The development of carcinoid cardiac valve disease is well recognized in association with patients with neuroendocrine tumours and its associated endocrinopathy. Although its exact aetiology is unknown, it is postulated that the release of serotonin causes endomyocardial fibrosis leading to valvular dysfunction.

Patients typically, although not exclusively develop tricuspid and pulmonary valve disease. The valvular pathology is unique in its appearance, causing retraction of the leaflet tissue leading to severe valvular regurgitation, right heart dilatation and dysfunction, and presents a highly complex pathophysiology, which has been demonstrated to adversely affect prognosis. Additionally, the presence of active cardiac disease presents challenges to the oncological management of the neuroendocrine tumour patient.

Cardiac valvular surgery is indicated in patients with severe tricuspid and or pulmonary regurgitation who have exertional symptoms, episodes of heart failure or echocardiographic findings of right ventricular dilatation or dysfunction. Tissue valves are the valve substitutes of choice.

Surgery should be performed by an experienced team involving endocrinology and cardiology specialists, in addition to cardiac anaesthesia and surgeons with experience in complex right-sided heart valve surgery. The perioperative management of theses patients is highly complex, and routinely involves the use of intravenous octreotide infusions and inotropes to ensure cardiovascular stability.

Over the last 3 years, 15 patients with carcinoid heart disease have been operated upon at The Royal Melbourne and Epworth Hospitals in Melbourne. There was a 1 early (6.7%) and one late mortality. No prosthetic valve dysfunction has been seen during the follow up period, and all patients were symptomatically improved.

Patients with right sided Carcinoid valve disease can be offered surgery at relatively low perioperative risk by experienced centres with specialized teams, with preexisting experience in adult congenital heart disease and with appropriate multidisciplinary support.