Greminger P, Hess O M, Müller A E, von Segesser L, Schneider J, Sütsch G, Siegenthaler W, Heitz P U
Departement für Innere Medizin, Universitätsspital Zürich, Schweiz.
Klin Wochenschr. 1991 Feb 6;69(3):128-33. doi: 10.1007/BF01795957.
A female patient suffering from a bronchial neuroendocrine tumor with unilateral left-sided carcinoid heart disease is reported. Repeated x-ray films of the chest showed a slowly growing lung tumor in the left lower lobe. The patient refused any diagnostic or therapeutic procedure to define the type of the tumor. During the follow-up of 24 years she developed severe mitral and moderate to severe aortic insufficiency, both invasively quantified by thermodilution techniques. During surgery for double valve replacement the patient died from left ventricular heart failure. Necropsy revealed the typical pattern of a bronchial neuroendocrine tumor without metastases. Examination of the heart disclosed the characteristic deposits of fibrous tissue on the cusps of both the mitral and the aortic valves whereas the right heart showed no abnormalities. Review of the literature suggests the unilateral left-sided carcinoid heart disease to be a very rare finding, its pathogenesis remains to be elucidated.
报告了一名患有支气管神经内分泌肿瘤并伴有左侧单侧类癌性心脏病的女性患者。胸部X线片复查显示左肺下叶有一个生长缓慢的肺部肿瘤。患者拒绝进行任何诊断或治疗程序以明确肿瘤类型。在24年的随访期间,她出现了严重的二尖瓣和中重度主动脉瓣关闭不全,两者均通过热稀释技术进行了有创量化。在进行双瓣膜置换手术时,患者死于左心室心力衰竭。尸检显示为典型的无转移支气管神经内分泌肿瘤模式。心脏检查发现二尖瓣和主动脉瓣叶上有特征性的纤维组织沉积,而右心未发现异常。文献回顾表明,左侧单侧类癌性心脏病是一种非常罕见的发现,其发病机制仍有待阐明。