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3, Приложение . 2021

Fifteen years of experience in surgical treatment of total anomalous pulmonary venous connection: from the first operations to the present time

Abstract

Background. Total anomalous pulmonary venous connection (TAPVC) is one of the critical cyanotic congenital heart diseases (CHD). Among all CHD TAPVC occurs from 1 to 3% of all cases. The mortality rate is 80% in the first year of life in case of the natural course of the disease. At the present stage of development of pediatric cardiac surgery, TAPVC is one of the congenital heart diseases that have a number of topical issues requiring attention, ranging from prenatal diagnosis of the defect to the choice of the optimal strategy for surgical treatment and prevention of complications in the long-term follow-up period. This retrospective study presents our fifteen-year experience of surgical treatment of TAPVC, the purpose of the study was to analyze the immediate and longterm results of surgical treatment of TAPVC.

Material and methods. Between 2005 and 2020 61 patients underwent radical correction of TAPVC. The median age at the time of surgery was 31 (1; 2201) days. The average body weight was 4.3± 2.6 kg. In order to identify differences in immediate and long-term results, two periods of surgical experience were identified (early before 2014 and late after 2014), patients were divided into two groups, in accordance with the identified periods.

Results. The hospital mortality rate according to our study was 1.6%. In the long-term follow-up, there were 5 (8%) deaths. In all cases, the cause of death was pulmonary vein obstruction (PV). In 3 patients, the stenosis was bilateral, with the involvement of all PVs in the pathological process; the patients were considered inoperable. In 2 cases, reoperation were performed for obstruction of the PV collector. The median time between radical correction of TAPVC and reoperation for PV obstruction was 109 (73; 138) days. Thus, all cases of restenosis were observed up to 4 months after radical correction of the defect. The actuarial freedom from reoperations after 4 months and the entire follow-up period in patients who underwent surgery for TAPVC was 91.7%.

Conclusion. Despite the decrease in hospital mortality rates, long-term mortality is still quite high. PV obstruction, which usually develops within 4 months after surgery, is one of the main causes of high mortality in the long-term follow-up period after TADLV correction. The occurrence of PV obstruction does not depend on the period of surgical experience. Careful dynamic monitoring of patients in the postoperative period is required for early detection of PV obstruction.

Keywords:total anomalous pulmonary venous connection, postoperative pulmonary vein stenosis

Funding. The study had no sponsor support.
Conflict of interest. The authors declare no conflict of interest.
For citation: Bdiskov M.V., Karakhalis N.B., Tkachenko I.A., Efimochkin G.A., Petshakovskiy P.J., Vanin O.A., Serova T.V., Коhtacheva O.V., Klycheva O.V., Shadrin A.K., Kovalchuk N.V., Morozov A.A. Fifteen years of experience in surgical treatment of total anomalous pulmonary venous connection: from the first operations to the present time. Clinical and Experimental Surgery. Petrovsky Journal. 2021; 9 (3). Supplement: 26-32. DOI: https://doi.org/10.33029/2308-1198-2021-9-3suppL-26-32 (in Russian)

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CHIEF EDITOR
CHIEF EDITOR
Sergey L. Dzemeshkevich
MD, Professor (Moscow, Russia)

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