Management of carcinoid heart disease

Carcinoid Heart Disease (CaHD) is defined as the constellation of all cardiac manifestations that occur in patients with carcinoid tumors. Cardiac manifestations are generally due to the paraneoplastic effects of vasoactive substances secreted by carcinoid tumors. These primarily cause cardiac valve dysfunction and resultant heart failure. Successful management of patients with CaHD requires a multidisciplinary team to address both the classical manifestations of carcinoid syndrome, as well as the additional manifestations of cardiac dysfunction. While the cornerstone of medical management for carcinoid syndrome are somatostatin analogs (SSAs), there is no evidence to suggest that the usage of SSAs influences the development or progression of CaHD. Additionally, while liver-directed therapies provide a survival benefit to symptomatic carcinoid syndrome patients with resectable disease, there are conflicting data on the survival benefit of hepatic resection among patients with CaHD. Cardiac surgery in patients with CaHD is a complex undertaking, and is the only definitive treatment for symptom management in CaHD with significant survival benefit for patients in advanced disease states. Two crucial surgical decisions to be made are determining which valve(s) should be replaced, and what prosthetic should be utilized. While challenging in this often medically frail population, cardiac surgery confers a survival benefit and should be pursued in cases of symptomatic CaHD or progressive right ventricular dysfunction.