Keuning Zoë A, Meccanici Frederike, Schreurs Bibi A, Veen Kevin, Bouma Berto J, Voskuil Michiel, Jongbloed Monique R M, van Melle Joost P, van Kimmenade Roland R J, Kapoor Rowina, Bozkurt Irem, Hirsch Alexander, Roos-Hesselink Jolien W, van den Bosch Annemien E
Cardiovascular Institute, Thorax Center, Department of Cardiology, Erasmus MC, Rotterdam, The Netherlands.
Department of Cardiology, Radboud University Medical Center, Nijmegen, The Netherlands.
Open Heart. 2025 Aug 27;12(2):e003534. doi: 10.1136/openhrt-2025-003534.
Previous research on congenital aortic stenosis (AS) mainly focused on children, while data on long-term outcomes in adults is scarce. Therefore, this study aims to evaluate outcomes in adult patients with congenital AS and identify prognostic markers for aortic valve replacement (AVR).
In this multicentre study, patients aged 18-55 years with congenital AS (peak velocity ≥2.5 m/s) registered in the Dutch CONCOR (Congential Cor Vitia) registry from expert centres for congenital heart disease between 2001 and 2019 were included. Exclusion criteria were severe aortic regurgitation (AR) or prior AVR. Associations with the composite endpoint of all-cause mortality and AVR were assessed with multivariable Cox regression.
Of the 427 included patients (median age 26 years, 62.5% male) median aortic peak velocity was 3.1 (IQR 2.7-3.6) m/s, 35% had moderate AR and 29% showed signs of left ventricular (LV) remodelling. During a median follow-up period of 7.9 (IQR 4.1-12.6) years, 7 patients died (1.6%) and 177 patients underwent AVR (41.5%). LV systolic dysfunction was the primary indication for AVR in three patients (1.7%). Peak velocity at baseline (HR 3.17, 95% CI 2.29 to 4.39), non-sinus rhythm (HR 3.12, 95% CI 1.62 to 6.02) and concentric LV geometry (HR 1.64, 95% CI 1.04 to 2.58) were associated with the primary endpoint beside age. Significant male-female differences were observed in prognostic factors for the primary endpoint.
AVR was often indicated in adult patients with congenital AS, even if the stenosis at baseline was mild. Moreover, monitoring LV remodelling alongside severity of AS is more important than focusing on systolic LV dysfunction.
先前关于先天性主动脉瓣狭窄（AS）的研究主要集中在儿童身上，而关于成人长期预后的数据却很稀少。因此，本研究旨在评估先天性AS成年患者的预后，并确定主动脉瓣置换术（AVR）的预后标志物。
在这项多中心研究中，纳入了2001年至2019年间在荷兰先天性心脏病专家中心的CONCOR（先天性心脏异常）登记处登记的18至55岁先天性AS患者（峰值流速≥2.5米/秒）。排除标准为严重主动脉瓣反流（AR）或既往AVR。采用多变量Cox回归评估与全因死亡率和AVR复合终点的相关性。
427例纳入患者（中位年龄26岁，62.5%为男性）的主动脉峰值流速中位数为3.1（四分位间距2.7 - 3.6）米/秒，35%有中度AR，29%有左心室（LV）重构迹象。在中位随访期7.9（四分位间距4.1 - 12.6）年期间，7例患者死亡（1.6%），177例患者接受了AVR（41.5%）。3例患者（1.7%）因LV收缩功能障碍接受AVR为主要指征。除年龄外，基线峰值流速（HR 3.17，95%CI 2.29至4.39）、非窦性心律（HR 3.12，95%CI 1.62至6.02）和向心性LV形态（HR 1.64，95%CI 1.04至2.58）与主要终点相关。在主要终点的预后因素方面观察到显著的男女差异。
先天性AS成年患者常需进行AVR，即使基线狭窄程度较轻。此外，监测LV重构以及AS的严重程度比关注LV收缩功能障碍更为重要。
