2664 You're Breaking My Heart: Carcinoid Heart Disease

Abstract

INTRODUCTION: Neuroendocrine tumors (NET) are rare tumors affecting the gastrointestinal tract and lungs with ∼8,000 cases diagnosed in the US each year. About 80% of small intestinal NET have metastasized to the liver and present as carcinoid syndrome with flushing, diarrhea, bronchospasm and heart failure, as the liver is unable to metabolize vasoactive substances. Carcinoid heart disease can occur in ∼60% of patients with carcinoid syndrome and is a late complication that can be associated with significant morbidity due to right sided heart failure. CASE DESCRIPTION/METHODS: A 60 year old female with anxiety and breast cancer presented to an outside clinic with chronic diarrhea. She began experiencing 10–20 watery stools daily for over a year but lost 8 pounds over the last four months. An upper and lower endoscopy done for evaluation of chronic diarrhea was normal. Stool studies revealed a negative stool culture, clostridium difficile PCR and normal fecal elastase, but laboratory studies showed an elevated chromogranin A (23,940 ng/ml), serotonin (1668 ng/ml) and urinary 5-HIAA (112 mg/day). A CT of the abdomen revealed multiple liver lesions. Liver biopsy was non-diagnostic. Four months later she sought a second opinion but soon after developed new onset ascites and lower extremity edema so was admitted to the hospital for further evaluation. Echocardiogram showed severe tricuspid regurgitation with the leaflets tethered and fixed in an open position. Cardiac MRI showed late gadolinium enhancement of tricuspid valve leaflets which was consistent with carcinoid heart disease. Repeat liver biopsy revealed grade 2 well differentiated NET, with presumed small bowel origin. She received octreotide and underwent mechanical tricuspid valve replacement. DISCUSSION: Carcinoid syndrome is a rare finding but is important in the differential of chronic diarrhea. This case also portrays the prolonged natural history of carcinoid syndrome with the late complication of carcinoid heart disease. Inability of upper and lower endoscopy to locate small bowel carcinoids can lead to a delay in diagnosis, and in 18% of small bowel carcinoids the primary location is not identified. Early identification and treatment of NET tumors can avoid late morbid conditions such as carcinoid heart syndrome.