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      Carcinoid Heart Disease: Pathophysiology, Pathology, Clinical Manifestations, and Management

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          Carcinoid heart disease (CHD) is a rare and potentially lethal manifestation of an advanced carcinoid (neuroendocrine) tumor. The pathophysiology of CHD is related to vasoactive substances secreted by the tumor, of which serotonin is most prominent in the pathophysiology of CHD. Serotonin stimulates fibroblast growth and fibrogenesis, which can lead to cardiac valvular fibrosis. CHD primarily affects right heart valves, causing tricuspid and pulmonic regurgitation and less frequently stenosis of these valves. Left heart valves are usually spared because vasoactive substances such as serotonin are enzymatically inactivated in the lung vasculature. The pathology of CHD is characterized by plaque-like deposition of fibrous tissue on valvular cusps, leaflets, papillary muscles, chordae, and ventricular walls. Symptomatic CHD usually presents between 50 and 70 years of age, initially as dyspnea and fatigue. Echocardiography is the mainstay of imaging and demonstrates thickened right heart valves with limited mobility and regurgitation. Treatment focuses on control of the underlying carcinoid syndrome, targeting subsequent valvular heart disease and managing consequent heart failure. Surgical valve replacement and catheter-directed valve procedures may be effective for selected patients with CHD.

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          Most cited references 64

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            Carcinoid tumors. An analysis of 2,837 cases.

            Two thousand eight hundred thirty-seven cases of carcinoid tumor from files at the National Cancer Institute were analyzed statistically. Tumors were found in the lung, ovary, and biliary and gastrointestinal tracts. Most were in the appendix, rectum, and ileum. Age-adjusted incidence rates were higher for black males, except for lung carcinoids. Carcinoids showed several differences from other kinds of tumor, including a low age for appendiceal and lung cases and low male/female and black/white ratios in the lung. Percentages of concurrent neoplasms and multiple carcinoids were low compared to other series. Five-year relative survival rates ranged from 99% (appendix) to 33% (sigmoid colon). Survival for colon cases was not so low as expected on the basis of the high rate of metastasis. Some appendiceal carcinoids were metastatic and may have killed 1 patient. Findings are compared with other studies.
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              Metastatic carcinoid tumors: a clinical review.

              Carcinoid tumors are neuroendocrine tumors derived from enterochromaffin cells, which are widely distributed in the body. They can originate from any location in the body, but they are traditionally described as originating from the foregut, midgut, and hindgut. Although the overall incidence of carcinoid tumors appears to have increased in the past decades, the prognosis for patients with metastatic carcinoid tumors has improved during the last decade. Due to longer survival times, complications, such as carcinoid heart disease, and new metastatic patterns, like skin and bone metastases, may become more important features of carcinoid disease. Therapy focused on these complications should be part of the management. Combining new diagnostic and treatment modalities in metastatic carcinoid patients may result in better quality of life and longer survival times. The increasing number of therapeutic options and diagnostic procedures requires a multidisciplinary approach, with decisions made in multidisciplinary meetings focused on "tailor-made" therapy based on patients' specific conditions. Because carcinoid tumors are uncommon, effort should be made to treat these patients in specialized centers and for these centers to join together in multicenter studies.

                Author and article information

                S. Karger AG
                January 2021
                16 October 2020
                : 146
                : 1
                : 65-73
                Division of Cardiovascular Medicine, Department of Internal Medicine, University of California (Davis) Medical Center, Sacramento, California, USA
                Author notes
                *Ezra A. Amsterdam, University of California (Davis) Medical Center, 4860 Y Street, Ellison Building, Suite 2820, Sacramento, CA 95817 (USA), eaamsterdam@ucdavis.edu
                507847 Cardiology 2021;146:65–73
                © 2020 S. Karger AG, Basel

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                Page count
                Figures: 2, Tables: 2, Pages: 9
                Valvular Heart Disease: Review Article


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