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Carcinoid heart disease: The rare and obscure becomes screened and well known

Published:February 12, 2022DOI:https://doi.org/10.1016/j.amjsurg.2022.02.003
      Carcinoid heart disease (CHD) is an insidious complication of carcinoid syndrome (CS) where myoproliferative plaques accumulate on the tricuspid and pulmonic valves as well as on the right ventricular endocardium and pulmonary artery intima.
      • Grozinsky-Glasberg S.
      • Grossman A.
      • Gross D.
      Carcinoid heart disease: from pathophysiology to treatment- ‘something in the way it moves.
      The pathophysiology has historically been obscure, but neuroendocrine tumor experts have recently concluded, supported by both human clinical data and animal studies, that the plaques are likely induced by elevated levels of 5-hydroxytrytophan (serotonin) or other substances secreted by the tumor such as prostaglandins, bradykinin, and substance P.
      • Grozinsky-Glasberg S.
      • Grossman A.
      • Gross D.
      Carcinoid heart disease: from pathophysiology to treatment- ‘something in the way it moves.
      ,
      • Davar J.
      • Connolly H.
      • Caplin M.
      • et al.
      Diagnosing and managing carcinoid heart disease in patients with neuroendocrine tumors.
      In more severe cases CHD may progress to right heart failure within several months.
      • Dobson R.
      • Burgess M.
      • Valle J.
      • et al.
      Serial surveillance of carcinoid heart disease: factors associated with echocardiographic progression and mortality.
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