Introduction: Neuroendocrine tumours (NETs) can release harmful vasoactive substances into the systemic circulation, causing the characteristic features of carcinoid syndrome (CS) such as flushing, diarrhoea and bronchospasm. Approximately 20% of patients with CS develop carcinoid heart disease (CHD) due to progressive valve thickening, retraction and reduced mobility. CHD primarily affects the right-sided valves resulting in tricuspid and/or pulmonary regurgitation, volume and pressure overload, right ventricular dilatation and subsequent failure. The left-sided valves can be involved if a patent foramen ovale (PFO) allows the substances to bypass pulmonary degradation. Patients with CHD have a worse prognosis, with average survival half that of NET patients without CHD.
Aim: We aim to identify the frequency of CHD in a subsection of our NET population.
Methods: We reviewed the electronic records of patients at our centre between January 2018 and December 2020 with a NET diagnosis. Data were collected on demographics, primary NET site, tumour grade, liver metastases, CS, CHD and echocardiography findings in patients with CHD.
Results: We identified 573 patients (mean 64 years, 58% male). The small bowel was the most common primary tumour site (46%), grade I tumours were the most common (63%) and 38% had liver metastases. Twenty-two percent had CS and of these, 24% had CHD. Severe tricuspid regurgitation was the most frequent finding on echocardiogram (93%), and pulmonary regurgitation of moderate or severe classification was seen in 43%. A PFO was documented in 47% with CHD.
Conclusion: The frequency of CHD in a snapshot of our NET population reflects previous data. As CHD confers a poor prognosis, research is needed to enhance our understanding of the pathophysiology behind valve destruction to develop effective therapies.