| Summary: | Introduction: Carcinoid heart disease is a para-neoplastic complication of neuroendocrine tumours, occurring in patients with carcinoid syndrome. Due perhaps to its rarity, there is conflicting evidence in the literature with regard to the optimum method of diagnosis and assessment of the condition. The aim of this thesis is to quantify the variation in clinical practice with regard to carcinoid heart disease and to identify the optimum biochemical and echocardiographic methods for the screening, diagnosis and assessment of progression of the condition. Methods: Patients were prospectively recruited from specialist neuroendocrine clinics in the North of England and underwent evaluation of their symptoms, disease burden, biochemical markers, and transthoracic echocardiography. Results: Wide variation in the screening and clinical management of carcinoid heart disease was identified. A total of 239 patients were recruited to the study and the prevalence of carcinoid heart disease was 21%. From a panel of biomarkers, N-terminal pro brain natriuretic peptide (NTproBNP) and plasma 5-hydroxyindoleacetic acid (5HIAA) were the most sensitive and specific biomarkers for the presence of carcinoid heart disease. All previously described echocardiographic scoring systems discriminated highly between those with/without carcinoid heart disease, with no single score performing significantly better than another. The complexity of the scoring systems varied considerably, with the simplest scoring system better suited for screening and the more complex systems most useful for pre-surgical assessment. A disease progression rate of 9% was demonstrated, with a further 22% of patients dying during the study. Plasma 5HIAA was the greatest predictor of disease progression and death. Conclusion: There is considerable heterogeneity across the UK and Ireland in multiple aspects of screening and management of carcinoid heart disease. NTproBNP and plasma 5HIAA should be used to screen for the disease with transthoracic echocardiography reserved for those with elevated biomarkers. A simple echocardiographic scoring system should be used to screen for the disease, with the more complex scoring systems reserved for those patients with established disease. Biomarkers can also be used to predict risk of disease progression and death.
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