The optimal use of cardiac imaging in the quantification of carcinoid heart disease

Abstract

Carcinoid heart disease is a rare cause of right-sided valvular dysfunction, primarily mediated by serotonin. It is an important complication in patients with carcinoid syndrome and occurs in 20-50% of such patients. Echocardiography is the main technique used for the assessment of carcinoid heart disease, but other imaging modalities are also important, particularly in the quantification of the severity of the disease. We sought to review the role of cardiac imaging in the assessment of carcinoid heart disease.

Carcinoid Heart Disease: Review of Current Knowledge

“…Chest radiographs in patients with carcinoid HD usually show normal findings but may also reveal nonspecific right-sided heart enlargement, pulmonary nodules, or pleural effusions. 10 Given the facility of 2-dimensional (2D) transthoracic echocardiography (TTE) to characterize RV, TV, and PV function, 59 it is the chief diagnostic tool for carcinoid HD, and the North American Neuroendocrine Tumor Society recommends its use. 60 The European Neuroendocrine Tumor Society (ENTS) recommends TTE screening for patients diagnosed with carcinoid syndrome, annually for patients with no cardiac involvement and semiannually for those with it.…”

Carcinoid-syndrome: recent advances, current status and controversies

“…Timely surgical treatment of advanced cases of CHD has been shown to markedly improve survival[92] and increased recognition and treatment of CHD has resulted in an improvement in 5-yr survival from <30% in the 1980s to 55% at present[99,133]. Therefore, it is essential to screen patients with carcinoid-syndrome for CHD and it is generally recommended that this be performed with echocardiography[•76,93,177,178]. This is recommended in most recent guidelines and reviews because of its sensitivity and because the clinical correlates to suggest the presence of CHD are generally poor[•76,91,178].…”

Carcinoid heart disease: a guide for screening and timing of surgical intervention

“…CMR imaging may therefore be a helpful tool when transthoracic echocardiography (TTE) is insufficient. Additionally, CMR imaging allows for careful assessment of myocardial tissue and may therefore aid in the detection of fibrous plaques and myocardial metastases [ 22 , 25 ]. The typical features of CaHD are summarised in Table 1 .…”

“…European Neuroendocrine Society (ENETS) guidelines recommend echocardiographic screening only in patients with carcinoid syndrome or if urinary 5‑HIAA and/or chromogranin A are elevated [ 19 ]. An algorithm for the screening for CaHD in patients with metastatic NET with or without carcinoid syndrome has been proposed by others, suggesting annual clinical assessment, TTE and NT-proBNP measurement [ 25 ]. In the case of uncertain RV function and suspicion of extracardiac involvement, CMR imaging is recommended.…”