Department of Congenital Heart Disease, Leeds Teaching Hospitals NHS Trust, Leeds, UK.
Cardiol Young. 2023 May;33(5):710-717. doi: 10.1017/S1047951122001524. Epub 2022 May 16.
Mortality between stages 1 and 2 single-ventricle palliation is significant. Home-monitoring programmes are suggested to reduce mortality. Outcomes and risk factors for adverse outcomes for European programmes have not been published.
To evaluate the performance of a home-monitoring programme at a medium-sized United Kingdom centre with regards survival and compare performance with other home-monitoring programmes in the literature.
All fetal and postnatal diagnosis of a single ventricle were investigated with in-depth analysis of those undergoing stage 1 palliation and entered the home-monitoring programme between 2016 and 2020. The primary outcome was survival. Secondary outcomes included multiple parameters as potential predictors of death or adverse outcome.
Of 217 fetal single-ventricle diagnoses during the period 2016-2020, 50.2% progressed to live birth, 35.4% to stage 1 and 29.5% to stage 2. Seventy-four patients (including 10 with postnatal diagnosis) entered the home-monitoring programme with six deaths making home-monitoring programme mortality 8.1%. Risk factors for death were the hybrid procedure as the only primary procedure (OR 33.0, p < 0.01), impaired cardiac function (OR 10.3, p < 0.025), Asian ethnicity (OR 9.3, p < 0.025), lower mean birth-weight (2.69 kg versus 3.31 kg, p < 0.01), and lower mean weight centiles during interstage follow-up (mean centiles of 3.1 versus 10.8, p < 0.01).
Survival in the home-monitoring programme is comparable with other home-monitoring programmes in the literature. Hybrid procedure, cardiac dysfunction, sub-optimal weight gain, and Asian ethnicity were significant risk factors for death. Home-monitoring programmes should continue to raise awareness of these factors and seek solutions to mitigate adverse events. Future work to generalise home-monitoring programme and single-ventricle fetus to stage 2 outcomes in the United Kingdom will require multi-centre collaboration.
1 期和 2 期单心室姑息术后的死亡率仍然较高。建议采用家庭监测方案以降低死亡率。但目前尚未发表有关欧洲方案的不良结局发生率和危险因素的数据。
评估英国一家中型中心的家庭监测方案在生存率方面的表现，并将其与文献中的其他家庭监测方案进行比较。
对所有经产前和产后诊断为单心室的患者进行了深入分析，这些患者均接受了 1 期姑息治疗，并于 2016 年至 2020 年期间进入家庭监测方案。主要结局是生存率。次要结局包括多个参数，这些参数可能是死亡或不良结局的预测指标。
在 2016 年至 2020 年期间，217 例胎儿单心室诊断中，50.2%存活至分娩，35.4%进展至 1 期，29.5%进展至 2 期。74 例患者（包括 10 例产后诊断）进入家庭监测方案，其中 6 例死亡，家庭监测方案死亡率为 8.1%。死亡的危险因素包括杂交手术作为唯一的初始手术（OR 33.0，p < 0.01）、心功能不全（OR 10.3，p < 0.025）、亚洲种族（OR 9.3，p < 0.025）、出生体重较低（2.69 kg 与 3.31 kg，p < 0.01）和随访期间体重百分位数较低（平均百分位数为 3.1 与 10.8，p < 0.01）。
家庭监测方案的生存率与文献中的其他家庭监测方案相当。杂交手术、心功能不全、体重增长不理想和亚洲种族是死亡的显著危险因素。家庭监测方案应继续提高对这些因素的认识，并寻求解决方案以减轻不良事件。未来的工作需要多中心合作，以将家庭监测方案和单心室胎儿推广至英国的 2 期结局。
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