• Janez Ravnik Klinični oddelek za kardiologijo Klinični center Zaloška 7 1525 Ljubljana
  • Maja Podlesnik Klinični oddelek za kardiologijo Klinični center Zaloška 7 1525 Ljubljana
  • Saša Markovič Klinični oddelek za gastroenterologijo Klinični center Japljeva 2 1525 Ljubljana
  • Mirta Koželj Klinični oddelek za kardiologijo Klinični center Zaloška 7 1525 Ljubljana
Keywords: carcinoid tumors, carcinoid syndrome, carcinoid heart disease, diagnostics, echocardiography


Background. Carcinoid heart disease is a rare heart disease which affects endocard and heart valves on the right side of heart. It affects only patients with manifested carcinoid syndrome, which is thought to be the consequence of secretory active metastases of carcinoid tumour. The carcinoid endocardial plaques cause structural changes of tricuspid and pulmonic valve and later on their stenosis and/or insufficiency.

Patients and methods. In this article we introduce a carcinoid valve heart disease (CVHD) scoring system for easier end exact echocardiographic diagnostics. Four echocardiographic parameters are beeing estimated: structural changes of tricuspid valve, tricuspid valve regurgitation, stenosis of pulmonic valve and pulmonic valve regurgitation.

Conclusions. The scoring system allows us to make an early diagnosis and evaluation of progression of carcinoid heart disease, which is very important for planning the treatment process. Our experiences confirm the usefulness of this scoring system in echocardiographic follow–up of patients with carcinoid syndrome.


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Kulke MH, Mayer RJ. Carcinoid tumors. N Engl J Med 1999; 340: 858–68.

Modlin IM, Sandor A. An analysis of 8305 cases of carcinoid tumors. Cancer 1997; 79: 813–29.

Soga J, Yakuwa Y. Bronchopulmonary carcinoids: An analysis of 1,875 reported cases with special reference to a comparison between typical carcinoids and atypical varieties. Ann Thorac Cardiovasc Surg 1999; 5: 211–9.

Kaplan LM. Endocrine tumors of the gastrointestinal tract and pancreas. In: Fauci AS, Braunwald E, Isselbacher KJ, Wilson JD, Martin JB, Kasper DL eds. Harrison’s principles of internal medicine. 14th ed. New York: McGraw-Hill, 1998: 584–92.

Kvols LK. Metastatic carcinoid tumors and the malignant carcinoid syndrom. Ann NY Acad Sci 1994; 733: 464–70.

Anderson AS, Krauss D, Lang R. Cardiovascular complications of malignant carcinoid disease. Am Heart 1997; 134: 693–702.

Öberg K. Carcinoid tumors: Current concepts in diagnosis and treatment. The Oncologist 1998; 3: 339–45.

Robiolio PA, Rigolin VH, Wilson JS, Harrison K, Sanders LL, Bashore TM, Feldman JM. Carcinoid heart disease: Correlation of high serotonin levels with valvular abnormalities detected by cardiac catheterization and echocardiography. Circulation 1995; 92: 790–5.

Kvols LK. Metastatic carcinoid tumors and the malignant carcinoid syndrom. Ann NY Acad Sci 1994; 733: 464–70.

Kocijančič A. Karcinoid. In: Kocijančič A, Mrevlje F eds. Interna medicina. 2nd ed. Ljubljana: DZS, 1998: 431–2.

Lundin L, Norheim I, Landelius J, Öberg K, Theodorsson-Norheim E. Carcinoid heart disease: relationship of circulating vasoactive substances to ultrasound-detectable cardiac abnormalities. Circulation 1988; 77: 264–9.

Pellikka PA, Tajik AJ, Khandheria BK, Seward JB, Callahan JA, Pitot HC, Kvols LK. Carcinoid heart disease: clinical and echocardiographic spectrum in 74 patients. Circulation 1993; 87: 1188–96.

Roberts WC. A unique heart disease associated with a unique cancer: carcinoid heart disease. Am J Cardiol 1997; 80: 251–6.

Himelman RB, Schiller NB. Clinical and echocardiographic comparison of patients with the carcinoid syndrome with and without carcinoid heart disease. Am J Cardiol 1989; 63: 347–52.

Lundlin L, Landelius J. Echocardiography for carcinoid heart disease. Ann NY Acad Sci 1994; 733: 437–45.

Denney WD, Kemp WE, Anthony LB, Oates JA, Byrd BF. Echocardiographic and biochemical evaluation of development and progression of carcinoid heart disease. J Am Coll Cardiol 1998; 32: 1017–22.

Kupfahl C, Pink D, Friedrich K, Zurbrügg HR, Neuss M, Warnecke C, Fielitz J, Graf K, Fleck E, Regitz-Zagrosek V. Angiotensin II directly increases transforming growth factor β1 and osteopontin and indirectly affects collagen mRNA expression in the human heart. Cardiovasc Res 2000; 46: 463–75.

Taimor G, Schlüter KD, Frischkopf K, Flesch M, Rosenkranz S, Piper HM. Autocrine regulation of TGF β expression in adult cardiomyocytes. J Mol Cell Cardiol 1999; 31: 2127–36.

Mirowitz SA, Guterrez FR. MR and CT diagnosis of carcinoid heart disease. Chest 1993; 103: 630–1.

The tricuspid valve. In: Kerut EK, McIlwain EF, Plotnick GD eds. Handbook of echo-Doppler interpretation. Armonk, New York: Futura Publishing Company, 1996: 107–13.

Callahan JA, Wroblewski EM, Reeder GS, Edwards WD, Seward JB, Tajik AJ. Echocardiographic features of carcinoid heart disease. Am J Cardiol 1982; 50: 762–8.

Lejko-Zupanc T, Koželj M, Kranjec I, Pikelj F. Right-sided endocarditis: clinical and echocardiographic characteristics. J Clin Bas Cardiol 1999; 2: 81–1.

How to Cite
Ravnik J, Podlesnik M, Markovič S, Koželj M. ECHOCARDIOGRAPHIC DIAGNOSTICS OF CARCINOID HEART DISEASE. ZdravVestn [Internet]. 1 [cited 22Nov.2019];71(9). Available from:
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