Assessment of the pulmonary arterial growth and results of two-stage repair in infants with severe form of tetralogy of Fallot

Aim: assess of the pulmonary artery growth and the outcomes of complete repair after palliative treatment in infants with critical form of Tetralogy of Fallot.

Methods. This was prospective randomized two-center study (2019 to 2022). Its included infants with Tetralogy of Fallot who underwent palliation with either stenting of the right ventricular outflow tract (stent group, n=21) or modified Blalock-Taussig shunt (shunt group, n=21).

Results. In the stent group Nakata index increase from median 104.2 to 208.6 mm²/m², while in the shunt group, it increased from 107.3 to 169.4 mm²/m² (p<0,01). Mixed model analysis showed that in stent group the right pulmonary artery growth rate was 2.05*10^-2 z scores/day, which was 3.01 times higher than in the shunt group. In stent group the left pulmonary artery growth rate was 2.3*10^-2 z scores/day, which was 1.47 times higher than in the shunt group. In the stent group, there was one noncardiac-related mortality during the intermediate period. Transannular patch repair of the right ventricular outflow tract was performed in 12 patients (60%) in the stent group and in 15 patients (71.4%) in the shunt group (p=0.52) during complete repair. At 8 cases (40%) in the stent group and 6 cases (28.6%) in the shunt group, pulmonary artery replacement was performed. Time to surgical repair was shorter in the stent group (p=0.046), while the aortic cross clamp time (p<0,01) and cardiopulmonary bypass time (p<0,01) were significantly shorter in the shunt group.

Conclusions. Right ventricular outflow tract stenting provides hemodynamic stabilization and uniform growth of the pulmonary artery compared to modified Blalock-Taussig shunt.

1. Al Habib HF, Jacobs JP, Mavroudis C, Tchervenkov CI, O’Brien SM, Mohammadi S, Jacobs ML. Contemporary patterns of management of tetralogy of Fallot: data from the Society of Thoracic Surgeons Database. Ann Thorac Surg. 2010 Sep;90(3):813–820. https://doi.org/10.1016/j.athoracsur.2010.03.110

2. Barron DJ. Tetralogy of Fallot: controversies in early management. World J Pediatr Congenit Heart Surg. 2013 Apr;4(2):186–191. https://doi.org/10.1177/2150135112471352

3. Petrucci O, O’Brien SM, Jacobs ML, Jacobs JP, Manning PB, Eghtesady P. Risk factors for mortality and morbidity after the neonatal BlalockTaussig shunt procedure. Ann Thorac Surg. 2011 Aug;92(2):642–652. https://doi.org/10.1016/j.athoracsur.2011.02.030

4. Quandt D, Ramchandani B, Stickley J, Mehta C, Bhole V, Barron DJ, Stumper O. Stenting of the Right Ventricular Outflow Tract Promotes Better Pulmonary Arterial Growth Compared With Modified BlalockTaussig Shunt Palliation in Tetralogy of Fallot-Type Lesions. JACC Cardiovasc Interv. 2017 Sep 11;10(17):1774–1784. https://doi.org/10.1016/j.jcin.2017.06.023

5. Dohlen G, Chaturvedi RR, Benson LN, Ozawa A, Van Arsdell GS, Fruitman DS, Lee KJ. Stenting of the right ventricular outflow tract in the symptomatic infant with tetralogy of Fallot. Heart. 2009 Feb;95(2):142–147. https://doi.org/10.1136/hrt.2007.135723

6. Stumper O, Ramchandani B, Noonan P, Mehta C, Bhole V, Reinhardt Z, Dhillon R, Miller PA, de Giovanni JV. Stenting of the right ventricular outflow tract. Heart. 2013 Nov;99(21):1603–1608. https://doi.org/10.1136/heartjnl-2013-304155

7. Quandt D, Ramchandani B, Penford G, Stickley J, Bhole V, Mehta C, Jones T, Barron DJ, Stumper O. Right ventricular outflow tract stent versus BT shunt palliation in Tetralogy of Fallot. Heart. 2017 Dec;103(24):1985-1991. https://doi.org/10.1136/heartjnl-2016-310620

8. Barron DJ, Jegatheeswaran A. How and When Should Tetralogy of Fallot be Palliated Prior to Complete Repair? Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 2021;24:77-84. https://doi.org/10.1053/j.pcsu.2021.02.002

9. François Godart; Shakeel A Qureshi; Ary Simha; Philip B Deverall; David R Anderson; Edward J Baker; Michael Tynan (1998). Effects of modified and classic Blalock-Taussig shunts on the pulmonary arterial tree. Ann Thorac Surg. 1998 Aug;66(2):512-7; discussion 518. https://doi.org/10.1016/s0003-4975(98)00461-5

10. Joseph Caspi; Timothy W. Pettitt; Theodorus Mulder; Aluizio Stopa. Development of the Pulmonary Arteries After the Norwood Procedure: Comparison Between Blalock-Taussig Shunt and Right Ventricular– Pulmonary Artery Conduit. Ann Thorac Surg. 2008 Oct;86(4):0–1304. https://doi.org/10.1016/j.athoracsur.2008.06.016

11. Rumball, Elizabeth M., McGuirk, Simon P., Stümper, Oliver, Laker, Simon J., de Giovanni, Joseph V., Wright, John G., Barron, David J., Brawn, William J. The RV–PA conduit stimulates better growth of the pulmonary arteries in hypoplastic left heart syndrome. European Journal of Cardio-Thoracic Surgery. Eur J Cardiothorac Surg. 2005 May;27(5):801–806. https://doi.org/10.1016/j.ejcts.2005.01.061

12. Jay D. Pruetz; Sarah Badran; Fred Dorey; Vaughn A. Starnes; Alan B. Lewis. Differential branch pulmonary artery growth after the Norwood procedure with right ventricle–pulmonary artery conduit versus modified Blalock–Taussig shunt in hypoplastic left heart syndrome. J Thorac Cardiovasc Surg. 2009 Jun;137(6):1342–1348. https://doi.org/10.1016/j.jtcvs.2009.03.019

13. Andrew C. Fiore; Courtney Tobin; Saadeh Jureidini; Mohammad Rahimi; Eric S. Kim; Kenneth Schowengerdt. A Comparison of the Modified Blalock-Taussig Shunt With the Right Ventricle-to-Pulmonary Artery Conduit. Ann Thorac Surg. 2011 May;91(5):1479–1485. https://doi.org/10.1016/j.athoracsur.2010.11.062

14. Jo T. K. et al. Outcome of neonatal palliative procedure for pulmonary atresia with ventricular septal defect or tetralogy of Fallot with severe pulmonary stenosis: experience in a single tertiary center. Korean J Pediatr. 2018 Jul;61(7):210-216. https://doi.org/10.3345/kjp.2018.61.7.210

15. Zheng S., Yang K., Li K., Li S. Establishment of right ventriclepulmonary artery continuity as the first-stage palliation in older infants with pulmonary atresia with ventricular septal defect may be preferable to use of an arterial shunt. Interact Cardiovasc Thorac Surg. 2014 Jul;19(1):88–94. https://doi.org/10.1093/icvts/ivu052

16. Lenoir M. et al. Outcomes of palliative right ventricle to pulmonary artery connection for pulmonary atresia with ventricular septal defect. European Journal of Cardio-Thoracic Surgery. 2017;52(3):590-598. https://doi.org/10.1093/ejcts/ezx194

17. Di Donato, Roberto M.; Jonas, Richard A.; Lang, Peter; Rome, Jonathan J.; Mayer, John E.; Castaneda, Aldo R. Neonatal repair of tetralogy of Fallot with and without pulmonary atresia. The Journal of Thoracic and Cardiovascular Surgery. 1991; 101(1):126–137. https://doi.org/10.1016/s0022-5223(19)36802-3

18. Audrey C Marshall; Barry A Love; Peter Lang; Richard A Jonas; Pedro J del Nido; John E Mayer; James E Lock. Staged repair of tetralogy of Fallot and diminutive pulmonary arteries with a fenestrated ventricular septal defect patch. J Thorac Cardiovasc Surg. 2003 Nov;126(5):1427–1433. https://doi.org/10.1016/s0022-5223(03)01182-6

19. Chubb, Henry; Simpson, John M. The use of Z-scores in paediatric cardiology. Annals of Pediatric Cardiology. 2012;5(2):179–84. https://doi.org/10.4103/0974-2069.99622

20. Pettersen, Michael D.; Du, Wei; Skeens, Mary Ellen; Humes, Richard A. Regression Equations for Calculation of Z Scores of Cardiac Structures in a Large Cohort of Healthy Infants, Children, and Adolescents: An Echocardiographic Study. Journal of the American Society of Echocardiography. 2008 Aug;21(8):922-934.. https://doi.org/10.1016/j.echo.2008.02.006

21. Dryzek P, Mazurek-Kula A, Moszura T, Sysa A. Right ventricle outflow tract stenting as a method of palliative treatment of severe tetralogy of Fallot. Cardiol J. 2008;15(4):376-9. PMID: 18698548

22. Cools, Bjorn; Boshoff, Derize; Heying, Ruth; Rega, Filip; Meyns, Bart; Gewillig, Marc. Transventricular balloon dilation and stenting of the RVOT in small infants with tetralogy of fallot with pulmonary atresia. Catheterization and Cardiovascular Interventions. 2013 Aug 1;82(2):260–265. https://doi.org/10.1002/ccd.24548

23. Niall Linnane, Mohamed Al Nasef, Colin J. McMahon, Jonathan McGuinness, Brian McCrossan, Paul Oslizlok, Kevin P. Walsh, Damien Kenny. Right ventricular outflow tract stenting in symptomatic infants without the use of a long delivery sheath. Catheterization and Cardiovascular Interventions. 2021;98(2):275-281. https://doi.org/10.1002/ccd.29708

24. Nokhrin, Andrey V.; Tarasov, Roman S.; Mukhamadiyarov, Rinat A.; Shishkova, Daria K.; Kutikhin, Anton G.; Dzyuman, Anna N.; Khlusov, Igor A.; Barbarash, Leonid S. Two‐stage approach for surgical treatment of tetralogy of Fallot in underweight children: Clinical and morphological outcomes. Journal of Cardiac Surgery. 2019;34(5):293-299. https://doi.org/10.1111/jocs.14031

25. Sandoval, Juan Pablo; Chaturvedi, Rajiv R.; Benson, Lee; Morgan, Gareth; Van Arsdell, Glen; Honjo, Osami; Caldarone, Christopher; Lee, Kyong-Jin. Right Ventricular Outflow Tract Stenting in Tetralogy of Fallot Infants With Risk Factors for Early Primary Repair. Circulation: Cardiovascular Interventions. 2016 Dec;9(12):e003979. https://doi.org/10.1161/circinterventions.116.003979

26. Zhou Tingwen; Wang Yixuan; Liu Junwei; Wang Yin; Wang Yongjun; Chen Si; Zhou Cheng; Dong Nianguo. Pulmonary artery growth after Modified Blalock-Taussig shunt: A single center experience. Asian Journal of Surgery, 2020;43(2):428-437. https://doi.org/10.1016/j.asjsur.2019.06.002