(CSEMP035) USE OF PERIOPERATIVE TELOTRISTAT IN A PATIENT WITH CARCINOID HEART DISEASE

Abstract: Carcinoid heart disease is a rare complication of carcinoid syndrome, resulting in right-sided valvular heart disease and subsequent heart failure due to long-term exposure to vasoactive substances. The management of this condition is complex, often requiring surgical intervention. Intraoperatively, patients are at risk of carcinoid crisis: a life-threatening complication of carcinoid syndrome related to the sudden release of vasoactive substances, resulting in hemodynamic instability, arrhythmias, and subsequent intra- and post-operative complications. Current perioperative regimens entail the use of prophylactic somatostatin analogues to prevent carcinoid crisis, however, regimens vary widely among practitioners and evidence supporting their efficacy in this clinical setting is mixed. This prompts the need for evaluation of novel adjuvant therapies to reduce the risk of perioperative carcinoid crisis.

This clinical case describes the perioperative management of a 65-year-old man with carcinoid heart disease requiring tricuspid and pulmonary valve replacement surgery. As an adjunct to somatostatin analogue therapy, the novel tyrosine hydroxylase inhibitor, telotristat, was initiated preoperatively. This combination resulted in normalization of preoperative urinary 5-HIAA levels, and the patient successfully underwent tricuspid and pulmonic valve replacement without evidence of carcinoid crisis. This clinical case is the first available documenting the use of telotristat in the perioperative period in a patient with carcinoid syndrome and carcinoid heart disease and was associated with a good long-term outcome despite the high-risk nature of the case.