Bidirectional Cavapulmonary Anastomosis as a Stage of Hemodynamic Correction of Hypoplastic Right Heart Syndrome
Abstract
Hypoplastic right heart syndrome (HRHS) is a rare cyanotic congenital heart disease with a wide spectrum of cardiac anomalies characterized by underdevelopment of the right heart. Survival and type of surgery in these patients directly correlate with structural anatomy and circulatory physiology.
The aim. To evaluate perioperative characteristics in patients with HRHS, immediate and long-term results after bidirectional cavapulmonary anastomosis.
Materials and methods. In the period from 1996 to 2022, 80 patients with HRHS underwent hemodynamic correction at the National Amosov Institute of Cardiovascular Surgery. All the patients were divided into three age groups: group I (n = 18) with patients who underwent hemodynamic correction under the age of 1 year, group II (n = 33) including patients who underwent surgery between the ages of 12 and 36 months, and group III (n = 29) comprised of those operated at the age older than 3 years.
Results. In the early postoperative period, 5 (6%) deaths were reported among the patients of three groups: 3 in group I and 2 in group III. There were no deaths among patients of the group II. Median durations of mechanical ventilation, total exudation, hourly and total sympathomimetic support, hospital stay were significantly lower in group II in contrast to the comparison groups. An uncomplicated course of the early postoperative period was observed in 59 (74%) patients.
Conclusions. In patients with HRHS, the determination of treatment tactics requires a careful approach, especially in newborns and infants. The implementation of hemodynamic correction shows significantly better results in early childhood.
References
- Dimopoulos A, Sicko RJ, Kay DM, Rigler SL, Druschel CM, Caggana M, et al. Rare copy number variants in a population-based investigation of hypoplastic right heart syndrome. Birth Defects Res. 2017;109(1):8-15. https://doi.org/10.1002/bdra.23586
- De Stefano D, Li P, Xiang B, Hui P, Zambrano E. Pulmonary atresia with intact ventricular septum (PA-IVS) in monozygotic twins. Am J Med Genet A. 2008;146A(4):525-528. https://doi.org/10.1002/ajmg.a.32160
- Minocha PK, Horenstein MS, Phoon C. Tricuspid Atresia. [Updated 2024 Jan 26]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2024 Jan. Available from: https://www.ncbi.nlm.nih.gov/books/NBK554495/
- 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. https://doi.org/10.1093/ije/dyz009
- 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):1166-1172. https://doi.org/10.1007/s00246-020-02371-6
- Cabrelle G, Castaldi B, Vedovelli L, Gregori D, Vida VL, Padalino MA. Long-term experience with the one-and-a-half ventricle repair for simple and complex congenital heart defects. Eur J Cardiothorac Surg. 2021;59(1):244-252. https://doi.org/10.1093/ejcts/ezaa289
- Billingsley AM, Laks H, Boyce SW, George B, Santulli T, Williams RG. Definitive repair in patients with pulmonary atresia and intact ventricular septum. J Thorac Cardiovasc Surg. 1989;97(5):746-754
- Prasad D, Romanowicz J, Banka P, Beroukhim R, Ghelani SJ, Emani S, et al. Cardiac magnetic resonance parameters associated with successful conversion from a single ventricular to a one-and-a-half or biventricular circulation in patients with a hypoplastic right ventricle. J Cardiovasc Magn Reson. 2023 Sep 28;25(1):51. https://doi.org/10.1186/s12968-023-00965-6
- Carlon CA, Mondini PG, DE Marchi R. Su una nuova anastomosi vasale per le terapia chirurgica di alcuni vizi cardiovascolari [A new vascular anastomosis for the surgical therapy of various cardiovascular defects]. G Ital Chir. 1950 Nov;6(11):760-774. Italian.
- Ma K, Qi L, Hua Z, Yang K, Zhang H, Li S, et al. Effectiveness of Bidirectional Glenn Shunt Placement for Palliation in Complex Congenitally Corrected Transposed Great Arteries. Tex Heart Inst J. 2020;47(1):15-22. https://doi.org/10.14503/THIJ-17-6555
- 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. https://doi.org/10.1161/01.cir.92.7.1860
- Salim MA, Case CL, Sade RM, Watson DC, Alpert BS, DiSessa TG. Pulmonary/systemic flow ratio in children after cavopulmonary anastomosis. J Am Coll Cardiol. 1995;25(3):735-738. https://doi.org/10.1016/0735-1097(94)00441-R
- 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):70-77. https://doi.org/10.22074/cellj.2019.5734
- Possner M, Gensini FJ, Mauchley DC, Krieger EV, Steinberg ZL. Ebstein’s Anomaly of the Tricuspid Valve: an Overview of Pathology and Management. Curr Cardiol Rep. 2020 Oct 9;22(12):157. https://doi.org/10.1007/s11886-020-01412-z
- Prasanna A, Tan CW, Anastasopulos A, Beroukhim RS, Emani SM. One and One-Half Ventricle Repair: Role for Restricting Antegrade Pulmonary Blood Flow. Ann Thorac Surg. 2022;114(1):176-183. https://doi.org/10.1016/j.athoracsur.2021.04.058
- Tariq M, Zahid I, Hashmi S, Amanullah M, Shahabuddin S. The Glenn procedure: Clinical outcomes in patients with congenital heart disease in pakistan. Ann Card Anaesth. 2021;24(1):30-35. https://doi.org/10.4103/aca.ACA_85_19
- Sarris GE, Giannopoulos NM, Tsoutsinos AJ, Chatzis AK, Kirvassilis G, Brawn WJ, et al.; European Congenital Heart Surgeons Association. Results of surgery for Ebstein anomaly: A multicenter study from the European Congenital Heart Surgeons Association. J Thorac Cardiovasc Surg. 2006;132(1):50-57. https://doi.org/10.1016/j.jtcvs.2005.10.062