Experience of Surgical Treatment of Complex Forms of Ebstein’s Anomaly through the Method of Hemodynamic Correction

DOI: https://doi.org/10.30702/ujcvs/23.31(02)/DT031-2533
Keywords: congenital heart defects, tricuspid valve, atrialization of the right ventricle, tricuspid insufficiency, one and a half ventricle repair, bidirectional cavapulmonary anastomosis

Abstract

Ebstein’s anomaly (EA) is a rare and complex congenital heart disease, with a prevalence of 4 to 7 cases per 100,000 live births. At this stage of the development of EA surgery, significant progress has been made in our understanding and treatment of EA, but the question of the tactics of treating complex forms, especially in the period of infancy and early childhood, remains open. Due to significant phenotypic heterogeneity, the treatment of EA varies greatly from patient to patient and depends on many factors that require a comprehensive approach. The choice of the method of surgical correction directly depends on the anatomical features: the degree of dysplasia, rotation and displacement of the tricuspid valve, the degree of atrialization of the right ventricle, the combination with other congenital heart diseases, and the severity of hemodynamic disorders.

The aim. To evaluate perioperative characteristics in patients with complex forms of EA, immediate and long-term results after hemodynamic correction.

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, one and a half ventricle repair was performed in 18 patients with complex forms of EA. There were 12 (67%) male patients, 6 (33%) female patients. The median age of the patients at the time of surgery was 30 months [3.5; 312]. The main diagnostic method in determining the defect and assessing the immediate and remote results was echocardiography and probing of the heart cavities. In order to qualitatively evaluate the perioperative, immediate and long-term results to determine the optimal tactics and terms of surgical intervention, prevention of possible complications, all the patients were divided into two age groups: I group up to 2.5 years including 9 patients (50%), II group older than 2.5 years including 9 patients (50%).

Results. In the postoperative period, 2 (11%) deaths were noted in both groups. In all the deceased patients, the cause of death was acute heart failure and acute respiratory failure. The analysis of the postoperative period showed normal results in such a cohort of highly complex patients of both groups, but a more stable course was noted among patients of the I group, as indicated by statistically significant values for the duration of mechanical ventilation, stay in the intensive care unit, stay in the hospital, the average dose of sympathomimetic support and its duration, the total duration of exudation in the comparison groups.

An uncomplicated course of the early postoperative period was observed in 8 (44%) patients. The remaining 10 (56%) patients of both groups had: I group, 3 (33%) patients: 7 complications; II group, 5 (55%) patients: 13 complications. In 5 patients (2 patients in I group and 3 patients in II group), attention-grabbing acute heart failure was observed, which required high doses and long-term sympathomimetic support, and in 2 patients caused death. The average observation period was 45 months [6; 190]. No deaths were observed during the observation period among 13 (72%) patients of both groups, contact was lost with 3 (17%) patients. There were no violations in the functioning of the right ventricle, but the recovery of the systolic function of the left ventricle occurred only in 8 (44%) patients.

Tricuspid insufficiency was minimal in 5 (28%) patients, moderate in 6 (33%), severe in 2 (11%) patients.

Conclusions. Determining the tactics of treatment of complex forms of EA is a serious problem in patients of various ages, especially in newborns and infants, due to pronounced dilatation and systolic dysfunction of the left ventricle, a sharp decrease in systemic output due to changes in the geometry of the left ventricle. The technique of hemiventricular correction shows very promising results and may become the technique of choice for patients with severe forms of EA and pronounced right ventricular insufficiency.

References

  1. Boyle B, Garne E, Loane M, Addor MC, Arriola L, Cavero-Carbonell C, et al. The changing epidemiology of Ebstein’s anomaly and its relationship with maternal mental health conditions: a European registry-based study. Cardiol Young. 2017;27(4):677-685. https://doi.org/10.1017/S1047951116001025
  2. 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;22(12):157. https://doi.org/10.1007/s11886-020-01412-z
  3. Torigoe F, Ishida H, Ishii Y, Ishii R, Narita J, Kawazu Y, et al. Fetal echocardiographic prediction score for perinatal mortality in tricuspid valve dysplasia and Ebstein’s anomaly. Ultrasound Obstet Gynecol. 2020;55(2):226-232. https://doi.org/10.1002/uog.20302
  4. Attenhofer Jost CH, Connolly HM, Dearani JA, Edwards WD, Danielson GK. Ebstein’s Anomaly. Circulation. 2007;115(2):277-285. https://doi.org/10.1161/CIRCULATIONAHA.106.619338
  5. Carpentier A, Chauvaud S, Macé L, Relland J, Mihaileanu S, Marino JP, et al. A new reconstructive operation for Ebstein’s anomaly of the tricuspid valve. J Thorac Cardiovasc Surg. 1988;96(1):92-101.
  6. Celermajer DS, Bull C, Till JA, Cullen S, Vassillikos VP, Sullivan ID, et al. Ebstein’s anomaly: Presentation and outcome from fetus to adult. J Am Coll Cardiol. 1994 Jan;23(1):170-176. https://doi.org/10.1016/0735-1097(94)90516-9
  7. Dearani JA, Danielson GK. Congenital Heart Surgery Nomenclature and Database Project: Ebstein’s anomaly and tricuspid valve disease. Ann Thorac Surg. 2000;69:S106-S117. https://doi.org/10.1016/s0003-4975(99)01265-5
  8. Pignatelli RH, Texter KM, Denfield SW, Grenier MA, Altman CA, Ayres NA, et al. LV Noncompaction in Ebstein’s Anomaly in Infants and Outcomes. JACC Cardiovasc Imaging. 2014;7(2):207-209. https://doi.org/10.1016/j.jcmg.2013.05.021
  9. Hassan A, Tan NY, Aung H, Connolly HM, Hodge DO, Vargas ER, et al. Outcomes of atrial arrhythmia radiofrequency catheter ablation in patients with Ebstein’s anomaly. Europace. 2018;20(3):535-540. https://doi.org/10.1093/europace/euw396. Erratum in: Europace. 2018 Mar 1;20(3):534.
  10. Stout KK, Daniels CJ, Aboulhosn JA, Bozkurt B, Broberg CS, Colman JM, et al. 2018 AHA/ACC Guideline for the Management of Adults With Congenital Heart Disease: Executive Summary: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines. Circulation. 2019;139(14):e637-e697. https://doi.org/10.1161/ CIR.0000000000000602. Erratum in: Circulation. 2019 Apr 2;139(14):e831-e832.
  11. Attenhofer Jost CH, Connolly HM, Scott CG, Burkhart HM, Warnes CA, Dearani JA. Outcome of Cardiac Surgery in Patients 50 Years of Age or Older With Ebstein Anomaly: Survival and Functional Improvement. J Am Coll Cardiol. 2012;59(23):2101-2106. https://doi.org/10.1016/j.jacc.2012.03.020
  12. Saran N, Dearani JA, Said SM, Greason KL, Pochettino A, Stulak JM, et al. Long-term outcomes of patients undergoing tricuspid valve surgery†. Eur J Cardiothorac Surg. 2019;56(5):950-958. https://doi.org/10.1093/ejcts/ezz081
  13. 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):370-380. https://doi.org/10.1007/s12055-017-0628-5
  14. 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 (Baltimore). 2018;97(2):e9510. https://doi.org/10.1097/MD.0000000000009510
  15. Knott-Craig CJ, Overholt ED, Ward KE, Ringewald JM, Baker SS, Razook JD. Repair of Ebstein’s anomaly in the symptomatic neonate: an evolution of technique with 7-year follow-up. Ann Thorac Surg. 2002;73(6):1786-1793. https://doi.org/10.1016/s0003-4975(02)03507-5
  16. 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
  17. Geerdink LM, Delhaas T, Helbing WA, du Marchie Sarvaas GJ, Heide HT, Rozendaal L, et al. Paediatric Ebstein’s anomaly: how clinical presentation predicts mortality. Arch Dis Child. 2018;103(9):859-863. https://doi.org/10.1136/archdischild-2017-313482
  18. Malhotra A, Agrawal V, Patel K, Shah M, Sharma K, Sharma P, et al. Ebstein’s Anomaly: “The One and a Half Ventricle Heart”. Braz J Cardiovasc Surg. 2018;33(4):353-361. https://doi.org/10.21470/1678-9741-2018-0100
Published
2023-06-26
How to Cite
1.
Dziuryi IV, Truba IP, Golovenko OS, Perepeka IA, Lazoryshynets VV. Experience of Surgical Treatment of Complex Forms of Ebstein’s Anomaly through the Method of Hemodynamic Correction. ujcvs [Internet]. 2023Jun.26 [cited 2024Dec.22];31(2):25-4. Available from: https://cvs.org.ua/index.php/ujcvs/article/view/561
Issue
Ukrainian Journal of Cardiovascular Surgery Vol 31 No 2 2023
Section
CONGENITAL HEART DEFECTS

Most read articles by the same author(s)

1 2 3 > >>