The blue bloater: a tale of cyanosis, platypnoea and right heart failure in a patient with an abdominal mass

An elderly woman in her 60s presented with generalised anasarca due to right heart failure, platypnoea and central cyanosis. On investigation, she was found to have dilatation of the right ventricle and right atrium, thickened and non-coapting tricuspid valve leaflets with severe organic low pressure tricuspid regurgitation and a small patent foramen ovale shunting right to left. Further evaluation revealed elevated serum chromogranin levels and 24-hour urinary 5-hydroxy indole acetic acid levels which suggested the possibility of a carcinoid-secreting tumour. A whole body PET scan revealed an ovarian mass with high uptake of the tracer, thus confirming the diagnosis of ovarian carcinoid with carcinoid heart disease. We discuss the intricacies of this case and then briefly review the literature on carcinoid heart disease. After initial decongestion, she underwent left salpingo-oophorectomy with tricuspid valve replacement and aortic valve repair in the same session and at a follow-up of 4 years is doing well and is disease free (Summary Figure).