Dealing With the Right Side: Carcinoid Heart Disease

Balancing carcinoid crisis and right ventricular dysfunction during tricuspid and pulmonic valve replacement for carcinoid heart disease: A case report

“…The incidence of carcinoid tumor is approximately 1 in 75,000. Approximately 10 to 50 % patients develop carcinoid syndrome, of which one-half to two-thirds develop valvular involvement because of endocardial deposition of plaque-like fibrous tissue [5] . Carcinoid heart disease manifests preferentially on right-sided valves because downstream metabolism of vasoactive products by the lung occurs [3] , [7] .…”

“…Carcinoid tumors produce and release several distinct bioactive peptides including serotonin, histamine, kinins, insulin, catecholamines, and prostaglandins [4] . Serotonin is the most important pathophysiological mediator of symptoms and disease progression [2] , [5] . The precise mechanism(s) by which serotonin causes valve injury is still unclear, but the relationship between serotonin and cardiac involvement may be mediated by upregulation of transforming growth factor-β and collagen synthesis by myofibroblasts in the extracellular matrix [2] , [4] , [9] , [12] , [13] .…”

“…Both valve lesions contribute to eventual right ventricular dysfunction [1] , [2] . Valve replacement often worsens preexisting right ventricular dysfunction perioperatively by eliminating the low-pressure outlet [2] , [5] . Myocardial stunning resulting from inadequate myocardial protection during cardiopulmonary bypass further exacerbates this phenomenon [1] , [2] , [5] , [16] .…”

“…Valve replacement often worsens preexisting right ventricular dysfunction perioperatively by eliminating the low-pressure outlet [2] , [5] . Myocardial stunning resulting from inadequate myocardial protection during cardiopulmonary bypass further exacerbates this phenomenon [1] , [2] , [5] , [16] . In our patient, inotropic support and inhaled nitric oxide were required to support right ventricular function during and after separation from bypass.…”

“…Anesthetic management of patients with carcinoid syndrome during cardiac surgery involves preventing cardiovascular instability from release of vasoactive substances (carcinoid crisis) and managing hemodynamic derangements resulting from tricuspid or pulmonary valve replacement and cardiopulmonary bypass to mitigate right ventricular dysfunction [4] , [5] . The use of vasoactive drugs is counterintuitive to conventional thinking because these medications may exacerbate release of vasoactive peptides from the tumor.…”