Tactics of Surgical Repair of Single Ventricle with Excessive Pulmonary Blood Flow and Obstruction of the Distal Arch of the Aorta in Newborns
Abstract
Pulmonary artery stenosis with distal aortic arch reconstruction and coarctation of the aorta in newborns is an effective palliative procedure for single ventricle and high pulmonary hypertension on the way to total cavo-pulmonary anastomosis.
The aim. To present a case of complex correction of a single ventricle of the heart with tricuspid valve atresia and high pulmonary hypertension, obstruction of the distal aortic arch and coarctation of the aorta, and a final effective Fontan procedure with a good long-term outcome.
Case report. On December 15, 2011, a 9-day-old newborn patient M. was admitted for treatment at the Department of Cardiovascular Surgery of Odesa Regional Children’s Clinical Hospital with a diagnosis of: a single ventricle of the heart, transposition of the great arteries, tricuspid atresia, distal arch hypoplasia, coarctation of the aorta, patent ductus arteriosus, high pulmonary hypertension. The first stage of surgical treatment was carried out on December 19, 2011: main pulmonary artery banding, reconstruction of the distal arch of the aorta through modified Amato technique, closure of the patent ductus arteriosus, resection of the coarctation of the aorta and extended end-to-end aortoplasty. At the age of 1 year (December 27, 2012), the second stage was performed: bidirectional Glenn procedure with plastic surgery of bifurcation and right pulmonary artery branch. The third stage was performed at the age of 3 years 11 months (November 19, 2015): Fontan procedure with extracardiac conduit.
Conclusions. Early elimination of pulmonary hypertension by pulmonary artery banding ensures the preservation of the pulmonary vascular bed with low resistance, which is a crucial condition for the effective final hemodynamic correction of the single ventricle of the heart – the Fontan procedure. In case of hypoplasia of the distal aortic arch with coarctation in newborns, one of the alternative approaches is the use of modified Amato technique avoiding artificial circulation.
References
- Hoffman JI, Kaplan S. The incidence of congenital heart disease. J Am Coll Cardiol. 2002;39(12):1890-1900. https://doi.org/10.1016/s0735-1097(02)01886-7
- Zou MH, Cao F, Ma L, Xia YS, Yang SC, Chen WD, et al. [Early-and midterm outcomes of pulmonary artery band as an initial palliation in patients with single ventricle associated with unrestricted pulmonary blood flow]. Zhonghua Wai Ke Za Zhi. 2019;57(12):939-943. Chinese. https://doi.org/10.3760/cma.j.issn.0529-5815.2019.12.013
- Corno AF, Findley TO, Salazar JD. Narrative review of single ventricle: where are we after 40 years? Transl Pediatr. 2023;12(2):221-244. https://doi.org/10.21037/tp-22-573
- Zinkovsky MF, Dovhan OM, Lazoryshynets VV. [Surgical treatment of univentricular heart by different methods of right ventricle “passing”]. Shpytalna khirurhiia. 2000;(2):29-32. Ukrainian.
- Haller C, Barron DJ. Surgical Strategies in Single Ventricle Management of Neonates and Infants. Can J Cardiol. 2022;38(7):909-920. https://doi.org/10.1016/j.cjca.2022.04.021
- Alsoufi B, Manlhiot C, Ehrlich A, Oster M, Kogon B, Mahle WT, et al. Results of palliation with an initial pulmonary artery band in patients with single ventricle associatedwith unrestricted pulmonary blood flow. J Thorac Cardiovasc Surg. 2015;149(1):213-220. https://doi.org/10.1016/j.jtcvs.2014.08.007
- Bradley SM, Simsic JM, Atz AM, Dorman BH. The infant with single ventricle and excessive pulmonary blood flow: results of a strategy of pulmonary artery division and shunt. Ann Thorac Surg. 2002;74(3):805-810. https://doi.org/10.1016/s0003-4975(02)03836-5
- Bairamov E, Poznyak Y, Stogova O, Romanyuk O. [The Experience of Subaortic obstruction repair Concomitantly with Total Cavopulmonary Connection]. Visnyk sertsevo-sudynnoi khirurhii. 2016;2:53-57. Ukrainian.
- Rijnberg FM, Sojak V, Blom NA, Hazekamp MG. Long-Term Outcome of Direct Relief of Subaortic Stenosis in Single Ventricle Patients. World J Pediatr Congenit Heart Surg. 2018;9(6):638-644. https://doi.org/10.1177/2150135118793087
- Lekan RY, Buzovskyi VP, Lekan IR, inventors; Odessa National Medical University, assignee. Method for eliminating hypoplasia of distal aortic arch and aortic coarctation in newborn and infant children. Ukraine patent UA 102971. 2013 Aug 27.
- Amato JJ, Rheinlander HF, Cleveland RJ. A Method of Enlarging the Distal Transverse Arch in Infants with Hypoplasia and Coarctation of the Aorta. Ann Thorac Surg. 1977;23(3):261-263. https://doi.org/10.1016/s0003-4975(10)64121-5
- Truba YaP, Golovenko OS, Dzyurii IV, Motrechko OO, Lazoryshynets VV. [Late results of surgical treatment of the aortal arch hypoplasia in newborns and babies]. Klinichna khirurhiia. 2021;88(3-4):8-15. Ukrainian. https://doi.org/10.26779/2522-1396.2021.3-4.08
- Park WK, Baek JS, Kwon BS, Im YM, Lee JH, Choi ES, et al. Revisitation of Double-Inlet Left Ventricle or Tricuspid Atresia With Transposed Great Arteries. Ann Thorac Surg. 2019;107(4):1212-1217. https://doi.org/10.1016/j.athoracsur.2018.11.052
- Fraser CD Jr. Management of Systemic Outlet Obstruction in Patients Undergoing Single Ventricle Palliation. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 2009;12(1):70-75. https://doi.org/10.1053/j.pcsu.2009.01.006
