Diagnosing and Managing Carcinoid Heart Disease in Patients With Neuroendocrine Tumors: An Expert Statement

Joseph Davar; Heidi M. Connolly; Martyn E. Caplin; Marianne Pavel; Jerome Zacks; Sanjeev Bhattacharyya; Daniel J. Cuthbertson; Rebecca Dobson; Simona Grozinsky-Glasberg; Richard P. Steeds; Giles Dreyfus; Patricia A. Pellikka; Christos Toumpanakis

doi:10.1016/j.jacc.2016.12.030

Journal of the American College of Cardiology

Volume 69, Issue 10, March 2017 DOI: 10.1016/j.jacc.2016.12.030

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Diagnosing and Managing Carcinoid Heart Disease in Patients With Neuroendocrine Tumors
An Expert Statement

Joseph Davar, Heidi M. Connolly, Martyn E. Caplin, Marianne Pavel, Jerome Zacks, Sanjeev Bhattacharyya, Daniel J. Cuthbertson, Rebecca Dobson, Simona Grozinsky-Glasberg, Richard P. Steeds, Giles Dreyfus, Patricia A. Pellikka and Christos Toumpanakis

Author + information

vol. 69 no. 10 1288-1304

DOI:

https://doi.org/10.1016/j.jacc.2016.12.030

Published By:

Journal of the American College of Cardiology

Print ISSN:

0735-1097

Online ISSN:

1558-3597

History:

Received July 5, 2016
Revision received November 8, 2016
Accepted December 10, 2016
Published online March 6, 2017.

American College of Cardiology Foundation

Author Information

Joseph Davar, MD, PhD^a,
Heidi M. Connolly, MD^b,
Martyn E. Caplin, BSc Hons, DM^c,
Marianne Pavel, MD^d,
Jerome Zacks, MD^e,
Sanjeev Bhattacharyya, MBChB, MD^f,
Daniel J. Cuthbertson, BSc Hons, PhD^g,
Rebecca Dobson, MBChB Hons^h,
Simona Grozinsky-Glasberg, MDⁱ,
Richard P. Steeds, MA, MD^j,
Giles Dreyfus, MD^k,
Patricia A. Pellikka, MD^b and
Christos Toumpanakis, MD, PhD^c,^∗ (c.toumpanakis{at}ucl.ac.uk)

^aCarcinoid Heart Disease Clinic, Department of Cardiology, Royal Free Hospital, London, United Kingdom
^bDepartment of Cardiovascular Diseases, Mayo Clinic, Rochester, Minnesota
^cNeuroendocrine Tumor Unit, ENETS Centre of Excellence, Royal Free Hospital, London, United Kingdom
^dInternal Medicine with Gastroenterology and Nephrology, Charité–Universitätsmedizin Berlin, Berlin, Germany
^eCenter for Carcinoid and Neuroendocrine Tumors of the Icahn School of Medicine at Mount Sinai, New York, New York
^fBarts Heart Centre, St. Bartholomew's Hospital, London, and UCL Institute of Cardiovascular Science, University College London, London, United Kingdom
^gObesity and Endocrinology Research Group, Institute of Ageing and Chronic Diseases, University of Liverpool, Liverpool, United Kingdom
^hDepartment of Cardiology, Liverpool Heart and Chest Hospital, Liverpool, United Kingdom
ⁱNeuroendocrine Tumor Unit, Endocrinology and Metabolism Service, Department of Medicine, Hadassah-Hebrew University Medical Center, Jerusalem, Israel
^jUniversity Hospital Birmingham, NHS Foundation Trust and Institute of Cardiovascular Sciences, University of Birmingham, Birmingham, United Kingdom
^kCardiothoracic Center of Monaco, Monaco, France

↵∗Address for correspondence:
Dr. Christos Toumpanakis, Neuroendocrine Tumour Unit, ENETS Centre of Excellence, Royal Free Hospital, London, United Kingdom.

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Abstract

Carcinoid heart disease is a frequent occurrence in patients with carcinoid syndrome and is responsible for substantial morbidity and mortality. The pathophysiology of carcinoid heart disease is poorly understood; however, chronic exposure to excessive circulating serotonin is considered one of the most important contributing factors. Despite recognition, international consensus guidelines specifically addressing the diagnosis and management of carcinoid heart disease are lacking. Furthermore, there is considerable variation in multiple aspects of screening and management of the disease. The aim of these guidelines was to provide succinct, practical advice on the diagnosis and management of carcinoid heart disease as well as its surveillance. Recommendations and proposed algorithms for the investigation, screening, and management have been developed based on an evidence-based review of the published data and on the expert opinion of a multidisciplinary consensus panel consisting of neuroendocrine tumor experts, including oncologists, gastroenterologists, and endocrinologists, in conjunction with cardiologists and cardiothoracic surgeons.

Key Words

Footnotes

Dr. Caplin has received honoraria for presentations and consultancy from Ipsen, Novartis, and Lexicon; and his institution has received support for scientific activities (research projects) from Novartis and Ipsen. Dr. Pavel has reported honoraria for presentations and consultancy from Pfizer, Ipsen, Novartis, and Lexicon; and her institution has received support for scientific activities (research projects) from Novartis and Ipsen. Drs. Zacks, Bhattacharyya, Cuthbertson, and Grozinsky-Glasberg are on the advisory board of Lexicon. Dr. Dreyfus is a course director with Edwards Lifesciences and Medtronic. Dr. Toumpanakis has received honoraria for presentations and consultancy from Ipsen, Novartis, and Lexicon; and his institution has received support for scientific activities (research projects) from Novartis and Ipsen. All other authors have reported that they have no relationships relevant to the contents of this paper to disclose. The other participants at the First International Symposium for Carcinoid Heart Disease (London, September 2014, under the auspices of the European Neuroendocrine Tumor Society and British Heart Valve Society) are listed in the Online Appendix.
Listen to this manuscript's audio summary by JACC Editor-in-Chief Dr. Valentin Fuster.