Determination of the Optimal Echocardiographic Scoring System to Quantify Carcinoid Heart Disease

Abstract

Background: Carcinoid heart disease (CHD) is an important complication of metastatic neuroendocrine disease, requiring regular monitoring to enable intervention prior to right heart failure. We aimed to identify the most appropriate echocardiographic scoring systems for the quantitative assessment of CHD. Methods: In this prospective study conducted between April and October 2012 in two European Neuroendocrine Tumor Society (ENETS) Centres of Excellence, patients with neuroendocrine tumours with liver metastases and/or carcinoid syndrome underwent transthoracic echocardiography and blood sampling for serum N-terminal pro-brain natriuretic peptide (NT-proBNP) and plasma 5-hydroxyindoleacetic acid (5-HIAA). Each patient was assessed according to six echocardiographic scoring systems. The individual scoring systems' feasibility, observer variability, sensitivity, specificity and correlation with the concentration biomarkers were determined. Results: 100 patients were included; 21% had echocardiographic evidence of CHD. All scores discriminated highly between those with/without CHD, with no single score performing significantly better than another. The severity, determined using all of the scoring systems, correlated with the concentration of both biomarkers, but the strongest correlations were seen between the Bhattacharyya score and serum NT-proBNP. Conclusion: All scoring systems are comparable in terms of sensitivity and specificity for the detection of CHD. There is a variation in the feasibility of the scoring systems due to varying complexity of the score components. All scores correlate with NT-proBNP and plasma 5-HIAA. The Westberg score appears to be the most optimal scoring system for use in screening of CHD whereas the more complex scoring systems are more suited to the patient with established disease who may require surgical intervention.

Echocardiography and monitoring patients receiving dopamine agonist therapy for hyperprolactinaemia: A joint position statement of the British Society of Echocardiography, the British Heart Valve Society and the Society for Endocrinology

“…In addition to this, however, careful attention should be taken to perform semi‐quantitative assessment of valve structure and function to detect the changes typical of DA agonist therapy. Although there are no methods validated for assessment in DA agonist therapy per se, the changes to be detected are the same as those in patients with carcinoid heart disease, for which there are validated scoring systems with high feasibility and discriminatory value . Of these, the most sensitive and specific is an echocardiographic scoring system that assesses leaflet thickening, mobility and morphology, severity of valvular regurgitation and stenosis, and the haemodynamic effects on (right) ventricular size and function with good inter‐observer agreement .…”

“…There have been no prospective validation studies of a scoring system and therefore, it is not possible to give a value or ‘score’ above which a patient should be categorized as affected by DA‐valvopathy. The sensitivity of the scoring system for identifying changes in patients with carcinoid increases with increasing score, with a median score in those affected 12 (range 8‐21) and in those not affected 2 (IQR 1‐3) . It could be argued that routinely performing a transthoracic echocardiogram before drug therapy is commenced may be unnecessary and that doing an echocardiogram if the dose is increased above 2 mg/wk may be sufficient.…”

“…Although there are no methods validated for assessment in DA agonist therapy per se, the changes to be detected are the same as those in patients with carcinoid heart disease, for which there are validated scoring systems with high feasibility and discriminatory value. 37 Of these, the most sensitive and specific is an echocardiographic scoring system that assesses leaflet thickening, mobility and morphology, severity of valvular regurgitation and stenosis, and the haemodynamic effects on (right) ventricular size and function with good inter-observer agreement. 38 Moreover, this incorporates assessment of all four cardiac valves, although focusses on haemodynamically-significant rightsided valvular lesions through secondary effects on right ventricular size and function, which have been most frequently identified in the literature in DA agonist therapy (Table 3).…”

“…The sensitivity of the scoring system for identifying changes in patients with carcinoid increases with increasing score, with a median score in those affected 12 (range 8-21) and in those not affected 2 (IQR 1-3). 37 It could be argued that routinely performing a transthoracic echocardiogram before drug therapy is commenced may be unnecessary and that doing an echocardiogram if the dose is increased above 2 mg/wk may be sufficient. The approach in this guideline is conservative, since the existing case-control and longitudinal follow-up studies cannot definitively exclude a small effect in the longer term.…”

Carcinoid Heart Disease: How to Diagnose and Treat in 2020?

“…Comparison of echocardiographic scores in subjects with carcinoid heart disease (modified after20 ).…”