P839 The lucky unlucky patient: a case report

Abstract

Abstract Introduction Carcinoid tumors are rare neuroendocrine malignancies arising from the neural crest amine precursor uptake decarboxylation cells and are most commonly (90%) located in the gastrointestinal system, the most malignant arising from the ileum. Approximately 50% of patients with carcinoid syndrome will develop carcinoid heart disease, which is commonly characterized by the thickening and retraction of the tricuspid ± the pulmonary valves resulting in significant regurgitation ± stenosis. Hepatic metastasis must be present for the carcinoid heart disease to develop. In order to develop left-sided heart valve lesions, communication between the left and right heart chambers must be present. Case presentation A 50-year-old female patient with a known neuroendocrine tumor of the ileum with hepatic and peritoneal metastasis presented at our facility for evaluation of the heart valves. She was diagnosed with the neuroendocrine tumor in 2017, a resection of the intestinal ileocecal segment and peritoneal nodules was done in the same year (stadium pT3N1M1b ki 67 2%), the tumor was angio and neuroinvasive. The disease was treated with somatostatin analogues with minimal regression of the disease. She started peptide receptor radionuclide therapy and completed 3 cycles. In October 2018 an increment of one of the hepatic lesions was documented, with other hepatic lesions remaining of stable dimensions. Due to the recent appearance of dyspnea in mild to moderate physical activities she was sent to our facility for echocardiography. The transthoracic and transesophageal echocardiography revealed severe tricuspid regurgitation with mild stenosis (tricuspid anatomic area 3.5 cm², max gradient 7 mmHg, mean gradient 4 mmHg) caused by thickened and retracted cusps with a significant coaptation deficit. Severe stenosis and insufficiency of the pulmonary valve was also noted (maximum gradient 22 mmHg, mean gradient 15 mmHg, pulmonary valve area 0.5 cm²), with a rapid deceleration regurgitation jet limited to protodiastole, caused by thickened and retracted pulmonary valve leaflets. The interatrial septum exhibited an aneurysm with a patent foramen ovale (PFO) and mild left-to-right shunt. Despite the shunt left-sided heart valves were not affected, therefore we recommended a replacement of the tricuspid and pulmonary valves with an intraoperative closure of PFO and intraoperative verification of the efficacy of the surgical closure. Conclusion Carcinoid heart disease is an important cardiac complication of the neuroendocrine neoplasms. Communication between the right and left heart chambers (for example a PFO) must be present for the development of left heart valve lesions. Our patient had a PFO with a mild left to right shunt, but fortunately no left sided lesions were found. Abstract P839 Figure. Carcinoid heart disease