Hendriks Paul M, van den Bosch Annemien E, Geenen Laurie W, Baggen Vivan J M, Eindhoven Jannet A, Kauling Robert M, Cuypers Judith A A E, Boersma Eric, Roos-Hesselink Jolien W
Department of Cardiology, Erasmus MC, University Medical Centre Rotterdam, Rotterdam, The Netherlands.
ERN-GUARD-Heart: European Reference Network for Rare and Low Prevalence Complex Diseases of the Heart.
JACC Adv. 2024 Jul 27;3(9):101130. doi: 10.1016/j.jacadv.2024.101130. eCollection 2024 Sep.
The adult congenital heart disease (ACHD) population is growing and risk prediction is important to predict adverse outcome and consult patients during their lifecourse.
This study aims to describe the long-term prognostic value of blood biomarkers in ACHD.
In this prospective observational cohort study, 602 patients with moderate or complex ACHD were included (median age 32.5 years [IQR: 24.7-41.2], 42% female, 90% New York Heart Association I). N-terminal pro-brain natriuretic peptide (NT-proBNP), high-sensitive-troponin T, growth differentiation factor 15, high-sensitive-C-reactive protein, suppression of tumorigenicity-2 and galectin-3, as well as full blood count, renal function, LDL, and HDL were measured. Cox models were applied to relate the selected biomarkers with the primary end point of all-cause mortality and secondary end point of mortality or heart failure. Standardized HRs adjusted for relevant prognostic factors, including age, sex, and complexity of diagnosis, were reported.
Abnormal biomarker levels were present in 424 (70.4%) patients. During a median follow-up of 10.1 years, 41 (6.8%) patients died and 81 (13.5%) developed heart failure. Associations were observed between the primary and secondary end point and red cell distribution width, NT-proBNP, and growth differentiation factor 15. In a multibiomarker model, only NT-proBNP remained associated with mortality (HR: 2.74; 95% CI: 2.01-3.74). NT-proBNP significantly improved the C-statistic of the clinical prediction model (0.85-0.92). Based on NT-proBNP alone, low-risk patients could be identified. Patients with NT-proBNP <76 ng/L showed a 10-year heart failure-free survival of 98.5%.
Blood biomarkers have prognostic value in ACHD. NT-proBNP improves risk prediction and is able to identify low-risk patients. Its routine use should be implemented in ACHD.
成人先天性心脏病（ACHD）患者群体不断壮大，风险预测对于预测不良结局以及在患者的生命历程中为其提供咨询至关重要。
本研究旨在描述血液生物标志物在ACHD中的长期预后价值。
在这项前瞻性观察性队列研究中，纳入了602例中度或复杂性ACHD患者（中位年龄32.5岁[四分位间距：24.7 - 41.2]，42%为女性，90%为纽约心脏协会心功能I级）。检测了N末端脑钠肽前体（NT-proBNP）、高敏肌钙蛋白T、生长分化因子15、高敏C反应蛋白、抑瘤素M2和半乳糖凝集素3，以及全血细胞计数、肾功能、低密度脂蛋白和高密度脂蛋白。应用Cox模型将选定的生物标志物与全因死亡的主要终点以及死亡或心力衰竭的次要终点相关联。报告了针对包括年龄、性别和诊断复杂性等相关预后因素进行调整后的标准化风险比（HR）。
424例（70.4%）患者存在生物标志物水平异常。在中位随访10.1年期间，41例（6.8%）患者死亡，81例（13.5%）发生心力衰竭。观察到主要和次要终点与红细胞分布宽度、NT-proBNP和生长分化因子15之间存在关联。在多生物标志物模型中，只有NT-proBNP仍与死亡率相关（HR：2.74；95%置信区间：2.01 - 3.74）。NT-proBNP显著改善了临床预测模型的C统计量（从0.85提高到0.92）。仅基于NT-proBNP，就可以识别出低风险患者。NT-proBNP <76 ng/L的患者10年无心力衰竭生存率为98.5%。
血液生物标志物在ACHD中具有预后价值。NT-proBNP改善了风险预测，并且能够识别低风险患者。应在ACHD中常规应用NT-proBNP。
