EXPERIENCE OF PERFORMING BIDIRECTIONAL CAVAPULMONARY ANASTOMOSIS IN THE SURGICAL TREATMENT OF A SINGLE VENTRICLE OF THE HEART: TACTICS AND OPTIMAL EXECUTION TIME

Dziuryi I. V., Truba Ia. P., Imanov E., Plyska O. I., Lazoryshynets V. V.

CLINICAL AND EXPERIMENTAL MEDICINE

Scentific article

The average birth rate of patients with CHD ranges from 7 to 9 cases per 1000 live births. Almost 8% of patients with CHD are diagnosed with anatomical variants, which leads to the functional physiology of SV. In 1984, Anderson et. al. coined the term univentricular atrioventricular junction to describe hearts in which both inlets (open or not) point predominantly to one dominant ventricle. The aim. To evaluate the perioperative characteristics of patients with a single ventricle of the heart, immediate and long-term results after bidirectional cavapulmonary anastomosis. Materials and methods. In the period from 1996 to 2022 at the National Amosov Institute of Cardiovascular Surgery of the National Academy of Medical Sciences of Ukraine an intermediate stage of hemodynamic correction was performed in 104 patients with a single heart ventricle. There were 62 (60%) male patients, 42 (40%) female patients. The median age of patients at the time of surgery was 36 months. [3; 420]. All patients were divided into three age groups: I-group up to 2.5 years 44 patients (42%), II – group from 2.5 to 5 years 30 patients (29%), III – group older than 5 years 30 patients (29%). Results. Death-rate among all operated patients was 1.9%. In the early postoperative period, there was 1 (2.3%) lethal case among patients of group I, and 1 (3.3%) lethal case among patients of group III. Death-rate in the II group was zero (0%). Patients of the II group had the shortest average duration of the operation with a median of 230 minutes [120; 300], duration of artificial blood circulation 68 min [28; 120], and the aortic clamping time is 5 min [2; 45]. The significantly lower, statistically reliable duration of mechanical ventilation of 3 hours pays attention. [1; 42], stay in the ICU for 76 hours. [42; 192], stay in the hospital for 19 days [10; 99], the average dose of sympathomimetic support is 5 μkg/kg/hour [1.5; 15] and its duration is 48 hours. [5; 120], the total duration of exudation is 48 hours. [4; 144] in patients of the II age group in compare to other groups. An uncomplicated course of the early postoperative period was observed in 76 (73%) patients. Other 28 (27%) patients of all groups: Group I 11 (10%) patients – 15 complications, Group II 5(5%) patients – 7 complications, Group III 12 (11%) patients – 14 complications regarding to groups. A significantly lower average indicator of systemic saturation at discharge in patients of the III group is 82% [74; 92] in relation to children of the I group, 84% [75; 97] and II – groups 86% [70; 94] clearly shows a decrease in the contribution of the SVC flow to the systemic blood flow depending on the age of the patient. The average observation period is 42 months. [5; 180], the final stage of hemodynamic correction was examined and performed in 85 (82%) patients. There were no lethal cases after the final stage of hemodynamic correction. Conclusions. The contribution of the flow of the superior vena cava to the systemic blood flow directly depends on the age of the patient, therefore the clinical effect of performing BCPA is much better when the operation is performed in the neonates and toddlers, and a complex treatment approach in patients with a single ventricle of the heart gives good immediate and long-term results in all stages of treatment.

congenital heart disease,single heart ventricle,reduced pulmonary blood flow,bidirectional cavapulmonary anastomosis,hemodynamic correction.

1. Hoffman JI, Kaplan S. The incidence of congenital heart disease. J Am Coll Cardiol. 2002 Jun 19;39(12):1890-900. DOI: 10.1016/s0735- 1097(02)01886-7.
2. O’Leary PW. Prevalence, clinical presentation, and natural history of patients with single ventricle. Prog Pediatr Cardiol. 2002;16:31-38.
3. Harvey W. Exercitatio anatomica de motu cordis et sanguinis in animalibus. Thomas; 1970. 126 p.
4. Florencia-Heredia M. Ventrículo único. Cirugía de Glenn y Fontan. Rev Latinoam Tecnol Extracorp. 2007;14:7-25.
5. Anderson RH, Shirali G. Sequential segmental analysis. Ann Pediatr Cardiol. 2009;2:24-35. DOI: 10.4103/0974-2069.52803.
6. Jacobs ML, Anderson RH. Nomenclature of the functionally univentricular heart. Cardiol Young. 2006;16(1):3-8. DOI: 10.1017/ S104795110500226X.
7. Sharma R. The bidirectional Glenn shunt for univentricular hearts. Indian J Thorac Cardiovasc Surg. 2018;34(4):453-456. DOI: https://doi. org/ 10.1007/ s12055-018-0653-z.
8. Edelson JB, Ravishankar C, Griffis H, Zhang X, Faerber J, Gardner MM, et al. A Comparison of Bidirectional Glenn vs. Hemi-Fontan Procedure: An Analysis of the Single Ventricle Reconstruction Trial Public Use Dataset. Pediatr Cardiol. 2020;41(6):1166-72. DOI: https:// doi.org/10.1007/s00246-020-02371-6.
9. Talwar S, Siddharth B, Choudhary SK, Airan B. One and half ventricle repair: rationale, indications, and results. Indian J Thorac Cardiovasc Surg. 2018;34(3):370-80. DOI: https://doi.org/10.1007/s12055-017-0628-5.
10. Chen LJ, Zhang YQ, Tong ZR, Sun AM. Evaluation of the anatomic and hemodynamic abnormalities in tricuspid atresia before and after surgery using computational fluid dynamics. Medicine. 2018;97:2e9510.
11. Carlon C, Mondini P, de Marchi R. Surgical treatment of some cardiovascular diseases. J Int Coll Surg. 1951;16(1):1-11.
12. Haller J, Adkins J, Worthington M, Rauenhorst J. Experimental studies on permanent bypass of the right heart. Surgery. 1966;59(6):1128- 1132.
13. Fontan F, Baudet E. Surgical repair of tricuspid atresia. Thorax. 1971;26: 240-8., 99.
14. Salim MA, DiSessa TG, Arheart KL, Alpert BS. Contribution of superior vena caval flow to total cardiac output in children. A Doppler echocardiographic study. Circulation. 1995;92(7):1860-1865. DOI: https://doi.org /10.1161/01. cir.92.7.1860.
15. Martin BJ, De Villiers Jonker I, Joffe AR, Bond GY, Acton BV, Ross DB, et al. Hypoplastic left heart syndrome is not associated with worse clinical or neurodevelopmental outcomes than other cardiac pathologies after the Norwood-Sano operation. Pediatr Cardiol. 2017;38:922- 931.
16. Liu MY, Zielonka B, Snarr BS, Zhang X, Gaynor JW, Rychik J. Longitudinal assessment of outcome from prenatal diagnosis through Fontan operation for over 500 fetuses with single ventricle-type congenital heart disease: The Philadelphia Fetus-to-Fontan Cohort Study. J Am Heart Assoc. 2018;7:e009145.
17. Spector LG, Menk JS, Knight JH, McCracken C, Thomas AS, Vinocur JM, et al. Trends in long-term mortality after congenital heart surgery. J Am Coll Cardiol. 2018;71:2434-2446.
18. Minocha P, Phoon C. Tricuspid atresia [Internet]. Treasure Island (FL): StatPearls Publishing; 2022. Available from: https://www.ncbi.nlm. nih. gov/books/NBK554495/#article-30595.s3.
19. King G, Ayer J, Celermajer D, Zentner D, Justo R, Disney P, et al. Atrioventricular valve failure in Fontan palliation. J Am Coll Cardiol. 2019;73:810-822.
20. Holst KA, Dearani JA, Said S, Pike RB, Connolly HM, Cannon BC, et al. Improving Results of Surgery for Ebstein Anomaly: Where Are We After 235 Cone Repairs? Ann Thorac Surg. 2018 Jan;105(1):160-8. DOI: https://doi. org/10.1016/j.athoracsur.2017.09.058.
21. Mat Bah MN, Sapian MH, Jamil MT, Abdullah N, Alias EY, Zahari N. The birth prevalence, severity, and temporal trends of congenital heart disease in the middle-income country: a population-based study. Congenit Heart Dis. 2018;13(6):1012-1027. ‘
22. Liu Y, Chen S, Zühlke L, Black GC, Choy MK, Li N, et al. Global birth prevalence of congenital heart defects 1970–2017: updated systematic review and meta-analysis of 260 studies. Int J Epidemiol. 2019;48(2):455-463.
23. Rao PS. Pediatric tricuspid atresia clinical presentation: history, physical examination [Internet]. 2017. Available from: https://emedicine. medscape. com/article/900832-clinical#b2.
24. Nozari A, Aghaei-Moghadam E, Zeinaloo A, Alavi A, Ghasemi Firouzabdi S, Minaee S, et al. A Pathogenic Homozygous Mutation in The Pleckstrin Homology Domain of RASA1 Is Responsible for Familial Tricuspid Atresia in An Iranian Consanguineous Family. Cell J. 2019;21(1):70-7. DOI: https://doi.org/10.22074/cellj.2019.5734.

«Bulletin of problems biology and medicine» Issue 4 (167), 2022 year, 138-150 pages, index UDK 616.124-007.2-053.2-089.841

10.29254/2077-4214-2022-4-167-138-150