4-Valve Heart Disease and Right Heart Failure

Carcinoid heart disease is a rare form of heart disease due to secretion of vasoactive compounds, including serotonin, from gastrointestinal tumors. This E-challenge examines the case of a patient with advanced carcinoid heart disease who presented to the operating room (OR) for a tricuspid valve replacement. Once the patient was in the OR, intraoperative transesophageal echocardiography was used to discover a patent foramen ovale and involvement of all 4 valves with regurgitant lesions. The patient underwent tricuspid valve replacement, pulmonic valve replacement, right ventricular outflow tract reconstruction, and patent foramen closure in the OR and experienced subsequent fulminant right heart failure. Mechanical circulatory support was required to separate the patient from cardiopulmonary bypass, which was first attempted with an intra-aortic balloon pump and subsequently achieved with implantation of a right ventricular assist device. Multiple reports of acute right heart failure are available in the literature; however, this case helps illustrate several important considerations for the anesthesiologist. The effects of chronic circulating vasoactive compounds on the heart valves are well documented; however, it is likely that advanced carcinoid heart disease also will trigger pre-existing myocardial dysfunction, which may be underappreciated. Identifying patients who are at high risk for intraoperative right heart failure and considering what constitutes an adequate preoperative assessment of right heart function aid in preparing for OR management. In addition, reviewing the potential options for managing these patients when the traditional therapies are inadequate, including mechanical support and extracorporeal circulation, is a useful exercise in preparation. This case also highlights the contributions of intraoperative transesophageal echocardiography in the diagnosis and management of carcinoid heart disease, the need for additional preoperative optimization of these patients, and the management and potential complications of mechanical support.