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Carcinoid heart disease

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Opinion statement

In patients with carcinoid heart disease, a multidisciplinary approach to patient care is required because treatment of the systemic disease and management of cardiac involvement must be considered at the same time. Somatostatin analogue use is a therapeutic cornerstone, effective in symptomatic control of the endocrine syndrome. Chemotherapy is of limited efficacy in patients with carcinoid tumors. Hepatic resection of metastases is the preferred treatment option and has been shown to result in improved outcome. It should be considered if at least 90% of the metastases in the liver are removable. Hepatic artery embolization is usually applied if a patient is not eligible for surgical debulking. Development and progression of carcinoid heart disease complicate the carcinoid syndrome and contribute to poor prognosis. In patients with severe cardiac involvement and well-controlled systemic disease, valve replacement surgery is an effective treatment modality that can relieve intractable symptoms and contribute to improved outcome.

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References and Recommended Reading

  1. Modlin IM, Sandor A: An analysis of 8305 cases of carcinoid tumors. Cancer 1997, 79:813–829.

    Article  PubMed  CAS  Google Scholar 

  2. Kulke MH, Mayer RJ: Carcinoid tumors. N Engl J Med 1999, 340:858–868.

    Article  PubMed  CAS  Google Scholar 

  3. Thorson A, Biorck G, Bjorkman G, Waldenstrom J: Malignant carcinoid of the small intestine with metastases to the liver, valvular disease of the right side of the heart (pulmonary stenosis and tricuspid regurgitation without septal defects), peripheral vasomotor symptoms, bronchoconstriction, and an unusual type of cyanosis; a clinical and pathologic syndrome. Am Heart J 1954, 47:795–817.

    Article  PubMed  CAS  Google Scholar 

  4. Isler P, Hedinger C: [Metastatic carcinoid of the small intestine with severe valvular defects especially in the right part of the heart and with pulmonary stenosis; a peculiar symptom complex]. Schweiz Med Wochenschr 1953, 83:4–7.

    PubMed  CAS  Google Scholar 

  5. Lundin L, Norheim I, Landelius J, et al.: Carcinoid heart disease: relationship of circulating vasoactive substances to ultrasound-detectable cardiac abnormalities. Circulation 1988, 77:264–269.

    PubMed  CAS  Google Scholar 

  6. Rajamannan NM, Caplice N, Anthikad F, et al.: Cell proliferation in carcinoid valve disease: a mechanism for serotonin effects. J Heart Valve Dis 2001, 10:827–831.

    PubMed  CAS  Google Scholar 

  7. Gustafsson BI, Tommeras K, Nordrum I, et al.: Long-term serotonin administration induces heart valve disease in rats. Circulation 2005, 111:1517–1522.

    Article  PubMed  CAS  Google Scholar 

  8. Pellikka PA, Tajik AJ, Khandheria BK, et al.: Carcinoid heart disease. Clinical and echocardiographic spectrum in 74 patients. Circulation 1993, 87:1188–1196.

    PubMed  CAS  Google Scholar 

  9. Robiolio PA, Rigolin VH, Harrison JK, et al.: Predictors of outcome of tricuspid valve replacement in carcinoid heart disease. Am J Cardiol 1995, 75:485–488.

    Article  PubMed  CAS  Google Scholar 

  10. Moertel CG: Treatment of the carcinoid tumor and the malignant carcinoid syndrome. J. Clin Oncol 1983, 1:727–740.

    PubMed  CAS  Google Scholar 

  11. Chaowalit N, Connolly HM, Schaff HV, et al.: Carcinoid heart disease associated with primary ovarian carcinoid tumor. Am J Cardiol 2004, 93:1314–1315.

    Article  PubMed  Google Scholar 

  12. Wilkowske MA, Hartmann LC, Mullany CJ, et al.: Progressive carcinoid heart disease after resection of primary ovarian carcinoid. Cancer 1994, 73:1889–1891.

    Article  PubMed  CAS  Google Scholar 

  13. Bernheim AM, Connolly HM, Pellikka PA: Carcinoid heart disease in patients without hepatic metastases. Am J Cardiol 2007, 99:292–294.

    Article  PubMed  Google Scholar 

  14. Roberts WC: A unique heart disease associated with a unique cancer: carcinoid heart disease. Am J Cardiol 1997, 80:251–256.

    Article  PubMed  CAS  Google Scholar 

  15. Ferrans VJ, Roberts WC: The carcinoid endocardial plaque; an ultrastructural study. Hum Pathol 1976, 7:387–409.

    Article  PubMed  CAS  Google Scholar 

  16. Simula DV, Edwards WD, Tazelaar HD, et al.: Surgical pathology of carcinoid heart disease: a study of 139 valves from 75 patients spanning 20 years. Mayo Clin Proc 2002, 77:139–147.

    PubMed  Google Scholar 

  17. Moller JE, Connolly HM, Rubin J, et al.: Factors associated with progression of carcinoid heart disease. N Engl J Med 2003, 348:1005–1015.

    Article  PubMed  Google Scholar 

  18. Denney WD, Kemp WE Jr, Anthony LB, et al.: Echocardiographic and biochemical evaluation of the development and progression of carcinoid heart disease. J Am Coll Cardiol 1998, 32:1017–1022.

    Article  PubMed  CAS  Google Scholar 

  19. Robiolio PA, Rigolin VH, Wilson JS, et al.: Carcinoid heart disease. Correlation of high serotonin levels with valvular abnormalities detected by cardiac catheterization and echocardiography. Circulation 1995, 92:790–795.

    PubMed  CAS  Google Scholar 

  20. Feldman JM: Urinary serotonin in the diagnosis of carcinoid tumors. Clin Chem 1986, 32:840–844.

    PubMed  CAS  Google Scholar 

  21. Moller JE, Pellikka PA, Bernheim AM, et al.: Prognosis of carcinoid heart disease: analysis of 200 cases over two decades. Circulation 2005, 112:3320–3327.

    Article  PubMed  Google Scholar 

  22. Kvols LK, Moertel CG, O’Connell MJ, et al.: Treatment of the malignant carcinoid syndrome. Evaluation of a long-acting somatostatin analogue. N Engl J Med 1986, 315:663–666.

    Article  PubMed  CAS  Google Scholar 

  23. Ansell SM, Pitot HC, Burch PA, et al.: A phase II study of high-dose paclitaxel in patients with advanced neuroendocrine tumors. Cancer 2001, 91:1543–1548.

    Article  PubMed  CAS  Google Scholar 

  24. Kvols LK, Reubi JC: Metastatic carcinoid tumors and the malignant carcinoid syndrome. Acta Oncol 1993, 32:197–201.

    PubMed  CAS  Google Scholar 

  25. Ritzel U, Leonhardt U, Stockmann F, Ramadori G: Treatment of metastasized midgut carcinoids with dacarbazine. Am J Gastroenterol 1995, 90:627–631.

    PubMed  CAS  Google Scholar 

  26. Que FG, Nagorney DM, Batts KP, et al.: Hepatic resection for metastatic neuroendocrine carcinomas. Am J Surg 1995, 169:36–42; discussion 42–43.

    Article  PubMed  CAS  Google Scholar 

  27. McEntee GP, Nagorney DM, Kvols LK, et al.: Cytoreductive hepatic surgery for neuroendocrine tumors. Surgery 1990, 108:1091–1096.

    PubMed  CAS  Google Scholar 

  28. Lehnert T: Liver transplantation for metastatic neuroendocrine carcinoma: an analysis of 103 patients. Transplantation 1998, 66:1307–1312.

    Article  PubMed  CAS  Google Scholar 

  29. Connolly HM, Nishimura RA, Smith HC, et al.: Outcome of cardiac surgery for carcinoid heart disease. J Am Coll Cardiol 1995, 25:410–416.

    Article  PubMed  CAS  Google Scholar 

  30. Godwin JD 2nd: Carcinoid tumors. An analysis of 2,837 cases. Cancer 1975, 36:560–569.

    Article  PubMed  Google Scholar 

  31. Vaughan DJ, Brunner MD: Anesthesia for patients with carcinoid syndrome. Int Anesthesiol Clin 1997, 35:129–142.

    Article  PubMed  CAS  Google Scholar 

  32. Warner RR, Mani S, Profeta J, Grunstein E: Octreotide treatment of carcinoid hypertensive crisis. Mt Sinai J Med 1994, 61:349–355.

    PubMed  CAS  Google Scholar 

  33. Propst JW, Siegel LC, Stover EP: Anesthetic considerations for valve replacement surgery in a patient with carcinoid syndrome. J Cardiothorac Vasc Anesth 1994, 8:209–212.

    Article  PubMed  CAS  Google Scholar 

  34. Claure RE, Drover DD, Haddow GR, et al.: Orthotopic liver transplantation for carcinoid tumour metastatic to the liver: anesthetic management. Can J Anaesth 2000, 47:334–337.

    Article  PubMed  CAS  Google Scholar 

  35. Connolly HM, Pellikka PA: Carcinoid heart disease. Curr Cardiol Rep 2006, 8:96–101.

    Article  PubMed  Google Scholar 

  36. Kubota A, Yamada Y, Kagimoto S, et al.: Identification of somatostatin receptor subtypes and an implication for the efficacy of somatostatin analogue SMS 201-995 in treatment of human endocrine tumors. J Clin Invest 1994, 93:1321–1325.

    PubMed  CAS  Google Scholar 

  37. Moertel CG: Karnofsky memorial lecture. An odyssey in the land of small tumors. J Clin Oncol 1987, 5:1502–1522.

    PubMed  CAS  Google Scholar 

  38. Rubin J, Ajani J, Schirmer W, et al.: Octreotide acetate long-acting formulation versus open-label subcutaneous octreotide acetate in malignant carcinoid syndrome. J Clin Oncol 1999, 17:600–606.

    PubMed  CAS  Google Scholar 

  39. Eriksson B, Oberg K: Summing up 15 years of somatostatin analog therapy in neuroendocrine tumors: future outlook. Ann Oncol 1999, 10(Suppl 2):S31–S38.

    Article  PubMed  Google Scholar 

  40. Harris AG, O’Dorisio TM, Woltering EA, et al.: Consensus statement: octreotide dose titration in secretory diarrhea. Diarrhea Management Consensus Development Panel. Dig Dis Sci 1995, 40:1464–1473.

    Article  PubMed  CAS  Google Scholar 

  41. Ansell SM, Mahoney MR, Green EM, Rubin J: Topotecan in patients with advanced neuroendocrine tumors: a phase II study with significant hematologic toxicity. Am J Clin Oncol 2004, 27:232–235.

    Article  PubMed  CAS  Google Scholar 

  42. Oberg K, Norheim I, Lundqvist G, Wide L: Cytotoxic treatment in patients with malignant carcinoid tumors. Response to streptozocin—alone or in combination with 5-FU. Acta Oncol 1987, 26:429–432.

    PubMed  CAS  Google Scholar 

  43. Eriksson BK, Larsson EG, Skogseid BM, et al.: Liver embolizations of patients with malignant neuroendocrine gastrointestinal tumors. Cancer 1998, 83:2293–2301.

    Article  PubMed  CAS  Google Scholar 

  44. Brown KT, Koh BY, Brody LA, et al.: Particle embolization of hepatic neuroendocrine metastases for control of pain and hormonal symptoms. J Vasc Interv Radiol 1999, 10:397–403.

    Article  PubMed  CAS  Google Scholar 

  45. Ahlman H, Westberg G, Wangberg B, et al.: Treatment of liver metastases of carcinoid tumors. World J Surg 1996, 20:196–202.

    Article  PubMed  CAS  Google Scholar 

  46. Ruszniewski P, Rougier P, Roche A, et al.: Hepatic arterial chemoembolization in patients with liver metastases of endocrine tumors. A prospective phase II study in 24 patients. Cancer 1993, 71:2624–2630.

    Article  PubMed  CAS  Google Scholar 

  47. Sutcliffe R, Maguire D, Ramage J, et al.: Management of neuroendocrine liver metastases. Am J Surg 2004, 187:39–46.

    Article  PubMed  Google Scholar 

  48. Osborne DA, Zervos EE, Strosberg J, et al.: Improved outcome with cytoreduction versus embolization for symptomatic hepatic metastases of carcinoid and neuroendocrine tumors. Ann Surg Oncol 2006, 13:572–581.

    Article  PubMed  Google Scholar 

  49. Moertel CG, Johnson CM, McKusick MA, et al.: The management of patients with advanced carcinoid tumors and islet cell carcinomas. Ann Intern Med 1994, 120:302–309.

    PubMed  CAS  Google Scholar 

  50. Grazi GL, Cescon M, Pierangeli F, et al.: Highly aggressive policy of hepatic resections for neuroendocrine liver metastases. Hepatogastroenterology 2000, 47:481–486.

    PubMed  CAS  Google Scholar 

  51. Chamberlain RS, Canes D, Brown KT, et al.: Hepatic neuroendocrine metastases: does intervention alter outcomes? J Am Coll Surg 2000, 190:432–445.

    Article  PubMed  CAS  Google Scholar 

  52. Sarmiento JM, Heywood G, Rubin J, et al.: Surgical treatment of neuroendocrine metastases to the liver: a plea for resection to increase survival. J Am Coll Surg 2003, 197:29–37.

    Article  PubMed  Google Scholar 

  53. McDonald ML, Nagorney DM, Connolly HM, et al.: Carcinoid heart disease and carcinoid syndrome: successful surgical treatment. Ann Thorac Surg 1999, 67:537–539.

    Article  PubMed  CAS  Google Scholar 

  54. Connolly HM, Schaff HV, Mullany CJ, et al.: Carcinoid heart disease: impact of pulmonary valve replacement in right ventricular function and remodeling. Circulation 2002, 106:151–156.

    Google Scholar 

  55. Caplin ME, Buscombe JR, Hilson AJ, et al.: Carcinoid tumour. Lancet 1998, 352:799–805.

    Article  PubMed  CAS  Google Scholar 

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Authors and Affiliations

  1. Division of Cardiovascular Diseases, Mayo Clinic, 200 First Street SW, Rochester, MN, 55905, USA

    Heidi M. Connolly

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  1. Alain M. Bernheim
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  2. Heidi M. Connolly
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  3. Patricia A. Pellikka
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Correspondence to Heidi M. Connolly.

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Bernheim, A.M., Connolly, H.M. & Pellikka, P.A. Carcinoid heart disease. Curr Treat Options Cardio Med 9, 482–489 (2007). https://doi.org/10.1007/s11936-007-0043-0

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