Carcinoid heart disease: case and literature review

Abstract

We present the case of a 69-year-old woman with a metastatic neuroendocrine tumour of the ileum and severe tricuspid valve regurgitation due to carcinoid valvulopathy. Carcinoid heart disease arises in the context of metastatic serotonin-producing neuroendocrine tumours and typically presents as structural and functional abnormalities of tricuspid (TV) or pulmonary valve (PV). Carcinoid heart disease arises from a low-grade neuroendocrine tumour derived from serotonin-producing enterochromaffin cells that reduces the mobility of the leaflets. Following the development of this type of heart failure, the prognosis is unfavourable and patients usually die as a result of heart failure and not because of the metastatic disease. Our patient was not considered a candidate for valvular surgery because of the progressive nature of the malignancy. However, surgical valve replacement is a therapeutic option that, although it has a significant mortality, needs to be considered in selected patients and leads to improvement in functional capacity and survival.