Comparison of Management Strategies for Neonates With Symptomatic Tetralogy of Fallot. Goldstein BH, Petit CJ, Qureshi AM, McCracken CE, Kelleman MS, Nicholson GT, Law MA, Meadows JJ, Zampi JD, Shahanavaz S, Mascio CE, Chai PJ, Romano JC, Batlivala SP, Maskatia SA, Asztalos IB, Kamsheh AM, Healan SJ, Smith JD, Ligon RA, Pettus JA, Juma S, Raulston JEB, Hock KM, Pajk AL, Eilers LF, Khan HQ, Merritt TC, Canter M, Juergensen S, Rinderknecht FA, Bauser-Heaton H, Glatz AC.J Am Coll Cardiol. 2021 Mar 2;77(8):1093-1106. doi: 10.1016/j.jacc.2020.12.048.
Hornik CP.J Am Coll Cardiol. 2021 Mar 2;77(8):1107-1109. doi: 10.1016/j.jacc.2020.12.043.
Take Home Points
- Neonates with symptomatic Tetralogy of Fallot may undergo early primary repair or staged repair consisting of initial palliation to augment pulmonary blood flow followed by complete repair.
- A retrospective, multicenter study found a similar observed risk of death at 4 years with early primary repair compared to a stager approach.
Commentary by Dr. Luis Quinonez (Boston USA), section editor of Congenital Heart Surgery Journal Watch:
Question: What is the best surgical strategy for the newborn with symptomatic TOF, complete or staged surgical repair?
Design: Retrospective, multicenter (Congenital Cardiac Research Collaborative)
Population: Symptomatic neonates with TOF with PS or PA undergoing early primary repair (n=230) and staged repair (n=342)
Analysis: Risk stratified; Propensity score adjustment
Outcomes: Death, cumulative morbidity, reinterventions
Pre-procedural ventilation, prematurity, DiGeorge syndrome, and pulmonary atresia were more common in the staged group.
Reintervention risk was higher in the SR group.
Less neonatal morbidity in the staged group, whereas overall cumulative morbidity burden favoured the primary group.
This is a very important paper that should be read. It addresses an ongoing dilemma in our specialty: Staged vs. primary repair of symptomatic neonatal TOF with PS or PA. The study is retrospective but multicenter. There is a selection bias to stage the sickest patients. The study attempts to risk adjust and uses propensity methodology. The main finding of the paper is that overall mortality is similar between staged and primary repair, despite early mortality risk in the primary repair group. The burden of reintervention occurs in the staged group. Cumulative morbidity favoured the primary repair group.
A few things are worth pointing out: The increased popularity of stent-based palliation may affect the outcomes of patients with the staged approach, in a positive or negative direction. It is unclear whether PDA stenting is superior to a Blalock-Taussig shunt and will likely be the subject of a randomized trial in the near future. Valve-sparing interventions were not different between the two groups, even when looking at the patients with PS alone. The argument that sparing the pulmonary valve is more likely in an older patient may not hold. The rate of pulmonary arterioplasty was similar in both groups, suggesting no advantaged for either treatment strategy. Because the median follow-up is about 4 years, we cannot know if either strategy will have any long-term advantage.
I am concerned that with the current public reporting systems that emphasize early mortality (30-day or operative), there may risk aversion against primary repair and in favour of a staged approach. This problem may be averted by public reporting of longer-term outcomes (even up to a year).
This paper provides equipoise to justify a randomized trial. The accompanying editorial focuses on the challenges of doing surgical randomized trials, suggesting randomization methodology to account for surgeon or institution expertise.