Department of Cardiothoracic Surgery, Mount Sinai Medical Center, New York, New York 10029-6574, USA.
Semin Thorac Cardiovasc Surg. 2012 Winter;24(4):254-60. doi: 10.1053/j.semtcvs.2012.11.003.
Carcinoid tumors are neuroendocrine tumors with an unpredictable clinical behavior. In the setting of hepatic metastases, the release of bioactive amines from the tumor into the systemic circulation results in carcinoid syndrome: a constellation of clinical symptoms, among which cutaneous flushing, gastrointestinal hypermotility, and cardiac involvement are the most frequent. Cardiac manifestations, also known as carcinoid heart disease, are secondary to a severe endocardial fibrotic reaction that leads to progressive valve thickening and retraction. Imaging studies commonly reveal severe right-sided valve disease, with fixed leaflets or cusps in a semiopen position. The replacement of the right-sided valves, including the patch enlargement of the right ventricular outflow tract, is currently the only definitive treatment to potentially improve quality of life and provide survival benefit. Although cardiac surgery has been traditionally reserved for those patients with symptomatic right ventricular failure, a significant trend toward improved surgical outcomes has triggered a more liberal referral for valve replacement during the past decade.
类癌肿瘤是具有不可预测临床行为的神经内分泌肿瘤。在肝转移的情况下，肿瘤向全身循环系统释放生物活性胺会导致类癌综合征：一系列临床症状，其中皮肤潮红、胃肠道高动力和心脏受累是最常见的。心脏表现也称为类癌心脏病，是严重心内膜纤维反应的继发表现，导致进行性瓣膜增厚和回缩。影像学研究通常显示严重的右侧瓣膜疾病，伴有固定的瓣叶或瓣膜在半开位置。目前，唯一能潜在改善生活质量并提供生存获益的治疗方法是更换右侧瓣膜，包括右心室流出道补片扩大。尽管心脏手术传统上保留给有症状的右心室衰竭患者，但过去十年中，手术结果的显著改善趋势促使更多患者在瓣膜置换时接受更宽松的转诊。
