Actual Experience of One and a Half Ventricle Repair in Patients with Ebstein’s Anomaly

  • V. А. Khanenova Ukrainian Children’s Cardiac Center, Kyiv, Ukraine
  • O. V. Stogova Ukrainian Children’s Cardiac Center, Kyiv, Ukraine
  • A. Y. Hrytsaiuk Ukrainian Children’s Cardiac Center, Kyiv, Ukraine
  • Y. L. Kuzmenko Ukrainian Children’s Cardiac Center, Kyiv, Ukraine
  • M. S. Meshkova Ukrainian Children’s Cardiac Center, Kyiv, Ukraine
  • T. A. Yalynska Ukrainian Children’s Cardiac Center, Kyiv, Ukraine
  • R. M. Kostrakevych Ukrainian Children’s Cardiac Center, Kyiv, Ukraine
  • І. М. Yemets Ukrainian Children’s Cardiac Center, Kyiv, Ukraine
Keywords: Ebstein’s anomaly, one and a half ventricular repair, bidirectional cavopulmonary shunt, tricuspid insufficiency

Abstract

Introduction. Ebstein’s anomaly (EA) is a complex congenital heart defect involving mainly tricuspid valve (TV), right atrium and right ventricle (RV). Severe RV systolic dysfunction is associated with a high risk of surgical intervention for EA. Bidirectional cavopulmonary shunt (BCPS), which complements the restoration of TV competence, is an important step in one and a half ventricle repair.

The aim. To analyze actual experience of EA treatment through the method of one and a half ventricle repair.

Materials and methods. In the period from 1999 to 2018, one and a half ventricle repair of EA was performed in 17 patients at the UCCC. Preoperative examinations showed type “B” EA in 4 (23.5 %) patients, type “C” in 8 (47 %), type “D” in 5 (29.5 %) patients. BCPS was combined with different types of TV plasty in 14 (82.4 %) patients, with TV replacement in 3 (17.6 %) patients.

Results and discussion. Early postoperative mortality rate was 5.9 %; 1 (6.2 %) reoperation was performed. In the early postoperative period, the contractility of both ventricles was satisfactory, TV insufficiency was from mild to moderate. The average duration of the late postoperative period was 102.6 ± 58.8 months. Most patients maintained small to moderate TV insufficiency, and contractility of both ventricles was satisfactory. The long-term mortality rate was 12.5 %. Also, 3 (18.7 %) reoperations were performed.

Conclusions. One and a half ventricular repair of EA may be used for surgical treatment of patients with EA and RV severe dilatation and systolic dysfunction, acute decrease in cardiac output due to the changes in LV geometry and contraction as well as non-ideal correction of TV insufficiency.

References

  1. Zinkovsky MF. [Congenital heart disease]. Vozianov AF, editor. Кyiv; 2009. (in Russian).
  2. Attenhofer Jost CH, Connolly HM, Dearani JA, Edwards WD, Danielson GK. Ebstein’s anomaly. Circulation. 2007 Jan 16;115(2):277–85.
  3. 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 Sep;103(9):859–63. https://doi.org/10.1136/archdischild-2017-313482
  4. 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– 61. https://doi.org/10.21470/1678-9741-2018-0100
  5. Liu J, Qiu L, Zhu Z, Chen H, Hong H. Cone reconstruction of the tricuspid valve in Ebstein anomaly with or without one and a half ventricle repair. J Thorac Cardiovasc Surg. 2011 May;141(5):1178–83. https://doi.org/10.1016/j.jtcvs.2011.01.015
  6. Quinonez LG1, Dearani JA, Puga FJ, O’Leary PW, Driscoll DJ, Connolly HM, et al. Results of the 1.5-ventricle repair for Ebstein anomaly and the failing right ventricle. J Thorac Cardiovasc Surg. 2007 May;133(5):1303–10. https://doi.org/10.1016/j.jtcvs.2006.12.007
  7. 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. https://doi.org/10.1016/j.athoracsur.2017.09.058
  8. Raju V, Dearani JA, Burkhart HM, Grogan M, Phillips SD, Ammash N, et al. Right Ventricular Unloading for Heart Failure Related to Ebstein Malformation. Ann Thorac Surg. 2014 Jul;98(1):167–73. https://doi.org/10.1016/j.athoracsur.2014.03.00
Published
2019-05-22
How to Cite
KhanenovaV. А., Stogova, O. V., Hrytsaiuk, A. Y., Kuzmenko, Y. L., Meshkova, M. S., Yalynska, T. A., Kostrakevych, R. M., & YemetsІ. М. (2019). Actual Experience of One and a Half Ventricle Repair in Patients with Ebstein’s Anomaly. Ukrainian Journal of Cardiovascular Surgery, (2 (35), 64-68. https://doi.org/10.30702/ujcvs/19.3505/039061-068