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Update in carcinoid heart disease - the heart of the matter

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Abstract

Carcinoid heart disease (CHD) is a paraneoplastic cardiac manifestation occurring in patients with carcinoid syndrome (CS) and advanced neuroendocrine malignancy. In about 20–40% of patients with CS, chronic exposure to tumor-released circulating vasoactive peptides typically results in right-sided valvular fibrosis leading to valve dysfunction and right heart failure. CHD remains a significant cause of morbidity and mortality. The management of patients with CHD is complex, as both the systemic malignant disease and the heart involvement have to be addressed. Early diagnosis and timely surgical intervention in selected patients are of utmost importance and offer a survival benefit. In patients with advanced carcinoid heart disease, valve replacement surgery is the most effective option to alleviate cardiac symptoms and contribute to survival outcomes. A collaboration of a multidisciplinary team in centers with experience is required to provide optimal patient management. Here, we review the current literature regarding CHD presentation, pathophysiology, diagnostic tools, and available treatment strategies.

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Authors and Affiliations

  1. Neuroendocrine Tumor Unit, Endocrinology and Metabolism Department, Division of Medicine, Hadassah-Hebrew University Medical Center, P.O.B. 12000, 91120, Jerusalem, Israel

    Kira Oleinikov & Simona Grozinsky-Glasberg

  2. ENETS Center of Excellence, Hadassah-Hebrew University Medical Center, Jerusalem, Israel

    Kira Oleinikov, Amit Korach, David Planer, Dan Gilon & Simona Grozinsky-Glasberg

  3. Department of Cardiothoracic Surgery, Hadassah-Hebrew University Medical Center, Jerusalem, Israel

    Amit Korach

  4. Department of Cardiology, Hadassah-Hebrew University Medical Center, Jerusalem, Israel

    David Planer & Dan Gilon

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  1. Kira Oleinikov
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Oleinikov, K., Korach, A., Planer, D. et al. Update in carcinoid heart disease - the heart of the matter. Rev Endocr Metab Disord 22, 553–561 (2021). https://doi.org/10.1007/s11154-020-09624-y

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