Dorobantu Dan M, Mahani Alireza S, Sharabiani Mansour T A, Pandey Ragini, Angelini Gianni D, Parry Andrew J, Tulloh Robert M R, Martin Robin P, Stoica Serban C
Cardiac Surgery, Bristol Royal Hospital for Children, University Hospital Bristol NHS Trust, Bristol, UK.
Cardiology Department, "Prof. CC Iliescu" Emergency Institute for Cardiovascular Diseases, Bucuresti, Romania.
Heart. 2018 Nov;104(22):1864-1870. doi: 10.1136/heartjnl-2018-312958. Epub 2018 May 2.
Treatment of infants with tetralogy of Fallot (ToF) has evolved in the last two decades with increasing use of primary surgical repair (PrR) and transcatheter right ventricular outflow tract palliation (RVOTd), and fewer systemic-to-pulmonary shunts (SPS). We aim to report contemporary results using these treatment options in a comparative study.
This a retrospective study using data from the UK National Congenital Heart Disease Audit. All infants (n=1662, median age 181 days) with ToF and no other complex defects undergoing repair or palliation between 2000 and 2013 were considered. Matching algorithms were used to minimise confounding due to lower age and weight in those palliated.
Patients underwent PrR (n=1244), SPS (n=311) or RVOTd (n=107). Mortality at 12 years was higher when repair or palliation was performed before the age of 60 days rather than after, most significantly for primary repair (18.7% vs 2.2%, P<0.001), less so for RVOTd (10.8% vs 0%, P=0.06) or SPS (12.4% vs 8.3%, P=0.2). In the matched groups of patients, RVOTd was associated with more right ventricular outflow tract (RVOT) reinterventions (HR=2.3, P=0.05 vs PrR, HR=7.2, P=0.001 vs SPS) and fewer pulmonary valve replacements (PVR) (HR=0.3 vs PrR, P=0.05) at 12 years, with lower mortality after complete repair (HR=0.2 versus PrR, P=0.09).
We found that RVOTd was associated with more RVOT reinterventions, fewer PVR and fewer deaths when compared with PrR in comparable, young infants, especially so in those under 60 days at the time of the first procedure.
在过去二十年中，法洛四联症（ToF）患儿的治疗方法不断演变，一期手术修复（PrR）和经导管右心室流出道姑息治疗（RVOTd）的应用日益增加，体肺分流术（SPS）的使用则减少。我们旨在通过一项对比研究报告使用这些治疗方案的当代结果。
这是一项回顾性研究，使用来自英国国家先天性心脏病审计的数据。纳入2000年至2013年间所有患有ToF且无其他复杂缺陷并接受修复或姑息治疗的婴儿（n = 1662，中位年龄181天）。采用匹配算法以尽量减少因接受姑息治疗的患儿年龄和体重较低而产生的混杂因素。
患者接受了一期手术修复（n = 1244）、体肺分流术（n = 311）或经导管右心室流出道姑息治疗（n = 107）。60日龄前进行修复或姑息治疗的患者12年死亡率高于60日龄后，一期手术修复最为显著（18.7%对2.2%，P<0.001），经导管右心室流出道姑息治疗则较少（10.8%对0%，P = 0.06），体肺分流术亦是如此（12.4%对8.3%，P = 0.2）。在匹配的患者组中，经导管右心室流出道姑息治疗在12年时与更多的右心室流出道（RVOT）再次干预相关（HR = 2.3，与一期手术修复相比P = 0.05，与体肺分流术相比HR = 7.2，P = 0.001），且肺动脉瓣置换术（PVR）较少（HR = 0.3与一期手术修复相比，P = 0.05），完全修复后的死亡率较低（HR = 0.2与一期手术修复相比，P = 0.09）。
我们发现，在年龄相仿的低龄婴儿中，尤其是首次手术时年龄小于60日龄的婴儿，与一期手术修复相比，经导管右心室流出道姑息治疗与更多的右心室流出道再次干预、更少的肺动脉瓣置换术及更低的死亡率相关。
