Clinical Challenges

Carcinoid Heart Disease

Anne Marie C. Flaherty

carcinoid, heart, gastrointestinal, tumor
ONF 2014, 41(6), 687-691. DOI: 10.1188/14.ONF.687-691

The patient, C.P., is a 59-year-old woman who was diagnosed with metastatic carcinoid of the terminal ileum in May 2003. In June 2003, she underwent an extensive resection including hemicolectomy, cholecystectomy, distal pancreatectomy, and splenectomy with metastatic disease in her pancreas, mesentery, and liver. She had been treated with octreotide, everolimus, oxaliplatin, and multiple hepatic artery embolizations in the past eight years and, most recently, capecitabine and bevacizumab with monthly octreotide. She has had intermittent pleural effusions not requiring intervention and a trace pericardial effusion. Her tumor is functional, meaning it demonstrates hormonal hypersecretion which causes flushing, diarrhea, bronchospasm, and abdominal pain.

Jump to a section


    Anthony, L. B. (2013). Practical guide to supportive care of patients with functional neuroendocrine tumors. Seminars in Oncology, 40, 45-55. doi:10.1053/j.seminoncol.2012.11.002
    Anthony, L. B., & Vinik, A. I. (2011). Evaluating the characteristics and the management of patients with neuroendocrine tumors receiving octreotide LAR during a 6 year period. Pancreas, 40, 987-994. doi:10.1097/MPA.0b013e31821f66b4
    Bernheim, A. M., Connolly, H. M., Hobday, T. J., Abel, M. D., & Pellikka, P. A. (2007). Carcinoid heart disease. Progress in Cardiovascular Disease, 49, 439-451. doi:10.1016/j.pcad.2006.12.002
    Bhattacharyya, S., Toumpanakis, C., Chilkunda, D., Caplin, M. E., & Davar, J. (2011). Risk factors for the development and progression of carcinoid heart disease. American Journal of Cardiology, 107, 1221-1226. doi:10.1016/j.amjcard.2010.12.025
    Burke, A., Thomas, R., Elsayed, A., & Sobin, L. H. (1997). Carcinoids of the jejunum and ileum: An immunohistochemical and clinicopathologic study of 167 cases. Cancer, 79, 1086-1093. doi:10.1002/(SICI)1097-0142(19970315)79:6<1086::AID-CNCR5>3.0.CO;2-E
    Castillo, J. G., Filsoufi, F., Rahmanian, P. B., Anyanwu, A., Zacks, J. S., Warner, R. R., & Adams, D. H. (2008). Early and late results of valvular surgery for carcinoid heart disease. Journal of the American College of Cardiology, 51, 1507-1509. doi:10.1016/j.jacc.2007.12.036
    Chowsanitphon, J. (2010). Carcinoid tumor. Oncology Nursing Forum, 37, 677-679. doi:10.1188/10.ONF.677-679
    Connolly, H. M. (2014). Carcinoid heart disease. Retrieved from
    Connolly, H. M., Nishimura, R. A., Smith, H. C., Pellikka, P. A., Mullany, C. J., & Kvols, L. K. (1995). Outcome of cardiac surgery for carcinoid heart disease. Journal of the American College of Cardiology, 25, 410-416. doi:10.1016/0735-1097 (94)00374-Y
    Fox, F. J., & Khattar, R. S. (2004). Carcinoid heart disease: Presentation, diagnosis and management. Heart, 90, 1224-1228. doi:10.1136/hrt.2004.040329
    Gardner-Roehnelt, N. M. (2012). Update on the management of neuroendocrine tumors: Focus on somatostatin antitumor effects. Clinical Journal of OncologyNursing, 16, 56-64. doi:10.1188/12.CJON.56-64
    Goldfinger, S. D., & Strosberg, J. R. (2014). Clinical characteristics of carcinoid tumors. Retrieved from
    Gustafsson, B. I., Hauso, O., Drozdov, I., Kidd, I. M., & Modlin, M. (2008). Carcinoid heart disease. International Journal of Cardiology, 129, 318-324. doi:10.1016/j.ijcard.2008.02.019
    Kulke, M. H., Benson, A. B., Bergsland, E., Berlin, J. D., Blaszkowsky, L. S., Choti, M. A., … Dwyer, M. A. (2012). Neuroendocrine tumors. Journal of the National Comprehensive Cancer Network, 10, 724-764.
    Kulke, M. H., & Raut, C. P. (2008). Neuroendocrine tumors of the gastrointestinal tract. In D. P. Kelsen, J. M. Daly, S. E. Kern, B. Levin, J. E. Tepper, & E. Van Cutsem (Eds.), Principles and practice of gastrointestinal oncology (2nd ed., pp. 653-670). Philadelphia, PA: Lippincott Williams and Wilkins.
    Lundin, L., Norheim, I., Landelius, J., Oberg, K., & Theodorsson-Norheim, E. (1988). Carcinoid heart disease: Relationship of circulating vasoactive substance to ultrasound-detectable cardiac abnormalities. Circulation, 77, 264-269.
    Makridis, C., Oberg, K., Juhlin, C., Rastad, J., Johansson, H., Lorelius, L. E., & Akerstrom, G. (1990). Surgical treatment of mid-gut carcinoid tumors. World Journal of Surgery, 14, 377-383. doi:10.1007/BF01658532
    Modlin, I. M., Lye, K. D., & Kidd, M. (2003). A 5-decade analysis of 13,715 carcinoid tumors. Cancer, 97, 934-959. doi:10.1002/cncr.11105
    National Comprehensive Cancer Network. (2014). NCCN guidelines: Carcinoid tumors [v.2.14]. Retrieved from
    Oates, J. A., Melmon, K., Sjoerdsma, A., Gillespie, L., & Mason, D. T. (1964). Release of a kinin peptide in the carcinoid syndrome. Lancet, 1, 514-517. doi:10.1016/S0140-6736(64)92907-1
    Pandya, U. H., Pellikka, P. A., Enriquez-Sarano, M., Edwards, W. D., Schaff, H. V., & Connolly, H. M. (2002). Metastatic carcinoid tumor to the heart: Echocardiographic-pathologic study of 11 patients. Journal of the American College of Cardiology, 40, 1328-1332. doi:10.1016/S0735-1097(02)02109-5
    Patel, D., Moses, M., Escarcega, R., & Bove, A. A. (2014). Carcinoid heart disease: Current understanding and future directions. American Heart Journal, 167, 789-795. doi:10.1016/j.ahj.2014.03.018
    Pellikka, P. A., Tajik, A. J., Khandheria, B. K., Seward, J. B., Callahan, J. A., Pitot, H. C., & Kvols, L. K. (1993). Carcinoid heart disease: Clinical and echocardiographic spectrum in 74 patients. Circulation, 87, 1188-1196. doi:10.1161/01.CIR.87.4.1188
    Reichlin, S. (1983). Somatostatin. New England Journal of Medicine, 309, 1495-1501.
    Rinke, A., Muller, H. H., Schade-Brittinger, C., Klose, K. J., Barth, P., Wied, M., … Arnold, R. (2009). Placebo-controlled, double blind, prospective, randomized study on the effect of octreotide LAR in the control of tumor growth in patients with metastatic neuroendocrine midgut tumors: A report from the PROMID Study Group. Journal of Clinical Oncology, 27, 4656-4663. doi:10.1200/JCO.2009.22.8510
    Scarsbrook, A. F., Ganeshan, A., Statham, J., Thakker, R. V., Weaver, A., Talbot, D., … Phillips, R. R. (2007). Anatomic and functional imaging of metastatic carcinoid tumors. Radiographics, 27, 455-477. doi:10.1148/rg.272065058
    Strosberg, J. (2012). Neuroendocrine tumours of the small intestine. Best Practice and Research in Clinical Gastroenterology, 26, 755-773. doi:10.1016/j.bpg.2012.12.002
    Yildiz, O., & Serdengecti, S. (2012). Gastrointestinal neuroendocrine tumors. In A. Lowell (Ed.), Neuroendocrine tumor (pp. 47-64). InTech. Retrieved from