Carcinoid Heart Disease and Review of Literature
DOI:
https://doi.org/10.17140/HROJ-4-132Keywords:
Carcinoid heart disease (CHD), Neuroendocrine tumors (NETs), Valvular heart disease, Carcinoid syndromeAbstract
Carcinoid Heart Disease (CHD) is one of the rare causes of acquired valvular heart disease.
CHD develops as a systemic manifestation of metastatic neuroendocrine tumors (NETs). When
these tumors metastasize to the liver, they can release vasoactive amines into the systemic circulation,
which can result in carcinoid syndrome and CHD. The key for diagnosing CHD is a
thorough history and physical examination supplemented with characteristic echocardiographic
findings. We report a case with classic echocardiographic features of CHD, which unfolded
the diagnosis of metastatic carcinoid tumor.
References
Modlin IM, Sandor A. An Analysis of 8305 cases of carcinoid tumors. Cancer. 1997; 79: 813-829. doi: 10.1002/(SICI)1097- 0142(19970215)79:4<813::AID-CNCR19>3.0.CO;2-2
Kulke MH, Mayer RJ. Carcinoid tumors. N Eng J Med. 1999; 340: 858-868. doi: 10.1056/NEJM199903183401107
Pellika PA, Tajik AJ, Khandheria BK, et al. Carcinoid heart disease: Clinical and echocardiographic spectrum in 74 pa-tients. Circulation. 1993; 87: 1188-1196. doi: 10.1161/01.CIR. 87.4.1188
Fox DJ, Khattar RS. Carcinoid heart disease: Presentation, diagnosis, and management. Heart. 2004; 90(10): 1224-1228. doi: 10.1136/hrt.2004.040329
Jonathan S, Gardner N, Kvols L. Survival and prognostic factor analysis of 146 metastatic neuroendocrine tumors of the mid-gut. Neuroendocrinology. 2009; 89(4): 471-476. doi: 10.1159/000197899
Waltenberger J, Lundin L, Oberg K, et al. Involvement of transforming growth factor-beta in the formation of fibrotic le-sions in carcinoid heart disease. Am J Pathol. 1993; 142(1): 71.
Jian B, Xu J, Connolly J, et al. Serotonin-induced up-regu-lation of transforming growth factor-β1 via G-protein signal transduction in aortic valve interstitial cells. Am J Pathol. 2002; 161(6): 2111-2121. doi: 10.1016/S0002-9440(10)64489-6
Møller JE, Connolly HM, Rubin J, Seward JB, Modesto K, Pellikka PA. Factors associated with progression of carcinoid heart disease. N Engl J Med. 2003; 348(11): 1005-1015. doi: 10.1056/NEJMoa021451
Simula DV, Edwards WD, Tazelaar HD, Connolly HM, Schaff HV. Surgical pathology of carcinoid heart disease: A study of 139 valves from 75 patients spanning 20 years. Mayo Clin Proc. 2002; 77(2): 139-147.
Connolly HM, Schaff HV, Mullany CJ, Rubin J, Abel MD, Pellikka PA. Surgical management of left-sided carcinoid heart disease. Circulation. 2001; 104(12 Suppl 1): I36-I40. doi: 10.1161/hc37t1.094898
Howard RJ, Drobac M, Rider WD, et al. Carcinoid heart dis-ease: diagnosis by two-dimensional echocardiography. Circula-tion. 1982; 66(5): 1059-1065. Web site. http://circ.ahajournals. org/content/circulationaha/66/5/1059.full.pdf. Accessed Sep-tember 5, 2016
Kvols LK, Moertel CG, O’Connell MJ, et al. Treatment of the malignant carcinoid syndrome. N Engl J Med. 1986; 315(11): 663-666. doi: 10.1056/NEJM198609113151102
Kvols LK. Metastatic carcinoid tumors and the carcinoid syndrome: A selective review of chemotherapy and hormonal therapy. Am J Med. 1986; 81(6): 49-55. doi: 10.1016/0002- 9343(86)90584-X
Lamberts SWJ. A guide to the clinical use of the soma-tostatin analogue SMS 201-995 (Sandostatin). Acta Endocri-nol (Copenh). 1987; 116(Suppl 4): S54-S66. doi: 10.1530/ acta.0.115S054
Martin JK, Moertel CG, Adson MA, Schutt AJ. Surgical treat-ment of functioning metastatic carcinoid tumors. Arch Surg. 1983; 118(5): 537-542. doi: 10.1001/archsurg.1983.01390050021004
Bernheim AM, Connolly HM, Rubin J, et al. Role of hepatic resection for patients with carcinoid heart disease. Mayo Clin Proc. 2008; 83(2): 143-150. doi: 10.4065/83.2.143
Møller JE, Pellikka PA, Bernheim AM, et al. Prognosis of Carcinoid heart disease analysis of 200 cases over two de-cades. Circulation. 2005; 112(21): 3320-3327. doi: 10.1161/ CIRCULATIONAHA.105.553750
Connolly HM, Nishimura RA, Smith HC, et al. Outcome of cardiac surgery for carcinoid heart disease. J Am Coll Cardiol. 1995; 25(2): 410-416. doi: 10.1016/0735-1097(94)00374-Y
Connolly HM, Schaff HV, Abel MD, et al. Early and late outcomes of surgical treatment in carcinoid heart disease. J Am Coll Cardiol. 2015; 66(20): 2189-2196. doi: 10.1016/j. jacc.2015.09.014
Downloads
Published
How to Cite
Issue
Section
License

This work is licensed under a Creative Commons Attribution 4.0 International License.
All content published in Heart Research (HROJ) is licensed under the Creative Commons Attribution 4.0 International License (CC BY 4.0). This license allows anyone to copy, distribute, transmit, and adapt the work — including for commercial purposes — provided appropriate credit is given to the original author(s) and source.