Darragh Brady, MD; David Murphy, MD; Jonathan D. Dodd, MD, MSc, MRCPI, FFR(RCSI)
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KEY FACTS
Terminology
Imaging
Top Differential Diagnoses
Clinical Issues
TERMINOLOGY
Synonyms
Hyperserotonemia
Thorson-Bioerck syndrome
Argentaffinoma syndrome
Cassidy-Scholte syndrome
Hedinger’s syndrome (carcinoid heart disease)
Definitions
Carcinoid tumor secretion of vasoactive substances causing clinical syndrome of flushing (90%), diarrhea (70%), and bronchospasm (10%)
Develops from neuroendocrine cells of enterochromaffin cell origin in submucosa
Most have midgut origin (ileocecal region/appendix)
Origin can also be from lung due to common origin of foregut and respiratory diverticulum during 4th week of fetal development; 18% of time primary origin cannot be found
Clinical symptoms occur when secretory products are directly released into systemic circulation or when hepatic metabolism is overwhelmed
Cells secrete vasoactive substances
Serotonin [5-hydroxytryptamine (5-HT)] production is most prominent, especially in midgut tumors
Bradykinins, tachykinins, histamine, substance P, and adrenocorticotropic hormone (ACTH) also reported
Tricuspid and pulmonary valves are involved on upstream side where vasoactive substance levels are highest
2nd most common cause of tricuspid stenosis (there is always concomitant regurgitation)
Left side can be involved in pulmonary carcinoid or patent foramen ovale but less likely due to pulmonary deactivation of vasoactive substances
IMAGING
General Features
Radiographic Findings
CT Findings
MR Findings
Echocardiographic Findings
Angiographic Findings
Nuclear Medicine Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Gross Pathologic & Surgical Features
Microscopic Features
CLINICAL ISSUES
Presentation
Natural History & Prognosis
Treatment
Selected References
Davar J et al: Diagnosing and managing carcinoid heart disease in patients with neuroendocrine tumors: an expert statement. J Am Coll Cardiol. 69(10):1288-1304, 2017
Fani M et al: Somatostatin receptor antagonists for imaging and therapy. J Nucl Med. 58(Suppl 2):61S-6S, 2017
Goldberg E et al: Serotonin and catecholamines in the development and progression of heart valve diseases. Cardiovasc Res. 113(8):849-57, 2017
Lee ST et al: Theranostics of neuroendocrine tumors. Visc Med. 33(5):358-66, 2017
Basuroy R et al: Neuroendocrine tumors. Gastroenterol Clin North Am. 45(3):487-507, 2016
Shah S et al: Multimodal imaging of the tricuspid valve: normal appearance and pathological entities. Insights Imaging. 7(5):649-67, 2016
Kesarwani M et al: First-in-human transcatheter pulmonic valve implantation through a tricuspid valve bioprosthesis to treat native pulmonary valve regurgitation caused by carcinoid syndrome. JACC Cardiovasc Interv. 8(10):e161-3, 2015
Patel C et al: Carcinoid heart disease: current understanding and future directions. Am Heart J. 167(6):789-95, 2014
Rajiah P et al: CT and MRI of pulmonary valvular abnormalities. Clin Radiol. 69(6):630-8, 2014
Haugaa KH et al: Evaluation of right ventricular dysfunction by myocardial strain echocardiography in patients with intestinal carcinoid disease. J Am Soc Echocardiogr. 24(6):644-50, 2011
Sandmann H et al: Cardiovascular magnetic resonance imaging in the assessment of carcinoid heart disease. Clin Radiol. 64(8):761-6, 2009
Scarsbrook AF et al: Anatomic and functional imaging of metastatic carcinoid tumors. Radiographics. 27(2):455-77, 2007
Zuetenhorst JM et al: Metastatic carcinoid tumors: a clinical review. Oncologist. 10(2):123-31, 2005
Mollet NR et al: MRI and CT revealing carcinoid heart disease. Eur Radiol. 13 Suppl 4:L14-8, 2003
Pellikka PA et al: Carcinoid heart disease. Clinical and echocardiographic spectrum in 74 patients. Circulation. 87(4):1188-96, 1993
Related Anatomy
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Related Differential Diagnoses
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References
Tables
Tables
KEY FACTS
Terminology
Imaging
Top Differential Diagnoses
Clinical Issues
TERMINOLOGY
Synonyms
Hyperserotonemia
Thorson-Bioerck syndrome
Argentaffinoma syndrome
Cassidy-Scholte syndrome
Hedinger’s syndrome (carcinoid heart disease)
Definitions
Carcinoid tumor secretion of vasoactive substances causing clinical syndrome of flushing (90%), diarrhea (70%), and bronchospasm (10%)
Develops from neuroendocrine cells of enterochromaffin cell origin in submucosa
Most have midgut origin (ileocecal region/appendix)
Origin can also be from lung due to common origin of foregut and respiratory diverticulum during 4th week of fetal development; 18% of time primary origin cannot be found
Clinical symptoms occur when secretory products are directly released into systemic circulation or when hepatic metabolism is overwhelmed
Cells secrete vasoactive substances
Serotonin [5-hydroxytryptamine (5-HT)] production is most prominent, especially in midgut tumors
Bradykinins, tachykinins, histamine, substance P, and adrenocorticotropic hormone (ACTH) also reported
Tricuspid and pulmonary valves are involved on upstream side where vasoactive substance levels are highest
2nd most common cause of tricuspid stenosis (there is always concomitant regurgitation)
Left side can be involved in pulmonary carcinoid or patent foramen ovale but less likely due to pulmonary deactivation of vasoactive substances
IMAGING
General Features
Radiographic Findings
CT Findings
MR Findings
Echocardiographic Findings
Angiographic Findings
Nuclear Medicine Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Gross Pathologic & Surgical Features
Microscopic Features
CLINICAL ISSUES
Presentation
Natural History & Prognosis
Treatment
Selected References
Davar J et al: Diagnosing and managing carcinoid heart disease in patients with neuroendocrine tumors: an expert statement. J Am Coll Cardiol. 69(10):1288-1304, 2017
Fani M et al: Somatostatin receptor antagonists for imaging and therapy. J Nucl Med. 58(Suppl 2):61S-6S, 2017
Goldberg E et al: Serotonin and catecholamines in the development and progression of heart valve diseases. Cardiovasc Res. 113(8):849-57, 2017
Lee ST et al: Theranostics of neuroendocrine tumors. Visc Med. 33(5):358-66, 2017
Basuroy R et al: Neuroendocrine tumors. Gastroenterol Clin North Am. 45(3):487-507, 2016
Shah S et al: Multimodal imaging of the tricuspid valve: normal appearance and pathological entities. Insights Imaging. 7(5):649-67, 2016
Kesarwani M et al: First-in-human transcatheter pulmonic valve implantation through a tricuspid valve bioprosthesis to treat native pulmonary valve regurgitation caused by carcinoid syndrome. JACC Cardiovasc Interv. 8(10):e161-3, 2015
Patel C et al: Carcinoid heart disease: current understanding and future directions. Am Heart J. 167(6):789-95, 2014
Rajiah P et al: CT and MRI of pulmonary valvular abnormalities. Clin Radiol. 69(6):630-8, 2014
Haugaa KH et al: Evaluation of right ventricular dysfunction by myocardial strain echocardiography in patients with intestinal carcinoid disease. J Am Soc Echocardiogr. 24(6):644-50, 2011
Sandmann H et al: Cardiovascular magnetic resonance imaging in the assessment of carcinoid heart disease. Clin Radiol. 64(8):761-6, 2009
Scarsbrook AF et al: Anatomic and functional imaging of metastatic carcinoid tumors. Radiographics. 27(2):455-77, 2007
Zuetenhorst JM et al: Metastatic carcinoid tumors: a clinical review. Oncologist. 10(2):123-31, 2005
Mollet NR et al: MRI and CT revealing carcinoid heart disease. Eur Radiol. 13 Suppl 4:L14-8, 2003
Pellikka PA et al: Carcinoid heart disease. Clinical and echocardiographic spectrum in 74 patients. Circulation. 87(4):1188-96, 1993
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