Bhattacharyya, S.; (2011) Carcinoid heart disease: diagnosis, investigation, progression and management. Doctoral thesis , UCL (University College London).

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Abstract

INTRODUCTION: Carcinoid heart disease is acquired form of valvular heart disease occurring in patients with carcinoid syndrome. We sought to identify the prevalence, predictive biomarkers, advanced echocardiographic features, risk factors for development and outcomes of cardiac surgery for carcinoid heart disease. METHODS: A prospective, observational, cohort study of 252 patients with a history of carcinoid syndrome attending a neuroendocrine tumour clinic was undertaken. Patients underwent serial evaluation of symptoms (cardiac and neuroendocrine), functional status, biochemical markers, echocardiography, and tumour staging over a three year period. RESULTS: Carcinoid heart disease was initially identified in 20% of patients with carcinoid syndrome. The sensitivity and specificity of NT-proBNP, at a cut-off level of 260pg/ml, for detection of carcinoid heart disease was 0.92 and 0.91, respectively. Involvement of the tricuspid, pulmonary, mitral and aortic valves were found in 90%, 69%, 29% and 27% of patients respectively. Myocardial metastases were found in 3.8% of patients. 3D echocardiography provided more detailed anatomical assessment, particularly for tricuspid and pulmonary valves, than 2D techniques. Independent predictors of the development or progression of carcinoid heart disease were a 5-HIAA greater than 300 ųmol/24hr and greater than 3 episodes of flushing per day. Overall 30 day mortality of cardiac surgery was 18.2%. 2 year survival was 44.4 %. Long term causes of death were related to advanced metastatic carcinoid tumour. No patient required re-operation for bio-prosthetic degeneration. CONCLUSION: The prevalence of carcinoid heart disease is significantly less than reported in previous decades. The high negative predictive value of NT-proBNP may allow its use as a screening test for carcinoid heart disease. 3D echocardiography allows more detailed assessment of valvulopathy than 2D techniques. A 5-HIAA > 300 ųmol/24hr and >3 episodes of flushing per day are predictors of the development and/or progression of carcinoid heart disease. Cardiac valve surgery is high risk but provides symptomatic relief.

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