Carcinoid heart disease: correlation of echocardiographic and histopathological findings.

To cite: Miles LF, Leong T, McCall P, et al. BMJ Case Rep Published online: [please include Day Month Year] doi:10.1136/bcr-2014207732 DESCRIPTION A 41-year-old man presented to an anaesthetic preassessment clinic with a metastatic neuroendocrine tumour and clinical features of carcinoid syndrome for consideration of hepatic resection. He gave a history of worsening exertional dyspnoea and peripheral oedema. A loud systolic murmur was audible over the right sternal edge. Transthoracic echocardiography revealed severe tricuspid and pulmonic regurgitation and right ventricular volume overload consistent with carcinoid heart disease, necessitating double valve replacement. Intraoperative transoesophageal echocardiography (figure 1A) demonstrated thickening and retraction of the tricuspid valve leaflets. Colour flow Doppler (figure 1B) revealed torrential tricuspid regurgitation. Three-dimensional imaging in systole (figure 1C) and diastole (figure 1D) shows minimal motion of the tricuspid valve over the cardiac cycle, and a large regurgitant orifice area. Histopathological analysis of the excised cardiac valves demonstrated thickening of valve leaflets due to carcinoid plaques, composed of a proliferation of myofibroblasts and the deposition of a myxoid extracellular matrix (figure 2A, B). Valvular dysfunction in carcinoid heart disease is caused by proliferation of endocardial fibroblasts in response to chronic inflammation, or induced by circulating vasoactive mediators. Deposition of plaques on the valve leaflets and subvalvular apparatus results in thickening, retraction, impaired leaflet motion and greatly enhanced echogenicity. Carcinoid heart disease is a challenge for anaesthetists, because of right ventricular failure and the risk of intraoperative carcinoid crisis. This manifests as severe bronchospasm, hypertension or hypotension, and is associated with mortality of up to 18%. With appropriate preventative measures, our patient encountered no serious intraoperative issues, was discharged from intensive care unit 24 h