BackgroundThe development of carcinoid heart disease (CaHD) is still relatively unclear. It is difficult to define an optimal follow‐up for patients without any cardiac involvement at baseline. The aim of this study was to assess the prevalence and natural history of CaHD by annual echocardiographic examinations.Methods and ResultsWe studied 137 consecutive patients (61±12 years, 53% men) with proven digestive endocrine tumor and carcinoid syndrome between 1997 and 2017. All patients underwent serial conventional transthoracic echocardiographic studies. Right‐sided and left‐sided CaHD were systematically assessed. We used a previous validated echocardiographic scoring system of severity for the assessment of CaHD. An increase of 25% of the score was considered to be significant. Mean follow‐up was 54±45 months. Prevalence of CaHD was 27% at baseline and 32% at 5‐year follow‐up. Disease progression was reported in 28% of patients with initial CaHD followed up for >2 years (n=25). In patients without any cardiac involvement at baseline, occurrence of disease was 21%. CaHD occurred >5 years from the initial echocardiographic examination in 42% of our cases, especially in patients presenting with new recurrence of a digestive endocrine tumor. An increase of urinary 5‐hydroxyindoleacetic acid by 25% during follow‐up was identified as an independent predictor of CaHD occurrence during follow‐up (hazard ratio [HR], 5.81; 95% CI, 1.19–28.38; P=0.03), as well as a maximum value of urinary 5‐hydroxyindoleacetic acid >205 mg/24 h during follow‐up (HR, 8.41; 95% CI, 1.64–43.07; P=0.01).ConclusionsOur study demonstrates that in patients without initial CaHD, cardiac involvement may occur late and is related to serotonin. Our data emphasize the need for cardiologic follow‐up in patients with recurrence of the tumor process.

中文翻译：

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现代管理时代类癌心脏病的进展

背景类癌性心脏病（CaHD）的发展仍相对不清楚。对于基线时没有任何心脏受累的患者，很难确定最佳随访。本研究的目的是通过年度超声心动图检查评估 CaHD 的患病率和自然病程。方法和结果我们研究了 1997 年至 2017 年间确诊的消化内分泌肿瘤和类癌综合征的 137 名连续患者（61±12 岁，53% 男性）。所有患者均接受了一系列常规经胸超声心动图检查。对右侧和左侧 CaHD 进行了系统评估。我们使用以前经过验证的严重程度超声心动图评分系统来评估 CaHD。分数增加 25% 被认为是显着的。平均随访时间为 54±45 个月。CaHD 的患病率在基线时为 27%，在 5 年随访时为 32%。据报道，28% 的初始 CaHD 患者随访超过 2 年（n=25）。在基线时没有任何心脏受累的患者中，疾病的发生率为 21%。在我们 42% 的病例中，CaHD 发生在初次超声心动图检查后 5 年以上，特别是在出现消化内分泌肿瘤新复发的患者中。随访期间尿 5-羟基吲哚乙酸增加 25% 被确定为随访期间发生 CaHD 的独立预测因子（风险比 [HR]，5.81；95% CI，1.19–28.38；发病率为21%。在我们 42% 的病例中，CaHD 发生在初次超声心动图检查后 5 年以上，特别是在出现消化内分泌肿瘤新复发的患者中。随访期间尿 5-羟基吲哚乙酸增加 25% 被确定为随访期间发生 CaHD 的独立预测因子（风险比 [HR]，5.81；95% CI，1.19–28.38；发病率为21%。在我们 42% 的病例中，CaHD 发生在初次超声心动图检查后 5 年以上，特别是在出现消化内分泌肿瘤新复发的患者中。随访期间尿 5-羟基吲哚乙酸增加 25% 被确定为随访期间发生 CaHD 的独立预测因子（风险比 [HR]，5.81；95% CI，1.19–28.38；P = 0.03），以及随访期间尿 5-羟基吲哚乙酸的最大值>205 mg/24 h（HR，8.41；95% CI，1.64-43.07；P = 0.01）。结论我们的研究表明，在没有初始 CaHD 的患者，心脏受累可能发生较晚，并且与血清素有关。我们的数据强调了对肿瘤复发患者进行心脏随访的必要性。