Department of Paediatric Cardiac Surgery, Heart Institute, Bristol Royal Hospital for Children, Bristol, UK.
Faculty of Health Sciences, University of Bristol, Bristol, UK.
Interact Cardiovasc Thorac Surg. 2020 Oct 1;31(4):547-554. doi: 10.1093/icvts/ivaa130.
Some patients with complex congenital heart disease (cCHD) also require aortic valve (AoV) procedures. These cases are considered high risk but their outcome has not been well characterized. We aim to describe these scenarios in the current practice, and provide outcome data for counselling and decision-making.
This was a retrospective study using the UK National Congenital Heart Disease Audit data on cCHD patients undergoing aortic valve replacement, balloon dilation (balloon aortic valvuloplasty) or surgical repair (surgical aortic valve repair) between 2000 and 2012. Coarsened exact matching was used to pair cCHD with patients undergoing AoV procedures for isolated valve disease.
A total of 201 patients with a varied spectrum of cCHD undergoing 242 procedures were included, median age 9.4 years (1 day-65 years). Procedure types were: balloon aortic valvuloplasty (n = 31, 13%), surgical aortic valve repair (n = 57, 24%) and aortic valve replacement (n = 154, 63%). Mortality at 30 days was higher in neonates (21.8% vs 5.3%, P = 0.02). Survival at 10 years was 83.1%, freedom from aortic valve replacement 83.8% and freedom from balloon aortic valvuloplasty/surgical aortic valve repair 86.3%. Neonatal age (P < 0.001), single ventricle (P = 0.08), concomitant Fontan/Glenn (P = 0.002) or aortic arch procedures (0.02) were associated with higher mortality. cCHD patients had lower survival at 30 days (93% vs 100%, P = 0.003) and at 10 years (86.4% vs 96.1%, P = 0.005) compared to matched isolated AoV disease patients.
AoV procedures in cCHD can be performed with good results outside infancy, but with higher mortality than in isolated AoV disease. Neonates and patients with single ventricle defects, especially those undergoing concomitant Fontan/Glenn, have worse outcomes.
一些患有复杂先天性心脏病（cCHD）的患者还需要主动脉瓣（AoV）手术。这些病例被认为风险较高，但他们的预后尚未得到很好的描述。我们旨在描述当前实践中的这些情况，并提供咨询和决策的结果数据。
这是一项回顾性研究，使用英国国家先天性心脏病审计数据，对 2000 年至 2012 年间接受主动脉瓣置换术、球囊扩张术（balloon aortic valvuloplasty）或手术修复术（surgical aortic valve repair）的 cCHD 患者进行分析。采用粗化精确匹配方法将 cCHD 患者与因孤立性瓣膜疾病接受 AoV 手术的患者进行配对。
共纳入 201 例患有不同类型 cCHD 的患者，共进行了 242 次手术，中位年龄为 9.4 岁（1 天-65 岁）。手术类型包括：球囊主动脉瓣扩张术（n=31，13%）、主动脉瓣手术修复术（n=57，24%）和主动脉瓣置换术（n=154，63%）。30 天死亡率在新生儿中较高（21.8%比 5.3%，P=0.02）。10 年生存率为 83.1%，主动脉瓣置换术的无失败率为 83.8%，球囊主动脉瓣扩张术/主动脉瓣手术修复术的无失败率为 86.3%。新生儿年龄（P<0.001）、单心室（P=0.08）、同期 Fontan/Glenn 术（P=0.002）或主动脉弓手术（P=0.02）与死亡率升高相关。与匹配的孤立性 AoV 疾病患者相比，cCHD 患者的 30 天死亡率（93%比 100%，P=0.003）和 10 年生存率（86.4%比 96.1%，P=0.005）较低。
cCHD 患者的 AoV 手术可在婴儿期后获得良好的结果，但死亡率高于孤立性 AoV 疾病。新生儿和单心室缺陷患者，特别是接受同期 Fontan/Glenn 术的患者，预后较差。
