The 30-Year Outcomes of Tetralogy of Fallot According to Native Anatomy and Genetic Conditions
The 30-Year Outcomes of Tetralogy of Fallot According to Native Anatomy and Genetic Conditions
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DOI：
10.1016/j.cjca.2020.10.002
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发表时间：
2021-06-18
影响因子：
6.2
通讯作者：
Dallaire, Frederic
中科院分区：
文献类型：
--
作者：
Blais, Samuel;Marelli, Ariane;Dallaire, Frederic
背景：法乐氏四联症（TOF）的存活率已超过97%。肺动脉闭锁和/或遗传性疾病的患者预后较差，但这些TOF亚组的长期生存率和发病率的估计很少。本研究的目的是描述的30年的结果TOF根据本地解剖和共存的遗传conditions.Methods：的TRIVIA（法洛四联症研究改善瓣膜置换术干预：跨越知识鸿沟的桥梁）研究是一项回顾性的人群为基础的队列，包括所有TOF受试者出生于1980年至2015年在魁北克。我们通过考克斯比例风险回归评估了全因死亡率，并使用边际均值/比率模型评估了心血管干预和计划外住院的累积平均数。我们根据TOF类型，即经典TOF（cTOF）和TOF合并肺动脉闭锁（TOF-PA），以及遗传条件的存在，计算了30年的预后估计值。中位随访时间为17年（四分位距，8-27）。非综合征性cTOF受试者的30年生存率为95%，平均每例受试者接受2.8次干预和0.5次住院治疗。相比之下，TOF-PA受试者的30年生存率较低，为78%，平均接受8.1次干预，住院次数为4倍。遗传性疾病的存在与较低的生存率相关（cTOF < 85%，TOF-PA < 60%），但干预和住院次数相似。结论：解剖类型和遗传性疾病的存在强烈影响TOF的长期结局。我们提供了可靠的30年预后的关键标志物，可用于改善风险分层，并为家庭提供更明智的咨询。
Background: The reported survival of tetralogy of Fallot (TOF) is > 97%. Patients with pulmonary atresia and/or genetic conditions have worse outcomes, but long-term estimates of survival and morbidity for these TOF subgroups are scarce. The objective of this study was to describe the 30-year outcomes of TOF according to native anatomy and the coexistence of genetic conditions.Methods: The TRIVIA (Tetralogy of Fallot Research for Improvement of Valve Replacement Intervention: A Bridge Across the Knowledge Gap) study is a retrospective population-based cohort including all TOF subjects born from 1980 to 2015 in Quebec. We evaluated all-cause mortality by means of Cox proportional hazards regression, and cumulative mean number of cardiovascular interventions and unplanned hospitalisations with the use of marginal means/rates models. We computed 30-year estimates of outcomes according to TOF types, ie, classic TOF (cTOF) and TOF with pulmonary atresia (TOF-PA), and the presence of genetic conditions.Results: We included 960 subjects. The median follow-up was 17 years (interquartile range, 8-27). Nonsyndromic cTOF subjects had a 30-year survival of 95% and had undergone a mean of 2.8 interventions and 0.5 hospitalisations per subject. In comparison, TOF-PA subjects had a lower 30-year survival of 78% and underwent a mean of 8.1 interventions, with 4 times as many hospitalisations. The presence of a genetic condition was associated with lower survival (< 85% for cTOF and < 60% for TOF-PA) but similar numbers of interventions and hospitalisations.Conclusions: The anatomic types and the presence of genetic conditions strongly influence the long-term outcomes of TOF. We provided robust 30-year estimates for key markers of prognosis that may be used to improve risk stratification and provide more informed counselling to families.