Carcinoid Heart Disease – A 2022 Retrospective

" We aim to introduce a 2022 update on CHD (carcinoid heart disease) considering a multidisciplinary perspective. This is a narrative mini-review. We searched English, full-length papers (PubMed). Inclusion criteria: original articles regardless of the level of statistical significance. We identified 44 papers and manually selected those with CHD, as follows: 8 original studies, 1 case series (N=9 patients), 16 case reports (N=1 patient/paper), and a total of 1,030 patients on published studies. The most remarkable results are the longest period of enrolment of 3-4 decades; CHD ratio among carcinoid syndrome of 37%; 30-day mortality post-cardiac surgery of 12%; median survival in CHD from 1.3 to 3.9 years (more than 2 years if valve intervention is provided); most useful prognostic markers: 5HIIA, NT-pro-BNP, but, potentially, chromogranin A. The specific protective role of somatostatin analogs against developing CHD is yet to be determined. CHD in the absence of carcinoid syndrome/liver metastasis may be related to ovarian NEN (neuroendocrine neoplasia) with a better outcome if prompt intervention. Remarkably, 3 guidelines are released regarding CHD on behalf of ENETS (European Neuroendocrine Tumor Society), and ESC (European Society of Cardiology). CHD still represents a most challenging entity situated at the crossroads of surgery, cardiology, oncology, endocrinology, and gastroenterology. "