Background:Small intestinal neuroendocrine tumors (SI-NETs) are characterized by carcinoid syndrome and carcinoid heart disease (CHD). The aim of the present study was to identify early risk markers for carcinoid heart disease and survival in a prospective median-term follow-up setting.Methods:We measured 5-HIAA and cumulative 5-HIAA exposure (Cum-5-HIAA) based on repeated measurements, proBNP, vascular function, hepatic tumor load, and transthoracic echocardiography (TTE) at baseline and during the median 5-year follow-up. Of 65 patients with SI-NETs, 54 patients underwent a prospective follow-up. In addition, survival was evaluated during the median follow-up of 6 years.Results:At baseline, three patients had CHD. During the median follow-up of 5 years, two patients (4%) developed CHD. Cum-5-HIAA and proBNP correlated with CHD (Westberg score, Spearman’s ρ = 0.32 and 0.31, respectively). Cum-5-HIAA had a superior diagnostic capability, predicting CHD in receiver operator characteristic analysis with an AUC of 0.98 (95% CI: 0.94–1.00) and outperformed proBNP, chromogranin A (CgA), and individual serum 5-HIAA measurements (AUC = 0.75, 0.85, and 0.91, respectively). Minor changes in valve regurgitation were frequently detected but did not correlate with vascular function. Regurgitation increased or decreased in 29% of tricuspid and 30% of pulmonic valves. CHD, hepatic tumor load, serum 5-HIAA, and elevated aortic pulse wave velocity (PWV) were associated with increased mortality in SI-NET patients.Conclusions:Cum-5-HIAA is a promising biomarker for CHD risk and outperformed other biomarkers. CHD and hepatic tumor load are the strongest predictors of mortality. PWV is a novel predictor of survival. The incidence of CHD was low among the SI-NET patients, probably reflecting successful treatment regimens.
背景：小肠神经内分泌肿瘤（SI-NETs）以类癌综合征和类癌心脏病（CHD）为特征。本研究旨在通过前瞻性中期随访，识别类癌心脏病及生存期的早期风险标志物。 方法：我们在基线和为期5年的中位随访期间，基于重复测量评估了5-HIAA、累积5-HIAA暴露量（Cum-5-HIAA）、proBNP、血管功能、肝肿瘤负荷以及经胸超声心动图（TTE）。在65例SI-NET患者中，54例接受了前瞻性随访。此外，在中位6年的随访期间对生存情况进行了评估。 结果：基线时，3例患者存在CHD。在为期5年的中位随访期间，2例患者（4%）新发CHD。Cum-5-HIAA和proBNP与CHD相关（Westberg评分，Spearman's ρ分别为0.32和0.31）。Cum-5-HIAA具有更优的诊断能力，在受试者工作特征分析中预测CHD的曲线下面积（AUC）为0.98（95% CI: 0.94–1.00），优于proBNP、嗜铬粒蛋白A（CgA）以及单次血清5-HIAA测量（AUC分别为0.75、0.85和0.91）。瓣膜反流的轻微变化常被检出，但与血管功能无相关性。29%的三尖瓣和30%的肺动脉瓣反流程度有所增加或减少。CHD、肝肿瘤负荷、血清5-HIAA以及升高的主动脉脉搏波速度（PWV）与SI-NET患者死亡率增加相关。 结论：Cum-5-HIAA是预测CHD风险的一个有前景的生物标志物，其表现优于其他标志物。CHD和肝肿瘤负荷是预测死亡率的最强因素。PWV是一个新的生存预测指标。SI-NET患者中CHD发生率较低，可能反映了治疗方案的成效。
肿瘤(癌症)患者之家