{"id":523597,"date":"2022-01-01T12:27:18","date_gmt":"2022-01-01T17:27:18","guid":{"rendered":"https:\/\/myendoconsult.com\/learn\/?p=523597"},"modified":"2023-03-16T06:06:41","modified_gmt":"2023-03-16T11:06:41","slug":"what-is-carcinoid-disease-of-the-heart","status":"publish","type":"post","link":"https:\/\/myendoconsult.com\/learn\/what-is-carcinoid-disease-of-the-heart\/","title":{"rendered":"What is Carcinoid Disease of the Heart"},"content":{"rendered":"

Carcinoid heart disease(CHD), has a prevalence rate ranging from 11-70% [1]. Patients can present with right-sided murmurs (tricuspid and pulmonary valves), peripheral edema, ascites, and other signs of right-sided heart failure[2].<\/p>\n

Carcinoid disease of the Heart<\/h2>\n

The most cited pathophysiologic mechanism happens to be a serotoninergic-mediated process.\u00a0The binding of serotonin to ubiquitous 5-HT2B<\/strong> receptors in the heart explains the clinical manifestations of CHD[1]. Serotonin and other vasoactive amines stimulate 5-HT2B receptors present on the surface of myofibroblasts.<\/p>\n

This leads to fibrosis and deposition of myxomatous substances in the endocardium. Telotristat, a novel tryptophan hydroxylase enzyme inhibitor, retarded CHD in two patients enrolled in the landmark TELESTAR (Telotristat Etiprate for Somatostatin Analogue Not Adequately Controlled Carcinoid Syndrome) <\/strong>phase III trial, further affirming the role of serotonin in this condition[1].<\/p>\n

Biosynthesis of serotonin by neuroendocrine cells<\/h2>\n

\"\"<\/p>\n

Fig. 1 <\/strong>Schematic diagram of the biosynthesis of serotonin. Tryptophan hydroxylase 1 (TH-1) is the rate-limiting enzyme in serotonin synthesis by neuroendocrine tumors and is expressed in various tissues, including the gastrointestinal tract, spleen, thymus, and pineal glands<\/em>[3]. Tryptophan is initially transported into the neuroendocrine cell (dashed arrow). Tryptophan is then converted into 5-hydroxytryptophan by tryptophan hydroxylase-1 enzyme <\/em>[1, 4]. The conversion of 5-hydroxytryptophan into serotonin (5-hydroxytryptamine, 5-HT) occurs in an intermediate decarboxylation step, which requires aromatic L-acid decarboxylase. 5-HT is packaged along with chromogranin A (a neuroendocrine tumor marker) and is then released into circulation (dotted arrow). 5-HT undergoes metabolism via various enzymatic conversion steps, which result in the formation of 5-HIAA(an inactive metabolite)(wavy arrow)<\/em>[1, 4]. (Redrawn and modified from Frazer et al. (1999) Serotonin. Basic Neurochemistry: Molecular, Cellular and Medical Aspects. 6th edition)<\/em><\/p>\n

References<\/h2>\n
    \n
  1. Ito T, Lee L, Jensen RT (2018) Carcinoid syndrome: recent advances, current status, and controversies. Current Opinion in Endocrinology, Diabetes and Obesity 25:22<\/li>\n
  2. Hassan SA, Banchs J, Iliescu C, Dasari A, Lopez-Mattei J, Yusuf SW (2017) Carcinoid heart disease. Heart 103:1488\u20131495<\/li>\n
  3. Matthes S, Bader M (2018) Peripheral Serotonin Synthesis as a New Drug Target. Trends Pharmacol Sci 39:560\u2013572<\/li>\n
  4. Frazer A, Hensler JG (1999) Serotonin. Lippincott-Raven Chapter 13:6th edition<\/li>\n<\/ol>\n

    <\/h4>\n","protected":false},"excerpt":{"rendered":"

    Carcinoid heart disease(CHD), has a prevalence rate ranging from 11-70% [1]. Patients can present with right-sided murmurs (tricuspid and pulmonary valves), peripheral edema, ascites, and other signs of right-sided heart failure[2]. Carcinoid disease of the Heart The most cited pathophysiologic mechanism happens to be a serotoninergic-mediated process.\u00a0The binding of serotonin to ubiquitous 5-HT2B receptors in […]<\/p>\n","protected":false},"author":1,"featured_media":523813,"comment_status":"open","ping_status":"open","sticky":false,"template":"","format":"standard","meta":{"footnotes":""},"categories":[98],"tags":[],"class_list":["post-523597","post","type-post","status-publish","format-standard","has-post-thumbnail","hentry","category-endocrine-reviews-medicine","post-wrapper","thrv_wrapper"],"_links":{"self":[{"href":"https:\/\/myendoconsult.com\/learn\/wp-json\/wp\/v2\/posts\/523597","targetHints":{"allow":["GET"]}}],"collection":[{"href":"https:\/\/myendoconsult.com\/learn\/wp-json\/wp\/v2\/posts"}],"about":[{"href":"https:\/\/myendoconsult.com\/learn\/wp-json\/wp\/v2\/types\/post"}],"author":[{"embeddable":true,"href":"https:\/\/myendoconsult.com\/learn\/wp-json\/wp\/v2\/users\/1"}],"replies":[{"embeddable":true,"href":"https:\/\/myendoconsult.com\/learn\/wp-json\/wp\/v2\/comments?post=523597"}],"version-history":[{"count":54,"href":"https:\/\/myendoconsult.com\/learn\/wp-json\/wp\/v2\/posts\/523597\/revisions"}],"predecessor-version":[{"id":4416015,"href":"https:\/\/myendoconsult.com\/learn\/wp-json\/wp\/v2\/posts\/523597\/revisions\/4416015"}],"wp:featuredmedia":[{"embeddable":true,"href":"https:\/\/myendoconsult.com\/learn\/wp-json\/wp\/v2\/media\/523813"}],"wp:attachment":[{"href":"https:\/\/myendoconsult.com\/learn\/wp-json\/wp\/v2\/media?parent=523597"}],"wp:term":[{"taxonomy":"category","embeddable":true,"href":"https:\/\/myendoconsult.com\/learn\/wp-json\/wp\/v2\/categories?post=523597"},{"taxonomy":"post_tag","embeddable":true,"href":"https:\/\/myendoconsult.com\/learn\/wp-json\/wp\/v2\/tags?post=523597"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}