Department of Cardiac Surgery, University Hospital of Ghent, Ghent, Belgium.
Department of Pediatric Cardiology, University Hospital of Ghent, Ghent, Belgium.
Eur J Cardiothorac Surg. 2023 Oct 4;64(4). doi: 10.1093/ejcts/ezad305.
The aim of this study was to evaluate the relationship between atrioventricular valve and ventricular function in Fontan survivors, including the effect of atrioventricular valve surgery.
Analysis focused on transplant-free survival and the need for atrioventricular valve surgery in single ventricle patients after Fontan completion. Longitudinal echocardiographic examination of long-term valve and ventricular function was performed.
Fontan completion was performed in 113 patients, having a right univentricular morphology in 33.6%, a left ventricle morphology in 62.8% and ambiguous in 3.6%. Perioperative mortality was 2.7% (n = 3). Within a median follow-up of 16.3 years (interquartile range 10.6-23.6), transplant-free survival was 96.1 ± 1.9% and 90.4 ± 5.8% at 10-25 years. Twenty AV valve procedures were performed in 14 (12.4%) children, respectively, pre-Fontan (n = 10), per-Fontan (n = 8) and post-Fontan (n = 2), resulting in a cumulative incidence of AV valve surgery is 5.7 ± 2.2% and 12.3 ± 3.2% at 1-5 years. Atrio-ventricular valve function deteriorated over time [hazard ratio (HR) 1.112, 95% confidence interval (CI) 1.089-1.138, P < 0.001], without difference for valve morphology (P = 0.736) or ventricular dominance (P = 0.484). AV valve dysfunction was greater in patients requiring AV valve surgery (HR 20.383, 95% CI 6.223-36.762, P < 0.001) but showed a comparable evolution since repair to those without valve surgery (HR 1.070, 95% CI 0.987-1.160, P = 0.099). Progressive time-related ventricular dysfunction was observed (HR 1.141, 95% CI 1.097-1.182, P < 0.001), significantly less in left ventricle-dominance (HR 0.927, 95% CI 0.860-0.999, P = 0.047) but more after AV valve surgery (HR 1.103, 95% CI 1.014-1.167, P = 0.022).
In a homogeneously treated Fontan population, 25-year transplant-free survival is encouraging. Atrio-ventricular valve surgery was necessary in 12.4%, resulting mostly in a durable valve function. However, a slow time-related decline of atrioventricular valve function as of ventricular function is worrisome, evoking a role for additional heart failure therapy.
本研究旨在评估 Fontan 术后患者房室瓣与心室功能之间的关系，包括房室瓣手术的影响。
分析重点为单心室患者 Fontan 完成后无移植生存率和房室瓣手术需求。对长期房室瓣和心室功能进行了纵向超声心动图检查。
113 例患者接受了 Fontan 手术，其中右心室单心室形态占 33.6%，左心室形态占 62.8%，形态不明确占 3.6%。围手术期死亡率为 2.7%（n=3）。中位随访 16.3 年（四分位距 10.6-23.6），无移植生存率在 10-25 年分别为 96.1±1.9%和 90.4±5.8%。14 例（12.4%）患儿分别在术前（n=10）、Fontan 术后即刻（n=8）和术后（n=2）行 20 次房室瓣手术，累积房室瓣手术发生率为 5.7±2.2%和 12.3±3.2%，1-5 年分别为 5.7±2.2%和 12.3±3.2%。房室瓣功能随时间推移而恶化（风险比[HR]1.112，95%置信区间[CI]1.089-1.138，P<0.001），但与房室瓣形态（P=0.736）或心室优势（P=0.484）无关。需要房室瓣手术的患者房室瓣功能障碍更严重（HR 20.383，95%CI 6.223-36.762，P<0.001），但与未行瓣膜手术的患者相比，其房室瓣功能具有类似的演变趋势（HR 1.070，95%CI 0.987-1.160，P=0.099）。观察到进行性时间相关的心室功能障碍（HR 1.141，95%CI 1.097-1.182，P<0.001），左心室优势患者的发生率明显较低（HR 0.927，95%CI 0.860-0.999，P=0.047），但在房室瓣手术后更高（HR 1.103，95%CI 1.014-1.167，P=0.022）。
在同质治疗的 Fontan 人群中，25 年无移植生存率令人鼓舞。12.4%的患者需要房室瓣手术，主要是为了获得持久的瓣膜功能。然而，房室瓣功能和心室功能随时间的缓慢下降令人担忧，提示需要进一步的心力衰竭治疗。
