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Cardiology189 papers

Carcinoid syndrome

Last edited: 4/14/2026

Overview

Carcinoid syndrome results from the systemic effects of vasoactive substances, particularly serotonin, secreted by advanced neuroendocrine tumors, often involving liver metastases. It can lead to characteristic symptoms and complications such as valvular heart disease, particularly affecting the right heart valves. 12

Diagnosis

  • Clinical Symptoms: Flushing, diarrhea, bronchospasm, and right-sided heart failure.
  • Imaging: CT scans for primary tumor and metastases; echocardiography for valvular involvement.
  • Laboratory Tests: Elevated serotonin levels, low tryptophan levels (indicative of pellagra in some cases).
  • Endoscopy and Biopsy: Essential for identifying primary carcinoid tumors, especially in the gastrointestinal tract. 183
  • Management

  • First-Line Treatment: Octreotide for symptom control and reducing hormone secretion.
  • Adjunctive Therapies: Interventional radiology for hypervascular tumors; surgical resection for localized disease.
  • Supportive Care: Management of valvular heart disease, including echocardiography monitoring and potential valve replacement.
  • Nutritional Support: Nicotinamide for treating pellagra secondary to carcinoid syndrome. 73
  • Special Populations

  • Comorbidities: Right-sided heart failure management critical in patients with carcinoid heart disease. 4
  • No Specific Data: Limited information on carcinoid syndrome in pregnancy, pediatrics, or elderly populations from provided abstracts. 123
  • Key Recommendations

  • Initiate octreotide therapy for symptom control and to reduce hormone secretion in patients with carcinoid syndrome (Evidence: Strong 7).
  • Regular echocardiograms are essential for monitoring carcinoid heart disease, particularly tricuspid and pulmonic valve involvement (Evidence: Moderate 14).
  • Consider surgical intervention for localized primary tumors and metastases when feasible (Evidence: Expert opinion 8).
  • Provide targeted nutritional support, such as nicotinamide, for patients presenting with pellagra-like symptoms (Evidence: Moderate 3).
  • References

    1 Jin C, Sharma AN, Thevakumar B, Majid M, Al Chalaby S, Takahashi N et al.. Carcinoid Heart Disease: Pathophysiology, Pathology, Clinical Manifestations, and Management. Cardiology 2021. link 2 Killu AM, Newman DB, Miranda WR, Maleszewski JJ, Pellikka P, Schaff HV et al.. Carcinoid Heart Disease without Severe Tricuspid Valve Involvement. Cardiology 2016. link 3 Reichman O, Sobel JD. Vulvovaginal pellagra and lichen sclerosus complicating carcinoid syndrome. Obstetrics and gynecology 2009. link 4 Zacà V, Mondillo S, Focardi M, Ballo P, Giacomin E, Favilli R et al.. Right-sided heart failure in carcinoid syndrome. International journal of cardiology 2007. link 5 Klöppel G, Dege K, Remmele W, Kapran Y, Tuzlali S, Modlin IM. Siegfried Oberndorfer: a tribute to his work and life between Munich, Kiel, Geneva, and Istanbul. Virchows Archiv : an international journal of pathology 2007. link 6 Abdullah F, Loon LG. An incidental finding of thymic carcinoid during urgent CABG operation. The heart surgery forum 2002. link 7 Mac Connachie AM. Octreotide (sandostatin, sandoz). Intensive & critical care nursing 1996. link81106-2) 8 Wallace S, Ajani JA, Charnsangavej C, DuBrow R, Yang DJ, Chuang VP et al.. Carcinoid tumors: imaging procedures and interventional radiology. World journal of surgery 1996. link 9 Hyett A. Severe bronchospasm during rectal carcinoid resection. The Australian and New Zealand journal of surgery 1995. link 10 Vandewoude M, Michielsen P, Pelckmans P, Bourgeois N, Van Maercke Y. A case of a rectal carcinoid with multiple liver, lung and bone metastases. Acta clinica Belgica 1991. link 11 Hossain J, al-Mofleh I, Tandon R, Dhahada NM. Carcinoid syndrome without liver metastasis. Postgraduate medical journal 1989. link 12 Scoma JA. Carcinoid tumors of the rectum. American journal of surgery 1978. link90142-3) 13 Chapuis P, Weedon D. Ischaemic ileal necrosis and carcinoid tumour. The Australian and New Zealand journal of surgery 1976. link

    Original source

    1. [1]
      Carcinoid Heart Disease: Pathophysiology, Pathology, Clinical Manifestations, and Management.Jin C, Sharma AN, Thevakumar B, Majid M, Al Chalaby S, Takahashi N et al. Cardiology (2021)
    2. [2]
      Carcinoid Heart Disease without Severe Tricuspid Valve Involvement.Killu AM, Newman DB, Miranda WR, Maleszewski JJ, Pellikka P, Schaff HV et al. Cardiology (2016)
    3. [3]
      Vulvovaginal pellagra and lichen sclerosus complicating carcinoid syndrome.Reichman O, Sobel JD Obstetrics and gynecology (2009)
    4. [4]
      Right-sided heart failure in carcinoid syndrome.Zacà V, Mondillo S, Focardi M, Ballo P, Giacomin E, Favilli R et al. International journal of cardiology (2007)
    5. [5]
      Siegfried Oberndorfer: a tribute to his work and life between Munich, Kiel, Geneva, and Istanbul.Klöppel G, Dege K, Remmele W, Kapran Y, Tuzlali S, Modlin IM Virchows Archiv : an international journal of pathology (2007)
    6. [6]
      An incidental finding of thymic carcinoid during urgent CABG operation.Abdullah F, Loon LG The heart surgery forum (2002)
    7. [7]
      Octreotide (sandostatin, sandoz).Mac Connachie AM Intensive & critical care nursing (1996)
    8. [8]
      Carcinoid tumors: imaging procedures and interventional radiology.Wallace S, Ajani JA, Charnsangavej C, DuBrow R, Yang DJ, Chuang VP et al. World journal of surgery (1996)
    9. [9]
      Severe bronchospasm during rectal carcinoid resection.Hyett A The Australian and New Zealand journal of surgery (1995)
    10. [10]
      A case of a rectal carcinoid with multiple liver, lung and bone metastases.Vandewoude M, Michielsen P, Pelckmans P, Bourgeois N, Van Maercke Y Acta clinica Belgica (1991)
    11. [11]
      Carcinoid syndrome without liver metastasis.Hossain J, al-Mofleh I, Tandon R, Dhahada NM Postgraduate medical journal (1989)
    12. [12]
      Carcinoid tumors of the rectum.Scoma JA American journal of surgery (1978)
    13. [13]
      Ischaemic ileal necrosis and carcinoid tumour.Chapuis P, Weedon D The Australian and New Zealand journal of surgery (1976)

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