类癌性心脏病

Carcinoid heart disease

类癌是一种主要发生于胃肠道,但也可位于胃肠外器官的能产生小分子多肽类或肽类激素的肿瘤。类癌综合征是由类癌细胞分泌、释放一些生物活性物质所引起的一组具有多种复杂症状、体征的症候群。类癌性心脏病是指类癌综合征累及心脏,引起的以右侧心脏瓣膜和心内膜病变为主的心脏病,多发生于类癌晚期出现肝脏转移的患者。由于生长抑素类似物的应用以及针对转移性肿瘤的治疗,类癌患者存活延长,应该考虑瓣膜外科治疗减轻心脏症状。

Carcinoid tumors are relatively rare neuroendocrine malignancies most commonly originating from enterochromaffin cells in the gastrointestinal tract. Carcinoid syndrome occurs when tumor cells metastasize to the liver, as the vasoactive substances produced are able to reach the systemic circulation via the hepatic vein. The development of carcinoid heart disease （CHD） is thought to be related to the vasoactive substances secreted by the metastatic tumor cells in the livcr,lvaching the right heart. This is associated with deposition of fibrous tissue on the endocardial surfaces of the heart. Several series have reported CHD in up to 70% of cases of carcinoid syn- drome. Somatostatin analogue use is a therapeutic cornerstone, effective in symptomatic control of the endocrine syndrome. In patients with severe cardiac involvement and well-controlled systemic disease,valve replacement surgery is an effective treatment modality that can relieve intractable symptoms and contribute to improved outcome.