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

Abstract

The cardiac manifestations of a neuroendocrine tumour are referred to as carcinoid heart disease (CaHD) and are associated with a poor prognosis. Surgical intervention is the only proven therapeutic option and may prolong survival and quality of life. No consensus has been reached internationally with regard to screening for CaHD and the optimal timing for surgery. Although limited evidence is available on this matter, a trend towards early surgery and subsequent reduced mortality has been observed. In this review we provide an overview of the current understanding and propose a protocol to guide cardiologists in the screening for CaHD and the timing of referral to a specialised surgical centre.

Keywords: Carcinoid heart disease; Hedinger syndrome; Screening; Surgery; Valvular disease.

Fig. 1

a Characteristic ‘dagger’ shaped jet on continuous wave Doppler b Parasternal view of RV inflow tract showing thickening and retraction of tricuspid leaflets (arrow) during systole. RV right ventricle, RA right atrium

Fig. 2

CMR image of one of the two apical intramyocardial lesions (arrow). LV left ventricle

Fig. 3

Proposed protocol for screening and referral in CaHD patients. Level of Evidence V. NET neuroendocrine tumour, NT-proBNP N-terminal pro b‑type natriuretic peptide, 5-HT 5-hydroxytryptamine, 5‑HIAA 5-hydroxyindoleacetic acid, CaHD carcinoid heart disease

Fig. 4

Postoperative 30-day mortality of CaHD patients according to surgical era, adapted from Connolly et al. [39, 40]