The Heart Centre for Children, The Children's Hospital at Westmead, Westmead, Australia; The University of Sydney, Sydney, Australia.
The Royal Children's Hospital, Melbourne, Australia; The Murdoch Children's Research Institute, Melbourne, Australia.
J Thorac Cardiovasc Surg. 2019 Oct;158(4):1158-1167.e1. doi: 10.1016/j.jtcvs.2019.05.061. Epub 2019 Jun 10.
D-transposition of the great arteries and l-transposition of the great arteries with ventricular septal defect and left ventricular outflow tract obstruction are complex biventricular congenital heart diseases for which decision-making regarding surgical strategy remains challenging. We investigated the intermediate-term outcomes of Fontan versus biventricular procedures in these patients.
We analyzed 129 patients with d-transposition of the great arteries/ventricular septal defect/left ventricular outflow tract obstruction (n = 85) or l-transposition of the great arteries/ventricular septal defect/left ventricular outflow tract obstruction (n = 44) and 2 functional ventricles from Australia who had primary surgical management (29 Fontan, 100 biventricular repair) undertaken between 1990 and 2015.
Median operative age of patients was 2.9 years (range, 0.2-26.8 years). During a median follow-up of 6.2 years (range, 2 days to 25.8 years), 9 patients died after biventricular repair (3 early and 6 late deaths). One patient received a transplant 1.2 years after Fontan completion. Overall transplant-free survivals at 1, 5, 10, and 15 years were 95%, 93%, 92%, and 90%, respectively. Overall reintervention-free survivals at 1, 5, 10, and 15 years were 79%, 64%, 45%, and 29% respectively. Biventricular repair tended to be associated with a higher rate of death, transplantation, or reintervention than the Fontan pathway (hazard ratio, 1.83; 95% confidence interval, 0.90-3.71; P = .10). Some 73% of transplant-free survivors had New York Heart Association class I. Functional status was similar between the Fontan and biventricular groups.
Intermediate-term outcomes were comparable between patients with d-transposition of the great arteries/ventricular septal defect/left ventricular outflow tract obstruction and patients with l-transposition of the great arteries/ventricular septal defect/left ventricular outflow tract obstruction. Both Fontan and biventricular pathways are associated with excellent mortality and functional outcomes. Biventricular patients have a greater risk of reintervention. The Fontan procedure is a viable option when anatomic risk factors preclude biventricular repair.
大动脉转位伴室间隔缺损和左心室流出道梗阻以及大动脉转位伴室间隔缺损和左心室流出道梗阻均为复杂的双心室先天性心脏病，其手术策略的决策仍然具有挑战性。我们研究了 Fontan 与双心室手术在这些患者中的中期结果。
我们分析了 1990 年至 2015 年间澳大利亚的 85 例大动脉转位/室间隔缺损/左心室流出道梗阻（n=85）或 44 例大动脉转位/室间隔缺损/左心室流出道梗阻（n=44）伴 2 个功能性心室的患者的资料，这些患者均接受了初次外科治疗（29 例接受了 Fontan 手术，100 例接受了双心室修复术）。
患者的中位手术年龄为 2.9 岁（范围，0.2-26.8 岁）。中位随访 6.2 年（范围，2 天至 25.8 年）期间，100 例接受双心室修复术的患者中有 9 例死亡（3 例早期死亡，6 例晚期死亡）。1 例患者在 Fontan 术后 1.2 年接受了移植。1 年、5 年、10 年和 15 年的无移植生存率分别为 95%、93%、92%和 90%。1 年、5 年、10 年和 15 年的无再干预生存率分别为 79%、64%、45%和 29%。与 Fontan 途径相比，双心室修复术的死亡率、移植率或再干预率较高（风险比，1.83；95%置信区间，0.90-3.71；P=0.10）。73%的无移植生存者心功能分级为纽约心脏协会Ⅰ级。Fontan 组和双心室组的功能状态相似。
大动脉转位伴室间隔缺损/左心室流出道梗阻和大动脉转位伴室间隔缺损/左心室流出道梗阻患者的中期结果相似。Fontan 和双心室两种途径都有极好的死亡率和功能结局。双心室患者的再干预风险较高。当解剖危险因素排除双心室修复时，Fontan 手术是一种可行的选择。
