Advertisement

How and When Should Tetralogy of Fallot be Palliated Prior to Complete Repair?

      The controversy regarding the best or ideal surgical management of Tetralogy of Fallot (ToF) stems from the recognition of there being a spectrum of morphology and associated lesions, each of which require a different approach to achieve the three goals of minimizing mortality preserving right ventricular function long-term and minimizing reinterventions. A one-size-fits-all approach to ToF needs to be replaced by a considered and personalized approach in order to yield the best outcomes possible for individual patients. The great majority of patients with ToF undergo primary complete repair between age 3–9 months with excellent outcomes. However, the greatest challenge is the severely cyanotic neonates where primary repair is still associated with high mortality and reintervention rates. Risk factors are low weight and small/poorly developed pulmonary vasculature. High-risk neonates have better outcomes with palliation—but mortality is still high. Palliative interventions in the catherization lab are showing better outcomes than traditional BT shunt and the RVOT stent is emerging as potential game-changer. Primary neonatal repair is still recommended if weight >3 kg and Nakata >100 mm2/m2. However, neonates with low weight, small pulmonary arteries or multiple comorbidities (including ToF/AVSD and anomalous LAD) may do better with a staged approach, There is good argument for RVOT stenting as a bridge to complete repair due to its stable circulation without diastolic run off and volume loading of the circulation, and its potential to allow branch PA growth.

      Keywords

      To read this article in full you will need to make a payment

      Purchase one-time access:

      Academic and Personal
      One-time access price info
      • For academic or personal research use, select 'Academic and Personal'
      • For corporate R&D use, select 'Corporate R&D Professionals'

      References

        • Walsh EP
        • Rockenmacher S
        • Keane JF
        • et al.
        Late results in patients with tetralogy of Fallot repaired during infancy.
        Circulation. 1988; 77: 1062-1067
        • DiDonato RM
        • Jonas RA
        • Lang P
        • et al.
        Neonatal repair of tetralogy of Fallot with and without pulmonary atresia.
        J Thorac Cardiovasc Surg. 1991; 101: 126-137
        • Al Habib HF
        • Jacobs JP
        • Mavroudis C
        • et al.
        Contemporary patterns of management of tetralogy of Fallot: data from the Society of Thoracic Surgeons Database.
        Ann Thorac Surg. 2010; 90: 813-819
        • Lindberg HL
        • Saatvedt K
        • Seem E
        • et al.
        Singlecenter 50 years’ experience with surgical management of tetralogy of Fallot.
        Eur J Cardiothorac Surg. 2011; 40: 538-542
        • d'Udekem Y
        • Galati JC
        • Konstantinov IE
        • et al.
        Intersurgeon variability in long-term outcomes after transatrial repair of tetralogy of Fallot: 25 years' experience with 675 patients.
        J Thorac Cardiovasc Surg. 2014; 147: 880-886
        • Hickey EJ
        • Veldtman G
        • Bradley TJ
        • et al.
        Late risk of outcomes for adults with repaired tetralogy of Fallot from an inception cohort spanning four decades.
        Eur J Cardiothorac Surg. 2009; 35: 156-164
        • Bailey J
        • Elci OU
        • Mascio CE
        • et al.
        Staged versus complete repair in the symptomatic neonate with tetralogy of Fallot.
        Ann Thorac Surg. 2020; 109: 802-808
        • Kanter KR
        • Kogon BE
        • Kirshbom PM
        • et al.
        Symptomatic neonatal tetralogy of Fallot: repair or shunt?.
        Ann Thorac Surg. 2010; 89: 858-863
        • Kirsch RE
        • Glatz AC
        • Gaynor JW
        • et al.
        Results of elective repair at 6 months or younger in 277 patients with tetralogy of Fallot: a 14-year experience at a single center.
        J Thorac Cardiovasc Surg. 2014; 147: 713-717
        • Kolcz J
        • Pizarro C.
        Neonatal repair of tetralogy of Fallot results in improved pulmonary artery development without increased need for reintervention.
        Eur J Cardiothorac Surg. 2005; 28: 394-399
        • Marshall AC
        • Love BA
        • Lang P
        • et al.
        Staged repair of tetralogy of Fallot and diminutive pulmonary arteries with a fenestrated ventricular septal defect patch.
        J Thorac Cardiovasc Surg. 2003; 126: 1427-1433
        • Savla JJ
        • Faerber JA
        • Huang YV
        • et al.
        2-Year outcomes after complete or staged procedure for tetralogy of Fallot in neonates.
        J Am Coll Cardiol. 2019; 74: 1570-1579
        • Ramakrishnan KV
        • Zurakowski D
        • Pastor W
        • et al.
        Symptomatic tetralogy of Fallot in young infants: primary repair or shunt-pediatric health information system database analysis.
        World J Pediatr Congenit Heart Surg. 2018; 9: 539-545https://doi.org/10.1177/2150135118780615
        • Steiner MB
        • Tang X
        • Gossett JM
        • et al.
        Timing of complete repair of non-ductal-dependent tetralogy of Fallot and short-term postoperative outcomes, a multicenter analysis.
        J Thorac Cardiovasc Surg. 2014; 147: 1299-1305
        • Ghimire LV
        • Chou FS
        • Devoe C
        • et al.
        Comparison of in-hospital outcomes when repair of tetralogy of Fallot is in the neonatal period versus in the post-neonatal period.
        Am J Cardiol. 2020; 125: 140-145
        • Fraser Jr CD
        We should reframe the discussion/debate about neonatal repair of tetralogy of Fallot.
        J Thorac Cardiovasc Surg. 2021; 161: 1421-1425https://doi.org/10.1016/j.jtcvs.2020.05.093
      1. Qureshi AM, Caldarone CA, Romano JC, et al. Comparison of management strategies for the neonate with symptomatic tetralogy of fallot and body weight less than 2.5 kg: insights from the congenital catheterization research collaborative. J Thorac Cardiovasc Surg. 2021 Feb 3:S0022-5223(21)00199-9. https://doi.org/10.1016/j.jtcvs.2021.01.100

        • Tamesberger MI
        • Lechner E
        • Mair R
        • et al.
        Early primary repair of tetralogy of fallot in neonates and infants less than four months of age.
        Ann Thorac Surg. 2008; 86: 1928-1935
        • Arenz C
        • Laumeier A
        • Lütter S
        • et al.
        Is there any need for a shunt in the treatment of tetralogy of Fallot with one source of pulmonary blood flow?.
        Eur J Cardiothorac Surg. 2013; 44: 648-654
        • d'Udekem Y.
        Primary repair of tetralogy of Fallot may be cheaper but for how long? Is there a universal truth?.
        World J Ped Cong Ht Surg. 2018; 9: 548-549
        • Cunningham ME
        • Donofrio MT
        • Peer SM
        • et al.
        Optimal timing for elective early primary repair of tetralogy of Fallot: analysis of intermediate term outcomes.
        Ann Thorac Surg. 2017; 103: 845-852
        • Wilder TJ
        • Van Arsdell GS
        • Benson L
        • et al.
        Young infants with severe tetralogy of Fallot: early primary surgery versus transcatheter palliation.
        J Thorac Cardiovasc Surg. 2017; 154 (.e2Epub 2017 May 19. PMID: 28666664): 1692-1700https://doi.org/10.1016/j.jtcvs.2017.05.042
        • Petrucci O
        • O'Brien SM
        • Jacobs ML
        • et al.
        Risk factors for mortality and morbidity after the neonatal blalock-taussig shunt procedure.
        Ann Thorac Surg. 2011; 92: 642-651
      2. Congenital cardiac audit database: arterial shunt outcomes. Available at:https://nicor4.nicor.org.uk/CHD/an_paeds.nsf/0/FA2C8318DAA46FFE802572E5004C04E1.

        • Chong BK
        • Baek JS
        • Im YM
        • et al.
        Systemic-pulmonary shunt facilitates the growth of the pulmonary valve annulus in patients with tetralogy of Fallot.
        Ann Thorac Surg. 2016; 102: 1322-1328
        • Jeon B
        • Kim DH
        • Kwon BS
        • et al.
        Surgical treatment of tetralogy of Fallot in symptomatic neonates and young infants.
        J Thorac Cardiovasc Surg. 2020; 159: 1466-1476
        • Bentham JR
        • Zava NK
        • Harrison WJ
        • et al.
        Duct stenting versus modified Blalock-Taussig shunt in neonates with duct-dependent pulmonary blood flow: associations with clinical outcomes in a multicenter national study.
        Circulation. 2018; 137: 581-588
        • Glatz AC
        • Petit CJ
        • Goldstein BH
        • et al.
        Comparison between patent ductus arteriosus stent and modified Blalock-Taussig shunt as palliation for infants with ductal-dependent pulmonary blood flow: insights from the congenital catheterization research collaborative.
        Circulation. 2018; 137: 589-601
        • Alsagheir A
        • Koziarz A
        • Makhdoum A
        • et al.
        Duct stenting versus modified Blalock-Taussig shunt in neonates and infants with duct-dependent pulmonary blood flow: a systematic review and meta-analysis.
        J Thorac Cardiovasc Surg. 2021; 161: 379-390https://doi.org/10.1016/j.jtcvs.2020.06.008
        • Sandoval JP
        • Chaturvedi RR
        • Benson L
        • et al.
        Right ventricular outflow tract stenting in tetralogy of Fallot infants with risk factors for early primary repair.
        Circ Cardiovasc Interv. 2016; 9e003979
        • Quandt D
        • Ramchandani B
        • Penford G
        • et al.
        Right ventricular outflow tract stent versus BT shunt palliation in tetralogy of Fallot.
        Heart. 2017; 103: 1985-1991
        • Quandt D
        • Ramchandani B
        • Stickley J
        • et al.
        Stenting of the right ventricular outflow tract promotes better pulmonary arterial growth compared with modified Blalock-Taussig shunt palliation in tetralogy of Fallot-type lesions.
        JACC Cardiovasc Interv. 2017; 10: 1774-1784
        • McGovern E
        • Morgan CT
        • Oslizlok P
        • et al.
        Transcatheter stenting of the right ventricular outflow tract augments pulmonary arterial growth in symptomatic infants with right ventricular outflow tract obstruction and hypercyanotic spells.
        Cardiol Young. 2016; 26: 1260-1265
        • Barron DJ
        • Ramchandani B
        • Murala J
        • et al.
        Surgery following primary right ventricular outflow tract stenting for Fallot's tetralogy and variants: rehabilitation of small pulmonary arteries.
        Eur J Cardiothorac Surg. 2013; 44: 656-662
        • Ghaderian M
        • Ahmadi A
        • Sabri MR
        • et al.
        Clinical outcome of right ventricular outflow tract stenting versus Blalock-Taussig shunt in tetralogy of Fallot: a systematic review and meta-analysis.
        Curr Probl Cardiol. 2020; 4100643