Zoë A Keuning, Frederike Meccanici, Bibi A Schreurs, Kevin Veen, Berto J Bouma, Michiel Voskuil, Monique R M Jongbloed, Joost P van Melle, Roland R J van Kimmenade, Rowina Kapoor, Irem Bozkurt, Alexander Hirsch, Jolien W Roos-Hesselink, Annemien E van den Bosch
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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.</p><p><strong>Results: </strong>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. 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引用次数: 0
摘要
背景：以往关于先天性主动脉瓣狭窄（AS）的研究主要集中在儿童，而成人的长期预后数据很少。因此，本研究旨在评估成人先天性AS患者的预后，并确定主动脉瓣置换术（AVR）的预后指标。方法：在这项多中心研究中，纳入了2001年至2019年期间在荷兰先天性心脏病专家中心的CONCOR （Congential Cor vita）登记处登记的18-55岁先天性AS（峰值速度≥2.5 m/s）患者。排除标准为严重主动脉反流（AR）或既往AVR。通过多变量Cox回归评估与全因死亡率和AVR复合终点的相关性。结果：纳入的427例患者（中位年龄26岁，62.5%为男性）主动脉峰速度中位数为3.1 (IQR 2.7-3.6) m/s， 35%为中度AR， 29%为左室重构征象。在中位随访7.9年（IQR 4.1-12.6）年期间，7例患者死亡（1.6%），177例患者发生AVR（41.5%）。左室收缩功能障碍是3例（1.7%）患者AVR的主要指征。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）和同心心室几何形状（HR 1.64, 95% CI 1.04至2.58）与年龄以外的主要终点相关。在主要终点的预后因素中观察到显著的男女差异。结论：成人先天性AS患者，即使基线时狭窄程度较轻，也常应用AVR。此外，监测左室重构和AS严重程度比关注收缩期左室功能障碍更重要。
Long-term clinical outcomes in adult congenital aortic stenosis: results from a national study.
Background: 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).
Methods: 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.
Results: 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.
Conclusion: 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.
期刊介绍：
Open Heart is an online-only, open access cardiology journal that aims to be “open” in many ways: open access (free access for all readers), open peer review (unblinded peer review) and open data (data sharing is encouraged). The goal is to ensure maximum transparency and maximum impact on research progress and patient care. The journal is dedicated to publishing high quality, peer reviewed medical research in all disciplines and therapeutic areas of cardiovascular medicine. Research is published across all study phases and designs, from study protocols to phase I trials to meta-analyses, including small or specialist studies. Opinionated discussions on controversial topics are welcomed. Open Heart aims to operate a fast submission and review process with continuous publication online, to ensure timely, up-to-date research is available worldwide. The journal adheres to a rigorous and transparent peer review process, and all articles go through a statistical assessment to ensure robustness of the analyses. Open Heart is an official journal of the British Cardiovascular Society.
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