Abstract A 61-year-old man with metastatic cancer was initially seen with a murmur that became increasingly louder. On examination, there was a right ventricular heave and a grade 2/6 holosystolic murmur along the left sternal edge at the fifth intercostal space that increased on inspiration. The liver was enlarged and pulsatile, and pedal edema was present. What is your diagnosis? Carcinoid syndrome is caused by the biologically active substances produced by metastatic carcinoid tumor tissue. The syndrome includes flushing of the skin, diarrhea, bronchospasm, and thickening of the endocardial structures of the right side of the heart. This latter feature is responsible for the abnormalities seen by echocardiography. The Figure shows thickened tricuspid leaflets that are retracted and do not meet properly during systolic closure. Most primary carcinoid tumors are located in the intestinal tract, most frequently in the small intestine. The vast majority are found in the appendix, where they are usually References 1. Grahame-Smith DG: The carcinoid syndrome. Am J Cardiol 1968; 21:376-387.Crossref 2. Roberts WC, Sjoerdsma A: The cardiac disease associated with the carcinoid syndrome (carcinoid heart disease). Am J Med 1965;36:5-34.Crossref 3. Roberts WC, Dangel JC, Bulkley BH: Nonrheumatic valvular cardiac disease: A clinicopathologic survey of 27 different conditions causing valvular dysfunction , in Likoff W (ed): Cardiovascular Clinics: Valvular Heart Disease . Philadelphia, FA Davis Co, 1973, vol 5, p 334. 4. Wroblewski EM, Tajik AJ, Callahan JA, et al: Distinctive echocardiographic features of carcinoid heart disease. Circulation 1981;64( (suppl) ):14. 5. Baker BJ, McNee VD, Scovil JA, et al: Tricuspid insufficiency in carcinoid heart disease: An echocardiographic description. Am Heart J 1981;101:107-108.Crossref 6. Schoen FJ, Hausner RJ, Howell JF, et al: Porcine heterograft valve replacement in carcinoid heart disease. J Thorac Cardiovasc Surg 1981;81:100-105.
Archives of Internal Medicine – American Medical Association
Published: Sep 1, 1982