Last update 01 Nov 2024

Carcinoid Heart Disease

Last update 01 Nov 2024

Basic Info

Synonyms

Carcinoid Heart Disease, Carcinoid Heart Diseases, Carcinoid heart disease

+ [10]

Introduction

Cardiac manifestation of gastrointestinal CARCINOID TUMOR that metastasizes to the liver. Substances secreted by the tumor cells, including SEROTONIN, promote fibrous plaque formation in ENDOCARDIUM and its underlying layers. These deposits cause distortion of the TRICUSPID VALVE and the PULMONARY VALVE eventually leading to STENOSIS and valve regurgitation.

Drugs associated with Carcinoid Heart Disease

Lutetium Dotatate LU-177

Target

SSTR2

Mechanism

SSTR2 antagonists [+1]

Active Org.

FUJIFILM Toyama Chemical Co., Ltd. [+9]

Originator Org.

Advanced Accelerator Applications SA

Active Indication

Neuroendocrine Tumors [+15]

Inactive Indication

Carcinoid Tumors, Intestinal [+1]

Drug Highest PhaseApproved

First Approval Ctry. / Loc.

EU [+3]

First Approval Date26 Sep 2017

Telotristat Etiprate

Target

TPH1 x TPH2

Mechanism

TPH1 inhibitors [+1]

Active Org.

Lexicon Pharmaceuticals, Inc. [+4]

Originator Org.

Lexicon Pharmaceuticals, Inc.

Active Indication

Malignant Carcinoid Syndrome [+3]

Inactive Indication

Advanced Bile Duct Carcinoma [+7]

Drug Highest PhaseApproved

First Approval Ctry. / Loc.

First Approval Date28 Feb 2017

Clinical Trials associated with Carcinoid Heart Disease

NCT05064514 / Active, not recruitingNot Applicable

Single Centre, Prospective, Single Arm Open Label Series of a Transcatheter Tricuspid Valved Stent Graft System for the Reduction of Tricuspid Regurgitation and Improved Patient Reported Outcomes in Patients With Carcinoid Heart Disease

The purpose of this investigation is to see if the TRICENTO Valved Stent Graft implant reduces tricuspid regurgitation (TR) and improves the symptoms and quality of life in 15 participants with carcinoid heart disease, and who are not able to have a new valve via a surgical procedure.

Start Date06 Apr 2022

Sponsor / Collaborator

Queen Mary University of London

[+1]

NCT04810091 / Active, not recruitingPhase 3IIT

TELEHEART: Telotristat Ethyl in a Heart Biomarker Study

This phase III trial compares the effect of telotristat ethyl and the current standard of care somatostatin analog therapy or somatostatin analog therapy alone in treating patients with neuroendocrine tumor that has spread to other places in the body (metastatic). Telotristat ethyl and somatostatin analog therapy may help to control carcinoid syndrome and carcinoid heart disease.

Start Date18 May 2021

Sponsor / Collaborator

The University of Texas MD Anderson Cancer Center

NCT04039516 / Not yet recruitingPhase 2IIT

Phase II Randomised Trial to Assess Progression of Carcinoid Heart Disease in Patients Treated With Lutathera Therapy Compared to Best Supportive Care.

Randomised trial to assess progression of carcinoid heart disease in patients treated with Lutathera therapy compared to best supportive care.

Start Date01 Oct 2020

Sponsor / Collaborator

King's College Hospital NHS Foundation Trust

[+1]

100 Clinical Results associated with Carcinoid Heart Disease

100 Translational Medicine associated with Carcinoid Heart Disease

0 Patents (Medical) associated with Carcinoid Heart Disease

659

Literatures (Medical) associated with Carcinoid Heart Disease

01 Nov 2024·ESMO Open

Carcinoid heart disease in patients with advanced small-intestinal neuroendocrine tumors and carcinoid syndrome: a retrospective experience from two European referral centers

Article

Author: Falkman, L. ; Welin, S. ; Welin, S ; Ciardiello, D. ; Gervaso, L. ; Cella, C A ; Benini, L. ; Boselli, S ; Cardinale, D ; Algeri, L ; Gervaso, L ; Algeri, L. ; Falkman, L ; Cardinale, D. ; Fazio, N ; Zanobini, M ; Tamayo, D ; Frassoni, S ; Bagnardi, V ; Spada, F ; Tamayo, D. ; Ciardiello, D ; Spada, F. ; Zampino, M.G. ; Cella, C.A. ; Crona, J ; Bagnardi, V. ; Mazzon, C ; Mazzon, C. ; Fazio, N. ; Benini, L ; Russo, A ; Russo, A. ; Boselli, S. ; Frassoni, S. ; Zampino, M G ; Zanobini, M. ; Badalamenti, G ; Crona, J. ; Badalamenti, G.

BACKGROUNDUp to 50% of patients with advanced small-intestinal neuroendocrine tumors (SI-NETs) and carcinoid syndrome (CS) develop carcinoid heart disease (CHD). However, the true frequency and prognostic markers for CHD in CS are lacking. We described the real-world management of patients in two NET referral centers in this clinical context and relationships between clinical features, including CHD and overall survival (OS).PATIENTS AND METHODSThis is a retrospective analysis of patients with stage IV SI-NET and CS, treated at the European Institute of Oncology in Milan and Uppsala University in Sweden between 2015 and 2021. CHD was defined as at least one moderate right-sided heart valve defect. Median OS and cumulative incidence of CHD were estimated from the diagnosis of metastatic disease, and the association between clinical parameters with both OS and occurrence of CHD was evaluated.RESULTSWe included 165 patients, with 97% having low-intermediate-grade SI-NETs and 86% having synchronous liver metastases. Ninety-eight patients (59%) became refractory to full label dose of somatostatin analogues and 25% developed a CHD. At CHD diagnosis, baseline urine 5-hydroxyindoleacetic acid (24-h u5-HIAA) value and plasma N-terminal pro-brain natriuretic peptide (NT-proBNP) value were known in 76% of patients. Moderate-to-severe tricuspid insufficiency was the most common alteration of CHD. Prognosis was significantly impaired by CHD (multivariable hazard ratio for OS = 2.85, P < 0.001). The median OS from the CHD diagnosis was 4.5 years [95% confidence interval (CI) 2.1-7.2 years], and the 5-year survival rate was 34% (95% CI 13% to 57%).CONCLUSIONSIn our study population of SI-NET patients with CS, more than half had a refractory carcinoid syndrome (RCS) and one-quarter developed a CHD, with a negative impact on OS. Therefore, it is recommended to screen and monitor patients with CS for CHD, ideally with a combination of u5-HIAA, NT-proBNP values, and echocardiography at CS baseline, preferably in NET referral centers.

01 Oct 2024·Journal of the American Society of Echocardiography

Right Atrial and Ventricular Strain Analysis in Patients With Carcinoid Heart Disease

Article

Author: Pellikka, Patricia A ; Alabdaljabar, Mohamad S ; Pislaru, Sorin V ; Alexandrino, Francisco ; Connolly, Heidi M ; Kucuk, Hilal Olgun ; Luis, S Allen ; Lloyd, James V ; Al-Shakarchi, Nader

01 Oct 2024·Current Problems in Cancer

Management of carcinoid heart disease

Review

Author: Hack, Madelaine ; Bhamidipati, Castigliano M

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