Baker David W, Dennis Mark R, Zannino Diana, Schilling Chris, Moreno Patricia D, Bullock Andrew, Disney Patrick, Radford Dorothy J, Hornung Tim, Grigg Leeanne, d'Udekem Yves, Ayer Julian, Celermajer David S, Cordina Rachael
Department of Cardiology, Royal Prince Alfred Hospital, Sydney, New South Wales, Australia.
The University of Sydney Sydney Medical School, Sydney, New South Wales, Australia.
Heart. 2020 Nov 23. doi: 10.1136/heartjnl-2020-317619.
A high risk of morbidity and mortality is well documented in adults with a Fontan circulation. The difference in outcomes between those with and without significant morbidity at the time of transition to adult care has not been well characterised.
We analysed clinical outcomes in patients enrolled in the Australian and New Zealand Fontan Registry ≥16 years of age. Low risk (LR) Fontan patients were defined as those without history of sustained arrhythmia, thromboembolic event, transplantation, Fontan conversion, protein-losing enteropathy, plastic bronchitis, New York Heart Association class III/IV and/or moderate/severe atrioventricular valve regurgitation or ventricular dysfunction. Increased risk (IR) patients had one or more risk factor.
Inclusion criteria were met in 822 patients; mean age 26±8 years, median follow-up from age 16 was 9 years, 203 had atriopulmonary connection (APC) and 619 had total cavopulmonary connection (TCPC). Survival at 30 years was higher in the LR versus IR; 94% versus 82% (p=0.005), 89% versus 77% (p=0.07) for APC and 96% versus 89% (p=0.05) for TCPC. LR patients experienced less Fontan failure (HR 0.34, 95% CI 0.23 to 0.49, p<0.001) and ventricular dysfunction (HR 0.46, 95% CI 0.29 to 0.71, p=0.001) compared with IR patients. For LR TCPC patients, modelled survival projections at 60 years were 49%-67%.
Clinical outcomes for adolescents LR at transition to adult care are markedly superior to those who have established risk factors for Fontan failure, which is an important consideration when formulating individualised long-term risk estimates and counselling patients.
有文献充分记载，接受Fontan循环手术的成年患者发病和死亡风险较高。向成人护理过渡时，有显著发病情况和无显著发病情况的患者在预后方面的差异尚未得到充分描述。
我们分析了澳大利亚和新西兰Fontan注册中心登记的年龄≥16岁患者的临床结局。低风险（LR）Fontan患者定义为无持续性心律失常、血栓栓塞事件、移植、Fontan转换、蛋白丢失性肠病、塑形支气管炎、纽约心脏协会III/IV级和/或中度/重度房室瓣反流或心室功能障碍病史的患者。风险增加（IR）患者有一个或多个风险因素。
822例患者符合纳入标准；平均年龄26±8岁，从16岁起的中位随访时间为9年，203例采用心房肺连接（APC），619例采用全腔肺连接（TCPC）。LR组30年生存率高于IR组；分别为94%对82%（p = 0.005），APC组为89%对77%（p = 0.07），TCPC组为96%对89%（p = 0.05）。与IR患者相比，LR患者发生Fontan衰竭（HR 0.34，95%CI 0.23至0.49，p<0.001）和心室功能障碍（HR 0.46，95%CI 0.29至0.71，p = 0.001）的情况较少。对于LR TCPC患者，60岁时的生存预测模型显示为49%-67%。
向成人护理过渡时，青少年LR患者的临床结局明显优于已确立Fontan衰竭风险因素的患者，这在制定个体化长期风险评估和为患者提供咨询时是一个重要考虑因素。
