Data regarding the diagnosis, management, and follow-up of carcinoid syndrome (CS) and carcinoid heart disease (CHD) were identified by searches of the MEDLINE database using specific terms in human studies: CS; CHD; screening; epidemiology; diagnosis; treatment; prognosis. The search results were supplemented by manual searching of relevant journals, reference lists in key articles and other appropriate documents, and expert opinion. All recommendations are offered on the basis of the best available evidence, supplemented by the authors' experiences in managing CS and CHD. Each recommendation for treatment will have a level of evidence and grade of recommendation as per the GRADE system (adapted in Infectious Disease society This first ENETS guidance paper on CS aims to provide practical guidance for NEN specialists in the diagnosis and management of CS, with special emphasis on CHD.

In this retrospective study the maze procedure did not seem to increase operative mortality of MV surgery, was effective in eliminating atrial fibrillation, and reduced the risk of thromboembolic complications and the need for long-term anticoagulation after mitral valve repair or replacement with a bioprosthesis.

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