Carcinoid heart disease from primary carcinoid tumour of the ovary. Haemodynamic and cine coronary angiocardiographic study after operation.

Eight cases of carcinoid heart disease associated with primary ovarian carcinoid tumour have been previously reported. A case is described in which rapid relief of all symptoms followed removal of an ovarian carcinoid argentaffin tumour. A diagnostic finding was the provocation offlushing by pressure on the ovarian mass on vaginal examination. Cardiological investigations, performed two and a half months after operation, are presented. A peculiar angiocardiographic feature was the loss of trabeculations of the right ventricle. Examples of primary carcinoid tumour of the ovary are being recognized with increasing frequency since the report of the first two cases by Stewart, Willis, and De Saram (I939) and more particularly after the classical description of the carcinoid syndrome in 1954 (Thorson et al.). Ghatterjee and Heather (I968) reviewed 31 cases up to I967. In the recent series of Haines (I97I), 6 cases out of 8 were considered primary. Primary ovarian car-cinoids commonly arise from an element of a benign cystic teratoma (dermoid cyst). When no teratomatous elements are found, a diligent search is necessary to rule' out ovarian metastasis from an extrinsic source, usually the small bowel. It is not known why some carcinoids release endocrine products while others do not. Chatterjee and Heather (I968) could find features of the carcinoid syndrome in I4 cases out of 3I, and Haines (197I) in 4 cases out of 8 at the time of operation. Carcinoid heart disease complicates primary carcinoid of the ovary only in some cases and is therefore more rare. Chatterjee and Heather (I968) reported a case and reviewed three others up to I965). A fifth case with necropsy findings is that of Bancroft, O'Brien, and Tickner (I964). Since I967, 3 new cases have been reported (Ruckes and Stall-kamp, I967; Mouchet, Guivarc'h, and Voinchet, I969; Acar et al., I970). Three patients died without operation. At necropsy the typical endo-cardial fibrosis was found with pulmonary stenosis and tricuspid valvar disease. The 6 other patients underwent laparotomy with oophorectomy. Rapid relief of symptoms was obtained in all 6 patients. The ovary is a unique site for primary carcinoids because endocrine products of the tumour are drained directly into the systemic venous circulation. Thus the carcinoid syndrome is likely to appear early and before any metastasis has occurred. Therefore, radical surgical cure is possible. Unlike carcinoids of the ovary, carcinoids of the small intestine have their endocrine products drained into the liver where they are …