Multimodality imaging in carcinoid heart disease

Abstract

Neuroendocrine neoplasms arise from the gastrointestinal tract and can lead to carcinoid syndrome. Carcinoid heart disease affects more than half of these patients and is the initial presentation of carcinoid syndrome in up to 20 % of patients. Carcinoid heart disease typically leads to valve dysfunction, but in rare instances, carcinoid tumours can also metastasise to the endocardium and myocardium. Cardiovascular imaging plays an integral role in the diagnosis and prognosis of carcinoid heart disease. The use of multimodality imaging techniques including echocardiography, cardiac MRI, cardiovascular CT and positron emission tomography have allowed for a more comprehensive assessment of carcinoid heart disease. In this review, we discuss the features of carcinoid heart disease observed on multimodality imaging, indications for obtaining imaging studies and their role in carcinoid heart disease management.

Keywords: cardiac remodelling; echocardiography; tricuspid valve disease.

Figure 1

(A) Transthoracic echocardiogram of the right heart showing fixed, immobile TV (arrows) and resultant (B) severe tricuspid regurgitation. Doppler interrogation (C) reveals a characteristic ‘dagger-shaped’ (arrows) spectrum on Doppler profile (early peak pressure with a subsequent rapid decline). TV, tricuspid valve.

Figure 2

Transthoracic echocardiogram showing thickening of the mitral valve (arrowheads) in a patient with left-sided carcinoid heart disease. LA, left atrium; LV, left ventricle.

Figure 3

Transoesophageal echocardiogram performed with ‘microbubble’ contrast in order to assess for the presence of a right to left interatrial shunt (arrow showing bubbles on the left side of the heart). LA, left atrium; RA, right atrium.

Figure 4

Myocardial strain with region of interest over entire RV in the RV focused view in a patient with severe RV enlargement and carcinoid heart disease. Note average RV lateral wall strain was −10.7 (normal −26.9%±4.4%). Two-dimensional speckle tracking analysis was performed using the vendor apical two-chamber view region of interest. RV, right ventricle.

Figure 5

Three-dmensional image from the ventricular projection during systole showing closure of neighbouring mV (arrow), while TV remains fully open (arrowhead) due to severe TV leaflet retraction. MV, mitral valve; TV, tricuspid valve.

Figure 6

Pictorial representation of quantification of valvular regurgitation done by CMR, along with example calculation of volumetric quantification of regurgitation across the pulmonic and tricuspid valves. CMR, cardiac magnetic resonance.

Figure 7

CCT of pulmonic prosthesis showing crescent shaped thrombus (red arrows) that obstructs the outflow (A) along with well seated normal functioning tricuspid valve prosthesis (red arrow) in the same patient (B). CCT, cardiovascular CT.

Figure 8

Gallium 68-DOTATATE PET-CT showing cardiac metastasis of a neuroendocrine tumour by tracer avidity (red arrow). PET, positron emission tomography.

Figure 9

Multimodality cardiovascular imaging flow diagram in the diagnosis and management of carcinoid heart disease (CHD). 2D TTE, two-dimensional transthoracic echocardiography; 3D TTE, three-dimensional transthoracic echocardiography; PET, positron emission tomography; TOE, transoesophageal echocardiography; TTE, transthoracic echocardiogram.

Figure 10

(A) Postoperative transthoracic echocardiogram done in a patient with carcinoid heart disease with three bioprosthetic valves replacements (arrows). (B) 3D TOE in the same patient shows a well-seated tricuspid bioprosthetic valve. 3D TOE, three‐dimensional transoesophageal echocardiography; AV, aortic valve; PV, pulmonic valve; TV, tricuspid valve.