Development and Progression of Carcinoid Heart Disease in a Cohort of Adult Patients With Neuroendocrine Tumors

Study Overview
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Brief Summary
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Carcinoid Heart Disease (CHD) is a rare form of heart disease, occurring in over 50% of the patients with carcinoid syndrome. Pathophysiology, prognostic factors of development of Carcinoid Heart Disease and progression of disease remain unclear. This observational multicenter cohort study is designed to study the occurrence of Carcinoid Heart Disease in patients with differentiated carcinoid tumors, to describe numerous factors influencing the occurrence, severity, progression and long-term survival of patients with Carcinoid Heart Disease. Basic informations and detailed diagnosis informations (oncological and cardiac parameters), are collected by professional doctors. Clinical outcomes (onset of Carcinoid Heart Disease, cardiac surgery, related death) will be followed up every year or every six/three months if clinically indicated.

Official Title
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Development and Progression of Carcinoid Heart Disease in a Cohort of Adult Patients With Neuroendocrine Tumors Carcinoid Heart Disease (CHD): An Observational French Multi-Centre Cohort Study CRUSOE - NETs (CaRdiac UltraSonic OutcomEs - NETs)

Conditions
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Carcinoid Heart Disease

Intervention / Treatment
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* Other: Study of the occurrence of Carcinoid Heart Disease


Participation Criteria
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Eligibility Criteria
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Inclusion Criteria: Patients over 18 years Patients followed in a NeuroEndocrine Tumor center with a reference cardiologist Patient with: Histologically documented metastatic well-differentiated ileum neuroendocrine tumor or Histologically documented metastatic well-differentiated bronchial neuroendocrine tumor, or Histologically documented well differentiated neuroendocrine tumor, regardless of the primitive site or unknown primitive site and presenting a carcinoid syndrome and / or an elevation of urinary 5HIAA > 2 fold the upper limit of normal range Information given to the patient and his documented non-opposition Exclusion Criteria: Poorly differentiated neuroendocrine carcinoma Patient unable / unwilling to follow the cardiac monitoring recommended by good practice data Any medical, geographical, sociological, psychological or legal situation that does not allow the patient to complete the study

Ages Eligible for Study
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Minimum Age: 18 Years

Sexes Eligible for Study
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All

Accepts Healthy Volunteers
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No

Study Plan
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How is the study designed?
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Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| Patients with or at risk of carcinoid heart disease<br>Adult patients with well-differentiated metastatic ileum or bronchial neuroendocrine tumor Adult patients with carcinoid syndrome or elevated urinary 5HIAA regardless of primary site | Other: Study of the occurrence of Carcinoid Heart Disease<br>* Patient at high risk of Carcinoid Heart Disease (metastatic ileum or bronchial well-differentiated neuroendocrine tumors, patients with high level of urinary 5HIAA or with carcinoid syndrome) are followed with annual echocardiography to detect the occurrence of carcinoid heart disease. In case of documented Carcinoid Heart Disease, a six or three months' cardiac follow-up is necessary to evaluate the progression and the severity of the disease. Investigators collect the data of clinical parameters (flushes, diarrhea, …) biological parameters (urinary 5HIAA, NT-ProBNP …) and cardiac parameters that may influence the occurrence, severity and progression of Carcinoid Heart Disease.<br>|

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Carcinoid Heart Disease | Percentage of patients with carcinoid heart disease at diagnosis and during follow-up (carcinoid heart disease diagnosis will be assessed by an annual echocardiography). | 10 years (at the end of study) | 

Secondary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Cardiac surgery | Percentage of patients requiring cardiac surgery for the cardiac carcinoid heart disease | 10 years (at the end of study) | 
| 5HIAA levels | Correlation between urinary 5HIAA levels at diagnosis and occurrence of carcinoid heart disease | 10 years (at the end of study) | 
| Survival | Overall survival in patents with and without carcinoid heart disease | 10 years (at death or at the end of study) |