y., Burkhart H.M. Staged unifocalization revisited. J Card Surg. 2022;37(4):967-968. PMID: 35137983. https://doi.org/10.1111/jocs.16296
  14. Onalan M.A., Cicek M., Rum M., Yurdakok O., Ozdemir F., Korun O., Altin H. F., Erdem H., Yilmaz E.H., Aydemir N.A., Sasmazel A. Unifocalization with pericardial roll technique in pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries. J Card Surg. 2022;37(9):2642-2650. PMID: 35661255. https://doi.org/10.1111/jocs.16656
  15. Ishidou M., Ota K., Kanno K., Murata M., Hirose K., Nagato H., Sakamoto K., Ikai A. Complete repair with unifocalization of the only unilateral lung. Gen Thorac Cardiovasc Surg. 2020;68(5):538-541. PMID: 30875003. https://doi.org/10.1007/s11748-019-01109-3
  16. Bhende V.V., Majmudar H.P., Sharma T.S., Rangwala V., Patel V.B., Kumar A., Panesar G., Pathan S.R., Mankad S.P. Concomitant Single-Stage Unifocalization and Cavopulmonary Anastomosis (Glenn Shunt) in an Adolescent Patient With Univentricular Physiology and Major Aortopulmonary Collateral Arteries: A Technically Challenging Case. Cureus. 2021;13(12):e20260. PMID: 34909352; PMCID: PMC8653758. https://doi.org/10.7759/cureus.20260
  17. Свободов А.А., Туманян М.Р., Ким А.И., Левченко Е.Г., Эргашов А.Ю., Гуласарян Р.С., Горбань Д.А., Макаренко М.В., Голухова Е.З. Многоэтапное лечение новорожденных и детей первого года жизни с синдромом гипоплазии левых отделов сердца: новый подход к оценке состояния в стационарном и межстадийном периодах (серия клинических случаев). Патология кровообращения и кардиохирургия. 2024;28(2):21-29.https://doi.org/10.21688/1681-3472-2024-2-21-29 Svobodov A.A., Tumanyan M.R., Kim A.I., Levchenko E.G., Ergashov A.Yu., Gulasaryan R.S., Gorban D.A., Makarenko M.V., Golukhova E.Z. Multistage treatment of newborns and infants with hypoplastic left heart syndrome: a new approach to the condition assessment throughout the hospital and interstage periods (a case series). Patologiya krovoobrashcheniya i kardiokhirurgiya = Circulation Pathology and Cardiac Surgery. 2024;28(2):21-29. (In Russ.) https://doi.org/10.21688/1681-3472-2024-2-21-29
  18. Синельников Ю.С., Арутюнян В.Б., Породиков А.А., Биянов А.Н., Туктамышев В.С., Шмурак М.И., Хайрулин А.Р., Кучумов А.Г. Применение математического моделирования для оценки результатов формирования системно-легочных анастомозов. Патология кровообращения и кардиохирургия. 2020;24(3):45- 61. http://dx.doi.org/10.21688/1681-3472-2020-3-45-61 Sinelnikov Yu.S., Arutunyan V.B., Porodikov A.A., Biyanov A.N., Tuktamyshev V.S., Shmurak M.I., Khairulin A.R., Kuchumov A.G. Application of mathematical modelling for the evaluation of the results of systemiс-pulmonary shunts formation. Patologiya krovoobrashcheniya i kardiokhirurgiya = Circulation Pathology and Cardiac Surgery. 2020;24(3):45-61. (In Russ.) http://dx.doi.org/10.21688/1681-3472-2020-3-45-61
  19. Войтов А.В., Омельченко А.Ю., Горбатых Ю.Н., Архипов А.Н., Сойнов И.А., Кулябин Ю.Ю., Ничай Н.Р., Горбатых А.В., Зубрицкий А.В., Богачев-Прокофьев А.В., Чернявский А.М. Сравнительный анализ различных паллиативных вмешательств у пациентов с атрезией легочной артерии и дефектом межжелудочковой перегородки. Кардиология и сердечно-сосудистая хирургия. 2020;13(6):517523. https://doi.org/10.17116/kardio202013061517 Voitov A.V., Omelchenko A.Yu., Gorbatykh Yu.N., Arkhipov A.N., Soynov I.A., Kulyabin Yu.Yu., Nichai N.R., Gorbatykh A.V., Zubritskiy A.V., Bogachev-Prokofiev A.V., Chernyavskiy A.M. Comparative analysis of different palliative procedures in patients with pulmonary atresia and ventricular septal defect. Russian Journal of Cardiology and Cardiovascular Surgery. 2020;13(6):517523. (In Russ.) https://doi.org/10.17116/kardio202013061517
  20. Zou M.H., Ma L., Cui Y.Q., Wang H.Z., Li W.L., Li J., Chen X.X. 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
  21. Hoashi T., Yazaki S., Kagisaki K., Kitano M., Shimada M., Shiraishi I., Ichikawa H. Importance of multidisciplinary management for pulmonary atresia, ventricular septal defect, major aorto-pulmonary collateral arteries and completely absent central pulmonary arteries. Gen Thorac Cardiovasc Surg. 2017;65(6):337-342. PMID: 28260150. https://doi.org/10.1007/s11748-017-0765-1
  22. Garg A., Sharma R. Ventricular septal defect with pulmonary atresia: approaches, results, prognosticators and current status. Indian J Thorac Cardiovasc Surg. 2022;38(1):28-37. PMID: 34898873; PMCID: PMC8630160. https://doi.org/10.1007/s12055-020-01133-6
  23. d’Udekem Y. Rehabilitation of Pulmonary Arteries in Pulmonary Atresia, VSD and Mapcas. Oper Tech Thorac Cardiovasc Surg. 2019;24(2):121-132. https://doi.org/10.1053/j.optechstcvs.2019.05.004
  24. Macalister S.J., Buratto E., Naimo P.S., Ye X.T., Fulkoski N., Weintraub R.G., Brizard C.P., Konstantinov I.E. Long-term Outcomes of Staged Complete Repair of Pulmonary Atresia With Ventricular Septal Defect. Ann Thorac Surg. 2023;115(2):445-451. PMID: 36155772. https://doi.org/10.1016/j.athoracsur.2022.09.022
  25. Malhotra S.P., Hanley F.L. Surgical management of pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals: a protocol-based approach. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 2009:145-51. PMID: 19349030. https://doi.org/10.1053/j.pcsu.2009.01.017
  26. Mainwaring R.D. Midline unifocalization for pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries. J Thorac Dis. 2020;12(3):1263-1273. PMID: 32274207; PMCID: PMC7139044. https://doi.org/10.21037/jtd.2019.10.44
  27. Watanabe N., Mainwaring R.D., Reddy V.M., Palmon M., Hanley F.L. Early complete repair of pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals. Ann Thorac Surg. 2014;97(3):909-15. PMID: 24480261. https://doi.org/10.1016/j.athoracsur.2013.10.115
  28. Trezzi M., Cetrano E., Albanese S.B., Borro L., Secinaro A., Carotti A. The Modern Surgical Approach to Pulmonary Atresia with Ventricular Septal Defect and Major Aortopulmonary Collateral Arteries. Children (Basel). 2022; 9(4):515. PMID: 35455558; PMCID: PMC9030555. https://doi.org/10.3390/children9040515