Young infants with symptomatic tetralogy of Fallot: Shunt or primary repair?

Xin Tao Ye MD , Soichiro Henmi MD, PhD , Edward Buratto MBBS, PhD , Mitchell C. Haverty MS , Can Yerebakan MD , Tyson Fricke MBBS, PhD , Christian P. Brizard MD, MS , Yves d’Udekem MD, PhD , Igor E. Konstantinov MD, PhD
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Abstract

Objectives

The optimal treatment strategy for symptomatic young infants with tetralogy of Fallot (TOF) is unclear. We sought to compare the outcomes of staged repair (SR) (shunt palliation followed by second-stage complete repair) versus primary repair (PR) at 2 institutions that have exclusively adopted each strategy.

Methods

We performed propensity score-matched comparison of 143 infants under 4 months of age who underwent shunt palliation at one institution between 1993 and 2021 with 122 infants who underwent PR between 2004 and 2018 at another institution. The primary outcome was mortality. Secondary outcomes were postoperative complications, durations of perioperative support and hospital stays, and reinterventions. Median follow-up was 8.3 years (interquartile range, 8.1-13.4 years).

Results

After the initial procedure, hospital mortality (shunt, 2.8% vs PR, 2.5%; P = .86) and 10-year survival (shunt, 95%; 95% confidence interval [CI], 90%-98% vs PR, 90%; 95% CI, 81%-95%; P = .65) were similar. The SR group had a greater risk of early reinterventions but similar rates of late reinterventions. Propensity score matching yielded 57 well-balanced pairs. In the matched cohort, the SR group had similar freedom from reintervention (55%; 95% CI, 39%-68% vs 59%; 95% CI, 43%-71%; P = .85) and greater survival (98%; 95% CI, 88%-99.8% vs 85%; 95% CI, 69%-93%; P = .02) at 10 years, as the result of more noncardiac-related mortalities in the PR group.

Conclusions

In symptomatic young infants with TOF operated at 2 institutions with exclusive treatment protocols, the SR strategy was associated with similar cardiac-related mortality and reinterventions as the PR strategy at medium-term follow-up.

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患有症状性法洛氏四联症的幼儿:分流术还是初次修复术?
目标法洛氏四联症(TOF)无症状幼婴的最佳治疗策略尚不明确。我们试图在两家完全采用分期修复(SR)(分流姑息后进行第二阶段完全修复)与初次修复(PR)策略的机构中比较两者的治疗效果。方法我们对 1993 年至 2021 年期间在一家机构接受分流姑息治疗的 143 名 4 个月以下婴儿与 2004 年至 2018 年期间在另一家机构接受 PR 治疗的 122 名婴儿进行了倾向评分匹配比较。主要结果是死亡率。次要结果是术后并发症、围手术期支持和住院时间以及再干预。中位随访时间为 8.3 年(四分位间范围为 8.1-13.4 年)。结果首次手术后,住院死亡率(分流,2.8% vs PR,2.5%;P = .86)和 10 年生存率(分流,95%;95% 置信区间 [CI],90%-98% vs PR,90%;95% CI,81%-95%;P = .65)相似。SR 组早期再干预的风险更大,但晚期再干预的比例相似。倾向评分匹配产生了 57 对平衡良好的配对。在匹配队列中,SR 组的再干预率相似(55%;95% CI,39%-68% vs 59%;95% CI,43%-71%;P = .85),10 年后的存活率更高(98%;95% CI,88%-99.8% vs 85%;95% CI,69%-93%;P = .02),这是因为 PR 组的非心脏相关死亡率更高。结论 对于在两家机构接受手术并采用独家治疗方案的有症状的年幼 TOF 婴儿,在中期随访时,SR 策略与 PR 策略具有相似的心脏相关死亡率和再干预率。
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