Vol. 26 No. 2 (2022)
CASE REPORTS

First experience of staged treatment of an infant with heterotaxy syndrome with total anomalous pulmonary vein drainage combined with a single ventricular heart and pulmonary atresia

PDF (Русский)
  • K.N. Kuatbekov,
  • A.V. Mishin,
  • A.D. Sepbayeva,
  • G.S. Nigay,
  • D.M. Kanzhigalin
K.N. Kuatbekov
Modern medicine center “Mediterra”, Institute of Surgery, Almaty
A.V. Mishin
Modern medicine center “Mediterra”, Institute of Surgery, Almaty
Bio
A.D. Sepbayeva
Perinatology and Pediatric Cardiac Surgery Center, Almaty
G.S. Nigay
Perinatology and Pediatric Cardiac Surgery Center, Almaty
D.M. Kanzhigalin
National Scientific Medical Center, Nur-Sultan
Бесшовная пластика тотального аномального дренажа легочных вен, расширяющая пластика бифуркации легочной артерии, системно-легочный анастомоз
DOI: https://doi.org/10.21688/1681-3472-2022-2-73-78

Published 2022-06-30

Keywords

  • case report,
  • pulmonary atresia,
  • total anomalous pulmonary venous return

How to Cite

Kuatbekov, K., Mishin, A., Sepbayeva, A., Nigay, G., & Kanzhigalin, D. (2022). First experience of staged treatment of an infant with heterotaxy syndrome with total anomalous pulmonary vein drainage combined with a single ventricular heart and pulmonary atresia. Patologiya Krovoobrashcheniya I Kardiokhirurgiya, 26(2), 73–78. https://doi.org/10.21688/1681-3472-2022-2-73-78

Copyright (c) 2022 Kuatbekov K.N., Mishin A.V., Sepbayeva A.D., Nigay G.S., Kanzhigalin D.M.

Creative Commons License

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

Abstract

The combination of monoventricular congenital heart disease with total anomalous pulmonary vein drainage and pulmonary atresia, is a rare pathology and characterized by an extremely severe course with the highest mortality. In the presented work we described a clinical case of the first and successful experience of two-stage treatment of an infant with total anomalous pulmonary vein drainage combined with single ventricular heart disease and pulmonary atresia in the Republic of Kazakhstan. For total anomalous pulmonary vein drainage correction we used a sutureless technique — primary sutureless repair, which is used in our center as a primary one. Due to severe anatomy of the defect, we used unusual area of the distal end of the central systemic-pulmonary artery anastomoses during primary surgery at the mouth of the severed patent ductus arteriosus in the area of pulmonary artery bifurcation, where it is associated with high surgical risks. Taking into account the presence of an additional obstacle to pulmonary blood flow in the form of left branch pulmonary artery orifice stenosis, we performed one-stage dilatation plasty of the pulmonary artery branch bifurcation with autopericardium. During the repeated surgery we faced such a serious complication as aortic damage for the first time, thanks to emergency actions it did not turn out to be fatal. We also managed to perform the most preferable dilation of hypoplastic branches of the pulmonary artery by plasty with the own tissues of the superior vena cava during the formation of Glenn-anastomosis.

Received 29 November 2021. Revised 21 December 2021. Accepted 22 December 2021.

Informed consent: Informed consent was obtained from the patient's mother for the publication and use of the patient's medical data for scientific purposes.

Funding: The study did not have sponsorship.

Conflict of interest: Authors declare no conflict of interest.

Contribution of the authors
Literature review: A.V. Mishin
Drafting the article: A.V. Mishin
Critical revision of the article: K.N. Kuatbekov, A.D. Sepbayeva
Surgical treatment: K.N. Kuatbekov, A.V. Mishin, A.D. Sepbayeva, G.S. Nigay, D.M. Kanzhigalin
Final approval of the version to be published: K.N. Kuatbekov, A.V. Mishin, A.D. Sepbayeva, G.S. Nigay, D.M. Kanzhigalin

PDF (Русский)

References

  1. Yong M.S., Zhu M.Z.L., d’Udekem Y., Konstantinov I.E. Outcomes of total anomalous pulmonary venous drainage repair in neonates with univentricular circulation. Interact Cardiovasc Thorac Surg. 2018;27(5):756-760. PMID: 29757371. https://doi.org/10.1093/icvts/ivy159
  2. Okamoto T., Nakano T., Goda M., Oda S., Kado H. Outcomes of systemic-to-pulmonary artery shunt for single ventricular heart with extracardiac total anomalous pulmonary venous connection. Gen Thorac Cardiovasc Surg. 2021;69(4):646-653. PMID: 32886275. https://doi.org/10.1007/s11748-020-01474-4
  3. Sugano M., Murata M., Ide Y., Ito H., Kanno K., Imai K., Ishidou M., Fukuba R., Sakamoto K. Midterm results and risk factors of functional single ventricles with extracardiac total anomalous pulmonary venous connection. Gen Thorac Cardiovasc Surg. 2019;67(11):941-948. PMID: 31134530. https://doi.org/10.1007/s11748-019-01141-3
  4. Alsoufi B., McCracken C., Schlosser B., Sachdeva R., Well A., Kogon B., Border W., Kanter K. Outcomes of multistage palliation of infants with functional single ventricle and heterotaxy syndrome. J Thorac Cardiovasc Surg. 2016;151(5):1369-1377.e2. PMID: 27085618. https://doi.org/10.1016/j.jtcvs.2016.01.054
  5. Khan M.S., Bryant R. 3rd, Kim S.H., Hill K.D., Jacobs J.P., Jacobs M.L., Pasquali S.K., Morales D.L.S. Contemporary outcomes of surgical repair of total anomalous pulmonary venous connection in patients with heterotaxy syndrome. Ann Thorac Surg. 2015;99(6):2134-2139; discussion 2139-2140. PMID: 25912749. https://doi.org/10.1016/j.athoracsur.2015.02.035
  6. Теплов П.В., Титов А.М., Миллер А.Ю., Сакович В.А., Дробот Д.Б., Морозов А.А. Сравнение прямого анастомоза и «бесшовной техники» при радикальной коррекции тотального аномального дренажа легочных вен. Клиническая и экспериментальная хирургия. Журнал имени академика Б.В. Петровского. 2021;9(3 Suppl):33-36. https://doi.org/10.33029/2308-1198-2021-9-3suppl-33-36 Teplov P.V., Titov A.M., Miller A.Yu., Sakovich V.A., Drobot D.B., Morozov A.A. Comparison of direct anastomosis and "sutureless" technique for radical correction of total anomaly drainage of pulmonary veins. Clinical and Experimental Surgery. Petrovsky Journal. 2021;9(3 Suppl):33-36. (In Russ.) https://doi.org/10.33029/2308-1198-2021-9-3suppl-33-36
  7. Gözgeç E., Kantarci M., Guven F., Ogul H., Ceviz N., Eren S. Determination of anomalous pulmonary venous return with high-pitch low-dose computed tomography in paediatric patients. Folia Morphol (Warsz). 2021;80(2):336-343. PMID: 32459365. https://doi.org/10.5603/FM.a2020.0054
  8. Xiong L., Patel M.D., Biliciler-Denktas G., Dodge-Khatami A., Salazar J., Adebo D.A. Perioperative anomalous pulmonary venous return evaluation with low-dose cardiac computed tomography. World J Pediatr Congenit Heart Surg. 2021;12(4):529-534. PMID: 34278867. https://doi.org/10.1177/21501351211010115