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Carcinoid Heart Disease: How to Diagnose and Treat in 2020?

Barbara Bober1, Marek Saracyn1, Maciej Kołodziej1

  • 1Department of Endocrinology and Isotope Therapy, Military Institute of Medicine, Warsaw, Mazowieckie, Poland.

Clinical Medicine Insights. Cardiology
|November 16, 2020
PubMed

Insights

Neuroendocrine tumors can lead to carcinoid syndrome and carcinoid heart disease due to prolonged high serotonin. Treatment involves somatostatin analogs, with cardiac surgery as an option for advanced cases.

Area of Science:

  • Cardiology
  • Endocrinology
  • Oncology

Background:

  • Neuroendocrine tumors (NETs) are rare neoplasms, with carcinoid syndrome (CS) affecting 30-40% and carcinoid heart disease (CaHD) impacting 20-50% of CS patients.
  • Prolonged exposure to elevated serum serotonin is a key factor in CaHD development.
  • CaHD is characterized by specific cardiac structural changes, including endocardial plaque-like deposits and thickening of valve leaflets and subvalvular apparatus.

Purpose of the Study:

  • To review the incidence, pathophysiology, diagnosis, and management of carcinoid heart disease (CaHD) in patients with neuroendocrine tumors (NETs).
  • To highlight the role of serotonin in CaHD development and progression.
  • To discuss current and emerging treatment strategies for CaHD.

Main Methods:

  • Review of existing literature on neuroendocrine tumors, carcinoid syndrome, and carcinoid heart disease.
  • Analysis of diagnostic markers such as NT pro-BNP and 5-hydroxyindoleacetic acid.
  • Evaluation of therapeutic interventions including somatostatin analogs, telotristat ethyl, peptide receptor radionuclide therapy, and cardiac surgery.

Main Results:

  • Long-acting somatostatin analogs are the standard for symptom control and first-line treatment in metastatic NETs.
  • For refractory cases, options include dose escalation of somatostatin analogs, telotristat ethyl, or peptide receptor radionuclide therapy.
  • Cardiac surgery, particularly valve replacement, is the most effective strategy for advanced CaHD, improving symptoms and prognosis.

Conclusions:

  • Carcinoid heart disease is a significant complication of neuroendocrine tumors, driven by serotonin.
  • Effective management requires a multidisciplinary approach, combining medical therapies for tumor and symptom control with surgical intervention for advanced cardiac disease.
  • Early recognition and intervention are crucial for improving outcomes in patients with CaHD.
Keywords:
Hedinger’s syndromecarcinoid crisissomatostatin analogstricuspid valve replacementurinary 5-hydroxyindoleacetic acid

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