King Gregory, Buratto Edward, Cordina Rachael, Iyengar Ajay, Grigg Leeanne, Kelly Andrew, Bullock Andrew, Ayer Julian, Alphonso Nelson, d'Udekem Yves, Konstantinov Igor E
Department of Cardiac Surgery, Royal Children's Hospital, Melbourne, Australia; Department of Paediatrics, University of Melbourne, Melbourne, Australia; Heart Research Group, Murdoch Children's Research Institute, Melbourne, Australia.
Department of Paediatrics, University of Melbourne, Melbourne, Australia; Heart Research Group, Murdoch Children's Research Institute, Melbourne, Australia; Department of Cardiac Surgery, The Royal Melbourne Hospital, Melbourne, Australia.
J Thorac Cardiovasc Surg. 2023 Feb;165(2):424-433. doi: 10.1016/j.jtcvs.2022.04.011. Epub 2022 Apr 20.
The effect of ventricular dominance and previous atrioventricular valve (AVV) surgery on patient outcomes after Fontan operation remains unclear. We sought to determine the effect of ventricular dominance and previous AVV surgery on transplantation-free survival and long-term AVV competency in patients with atrioventricular septal defect (AVSD) and Fontan circulation.
We conducted a retrospective study of 1703 patients in the Australia and New Zealand Fontan Registry, who survived Fontan operation between 1987 and 2021.
Of 174 patients with AVSD, 60% (105/174) had right ventricular (RV) dominance and 40% (69/174) had left ventricular (LV) dominance. The cumulative incidence of moderate or greater AVV regurgitation at 25 years after Fontan operation in patients with LV dominance was 56% (95% CI, 35%-72%), compared with 54% (95% CI, 40%-67%) in patients with RV dominance (P = .6). Nonetheless, transplantation-free survival at 25 years in patients with LV dominance was 94% (95% CI, 86%-100%), compared with 67% (95% CI, 52%-87%) in patients with RV dominance (hazard ratio, 5.9; 95% CI, 1.4-25.4; P < .01). Of note, transplantation-free survival was not different in patients who underwent AVV surgery before or at Fontan completion compared with those who did not (15 years: 81% [95% CI, 62%-100%] vs 88% [95% CI, 81%-95%]; P = .3).
In patients with AVSD and Fontan circulation the rate of moderate or greater common AVV regurgitation is similar in those with LV and RV dominance. RV dominance, rather than previous AVV surgery, is a risk factor for death or transplantation.
心室优势及既往房室瓣（AVV）手术对Fontan手术后患者预后的影响尚不清楚。我们旨在确定心室优势及既往AVV手术对房室间隔缺损（AVSD）并Fontan循环患者无移植生存及长期AVV功能的影响。
我们对澳大利亚和新西兰Fontan注册中心的1703例在1987年至2021年间存活Fontan手术的患者进行了一项回顾性研究。
在174例AVSD患者中，60%（105/174）为右心室（RV）优势，40%（69/174）为左心室（LV）优势。左心室优势患者Fontan手术后25年中重度或更严重AVV反流的累积发生率为56%（95%CI，35%-72%），右心室优势患者为54%（95%CI，40%-67%）（P = 0.6）。尽管如此，左心室优势患者25年无移植生存率为94%（95%CI，86%-100%），右心室优势患者为67%（95%CI，52%-87%）（风险比，5.9；95%CI，1.4-25.4；P < 0.01）。值得注意的是，在Fontan手术完成前或同时接受AVV手术的患者与未接受该手术的患者相比，无移植生存率并无差异（15年：81%[95%CI，62%-100%]对88%[95%CI，81%-95%]；P = 0.3）。
在AVSD并Fontan循环患者中，左心室和右心室优势患者中重度或更严重的共同AVV反流发生率相似。右心室优势而非既往AVV手术是死亡或移植的危险因素。
