Introduction
Carcinoid heart disease (CaHD) is a complication of neuroendocrine tumors (NETs), rare slow-growing malignancies which usually originate in the small intestine and metastasize to the liver. High levels of circulating serotonin and other vasoactive substances released by the tumors cause fibrosis and retraction of the tricuspid (TV) and pulmonary valves (PV), resulting in severe regurgitation and/or stenosis. This eventually leads to progressive right heart failure, often worsening the forecast of the patient further (11Hayes AR, Davar J, Caplin ME. Carcinoid Heart Disease: A Review. Endocrinol Metab Clin North Am. 2018;47(3):671-682.). Valve surgery in CaHD patients has been shown to reduce right heart failure, increase functional capacity, allow for more aggressive oncological treatment and improve long-term prognosis. Several smaller case series of 19-32 patients have been reported (22Bhattacharyya S, Raja SG, Toumpanakis C et al. Outcomes, risks and complications of cardiac surgery for carcinoid heart disease. Eur J Cardiothorac Surg. 2011;40:168-172.,33Mokhles P, van Herwerden LA, de Jong PL et al. Carcinoid heart disease: outcomes after surgical valve replacement. Eur J Cardiothorac Surg. 2012;41(6):1278-83.,44Edwards NC, Yuan M, Nolan O et al. Effect of Valvular Surgery in Carcinoid Heart Disease: An Observational Cohort Study. J Clin Endocrinol Metab. 2016;101(1):183-90.), with acceptable results given the oncological prognosis involved. Nguyen et al reported on 240 CaHD patients, who had valve surgery over the course of 30 years (55Nguyen A, Schaff HV, Abel MD et al. Improving outcome of valve replacement for carcinoid heart disease. J Thorac Cardiovasc Surg. 2019;158(1):99-107.,66Ayala AR, Salerno TA. Commentary: Dance with me to the end of love: Serotonin and the carcinoid heart. J Thorac Cardiovasc Surg. 2019;158(1):108-109.). Thirty-day mortality was 5%, while 2-year survival was 60%. Functional results were greatly improved postoperatively and most patients died from progressive tumor burden. Despite prevalent outcome reporting, there is a shortage in the surgical literature concerning the technical aspects and optimization of surgical details in these rare patients. Furthermore, replacement of the TV or PV is uncommon in routine adult cardiac surgery practice. The focus of the current report is not to report patient outcomes, but to describe some of the surgical technical issues concerning valve replacement in CaHD, based on our recent experience of 17 cases in the past 4 years (Table 1).