Carcinoid heart disease involving the left heart: a case report and biomarker analysis

Abstract

Herein, we report the case of a 67‐year‐old woman who was admitted to our hospital because of dyspnoea and oedema of the lower extremities. Transthoracic echocardiography revealed severe tricuspid and mitral regurgitation, and the leaflets of the tricuspid valve were found to be rigid and almost immobile. The plasma concentrations of serotonin and chromogranin A were elevated, and hence, suspicion for carcinoid heart disease was raised. In addition to the diagnostic workup and medical and surgical treatment, we analysed levels of novel cardiovascular biomarkers throughout the entire follow‐up by means of enzyme‐linked immunosorbent assay. A dopa positron emission tomography (DOPA‐PET) was conducted and showed a neoplasm in the terminal ileum. Tricuspid valve replacement, mitral valve repair, and a closure of the patent foramen ovale (PFO) were conducted. Two months later, hemicolectomy and liver segment resection were performed. The tumour was resected, and the diagnosis of a neuroendocrine tumour (NET) was confirmed. Throughout the follow‐up, we observed a decrease in the plasma levels of novel biomarkers [e.g. interleukin‐8 (IL‐8), soluble suppression of tumorigenicity‐2 (sST2), and heart‐type fatty acid‐binding protein (H‐FABP)] over the follow‐up period. In our case, carcinoid heart disease resulted in a severe tricuspid regurgitation as commonly seen in these patients. Moreover, a pre‐existent mitral regurgitation was likely aggravated by fibrotic remodelling, because a PFO has led to a right‐to‐left shunt and might have caused left heart involvement. As IL‐8 was associated with adverse outcomes in patients with NETs, and sST2 and H‐FABP were associated with adverse outcomes in patients with heart failure previously, these biomarkers could aid in the risk stratification of patients with NET.

2022 ESC Guidelines on cardio-oncology developed in collaboration with the European Hematology Association (EHA), the European Society for Therapeutic Radiology and Oncology (ESTRO) and the International Cardio-Oncology Society (IC-OS)

“…Cardiac metastases are reported with an incidence of 3.8% on the ventricles, confirmed by PET-CT scans. 773,774 Data from the SEER (Surveillance, Epidemiology, and End Results) registry identified that approximately 20% of patients with neuroendocrine malignancies develop carcinoid syndrome (7.6-32.4%), which is associated with shorter survival (4.7 years compared with 7.1 years in patients without carcinoid syndrome) and poor quality of life. 775 It is estimated that 20-50% of these patients present cardiac involvement, especially of the right-sided cardiac valves.…”

Four decades of experience with carcinoid heart disease: An analysis of 84 patients

“…Currently, no other screening options besides NT-proBNP and echocardiography exist. Other biomarkers (activin A, connective tissue growth factor and soluble suppression of tumorigenicity 2) have been investigated for CHD, [40][41][42] but we have recently found that these markers did not outperform NT-proBNP in the prediction or detection of CHD. 35 Yet, as mentioned previously, NT-proBNP is a marker of cardiac stress and shows the consequences of CHD, rather than the causes thereof.…”

Multimodality imaging in the diagnostic approach to a patient with carcinoid heart disease involving four heart valves

“…8 In literature, there are reports of carcinoid heart disease involving the left and right heart, which were managed by tumor debulking and repair/replacement of the affected valves in certain cases. 9 We followed the same approach; however, regarding the oncologist consult, tumor debulking was not recommended for our patient.…”