Two-Stage Repair of Aortic Arch Hypoplasia Associated with Other Congenital Heart Defects in Newborns and Infants

Keywords: echocardiography, aortic arch segments, pressure gradient, left ventricular ejection fraction, treatment tactics

Abstract

Background. The optimal tactics of surgical treatment of aortic arch hypoplasia combined with other congenital heart defects remain controversial. In recent years, the leading clinics of the world prefer one-stage repair of such a combination, however, two-stage treatment is still used by cardiac surgeons and remains one of the variants of surgical tactics.

The aim. To analyze immediate and long-term results of a two-stage repair of aortic arch hypoplasia with other con-genital heart defects in newborns and infants.

Materials and methods. The study included 138 infants who were selected for a two-stage repair of aortic arch hy-poplasia with other congenital heart defects. There were 79 (57.2%) male and 59 (42.8%) female patients. The mean age of the patients was 1.4 ± 0.7 months, mean body weight was 4.1 ± 2.0 kg. The main method of the defect diagnosis and evaluation of immediate and long-term results was echocardiographic examination. The aortic arch segment was consid-ered hypoplastic if the Z-score deviation was less than –2.0.

Results. The hospital mortality rate was 5.8% (n = 8). Two patients (1.4%) had delayed sternal closure in the early postoperative period. As a result of severe myocardial failure, two patients underwent extracorporeal membrane oxy-genation (ECMO). According to echocardiography before discharge, the mean pressure gradient at the site of aortic arch repair was 16 ± 6 mmHg. There was one death in the follow-up period. In 16 (11.5%) patients, aortic arch restenosis developed in the postoperative period. In total, 20 re-interventions were performed: 11 endovascular balloon dilatations and 9 surgical re-interventions. In the early postoperative period, aortopexia was performed in two patients (1.4%) due to compression of the left main bronchus.

Conclusions. Two-stage repair of aortic arch hypoplasia with other congenital heart defects in newborns and infants is effective and safe treatment with good immediate and long-term results. This study shows that this surgical strategy can be effectively used in this complex pathology.

References

  1. Hoffman J, Kaplan S. The incidence of congenital heart disease. J Am Coll Cardiol. 2002 Jun 19;39(12):1890-1900. https://doi.org/10.1016/s0735-1097(02)01886-7.
  2. de Cruz E, Ivy D, Jaggers J. Pediatric and congenital Cardiology, cardiac surgery and intensive care. London: Springer-Verlag; 2014. 3572 p.
  3. Elgamal MA, McKenzie D, Fraser C. Aortic arch advancement: the optimal one-stage approach for surgical management of neonatal coarctation with arch hypoplasia. Ann Thorac Surg. 2002 Apr;73(4):1267-1272. https://doi.org/10.1016/s0003-4975(01)03622-0.
  4. St Louis JD, Harvey BA, Menk JS, O’Brien JE Jr, Kochilas LK. Mortality and operative management for patients undergoing repair of coarctation of the aorta: a retrospective review of the pediatric cardiac care consortium. World J Pediatr Congenit Heart Surg. 2015;6(3):431-437. https://doi.org/10.1177/2150135115590458.
  5. Wu Y, Li J, Wu C, Zhu J, He L, Feng C, Yang Y, Jin X. Diagnosis and Surgical Repair for Coarctation of the Aorta With Intracardiac Defects: A Single Center Experience Based on 93 Infants. Front Pediatr. 2020;8:49. https://doi.org/10.3389/fped.2020.00049.
  6. Gray WH, Wells WJ, Starnes VA, Kumar SR. Arch augmentation via median sternotomy for coarctation of aorta with proximal arch hypoplasia. Ann Thorac Surg. 2018;106(4):1214-1219. https://doi.org/10.1016/j.athoracsur.2018.04.025.
  7. Rudolph AM, Heymann MA, Spitznas U. Hemodynamic considerations in the development of narrowing of the aorta. Am J Cardiol. 1972 Oct;30(5):514-525. https://doi.org/10.1016/0002-9149(72)90042-2.
  8. Rakhra S, Lee M, Iyengar A, Wheaton G, Grigg L, Konstantinov I, Brizard C, d’Udekem Y. Poor outcomes after surgery for coarctation repair with hypoplastic arch warrants more extensive initial surgery and close long-term follow-up. Interact Cardiovasc Thorac Surg. 2013 Jan;16(1):31-36. https://doi.org/10.1093/icvts/ivs301.
  9. Axelrod D, Chock V, Reddy V. Management of the Preterm Infant with Congenital Heart Disease. Clin Perinatol. 2016 Mar;43(1):157-171. https://doi.org/10.1016/j.clp.2015.11.011.
  10. Kulyabin Y, Gorbatykh Y, Soynov I, Zubritskiy A, Voitov A, Bogachev-Prokophiev A. Selective Antegrade Cerebral Perfusion With or Without Additional Lower Body Perfusion During Aortic Arch Reconstruction in Infants. World J Pediatr Congenit Heart Surg. 2020 Jan;11(1):49-55. https://doi.org/10.1177/2150135119885887.
  11. De León LE, McKenzie ED. Aortic Arch Advancement and Ascending Sliding Arch Aortoplasty for Repair of Complex Primary and Recurrent Aortic Arch Obstruction. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 2017 Jan;20:63-66. https://doi.org/10.1053/j.pcsu.2016.09.007.
Published
2021-06-29
How to Cite
Truba, Y., Dziuryi, I., Sekelyk, R., Golovenko, O., & Lazoryshynets, V. (2021). Two-Stage Repair of Aortic Arch Hypoplasia Associated with Other Congenital Heart Defects in Newborns and Infants. Ukrainian Journal of Cardiovascular Surgery, (2 (43), 56-61. https://doi.org/10.30702/ujcvs/21.4306/t023056-061/007.21-053.2