Improving Outcome of Valve Replacement for Carcinoid Heart Disease
Anita Nguyen1, Hartzell V Schaff1, Martin D Abel2, S. Allen Luis1, Brian D Lahr1, Thorvardur R Halfdanarson1, Heidi M Connolly1
1Mayo Clinic, Rochester, MN;2Mayo Clinic, Jacksonville, FL
Objective - Carcinoid heart disease is characterized by tricuspid valve regurgitation and varying degrees of pulmonary valve regurgitation and/or stenosis. Valve replacement techniques are standardized, but procedures may be complicated by systemic effects of carcinoid syndrome, hepatic dysfunction due to metastatic tumors, and right heart failure. During the last 2 decades, we have noted improved early outcome of valve replacement in carcinoid heart disease. This study was performed to identify factors that might be associated with reduced early mortality. Methods - Between November 1985 and January 2017, 232 adult patients underwent surgery for carcinoid heart disease at our Clinic. We analyzed association of multiple clinical and echocardiographic variables on early mortality, including perioperative treatment of carcinoid syndrome with short- and long-acting somatostatins. Results - The mean age of patients was 62±11 years, and 115 (50%) were male. Prior to operation, 153 (66%) had New York Heart Association class III/IV limitation. Common cardiac symptoms were dyspnea, edema, ascites, and profound exercise intolerance. Somatostatins were used in 217 (94%), and long-acting somatostatins in 126 (54%). Diuretic therapy was used preoperatively in 147 (63%). On preoperative echocardiography, 226 (97%) had moderate/severe tricuspid regurgitation, and 176 (76%) had moderate/severe pulmonary regurgitation. At operation, the tricuspid valve was replaced in 229 (99%) patients, and the pulmonic valve was excised or replaced in 196 (84%). Surgical intervention on left-sided valves was necessary in 36 (16%). Thirty-one (13%) patients underwent single valve replacement, 170 (73%) had double valve replacement, 23 (10%) triple valve replacement, and 8 (3%) patients had replacement of all 4 valves. In 194 (84%), biologic tissue valves were implanted, and the remaining patients had mechanical prostheses. Additionally, metastatic tumor was removed in 6 (3%) patients. Reoperation due to bleeding was necessary in 18 (8%). There were 22 (9%) deaths within 30 days of operation, and survival estimates at 1, 3, and 5 years were 67%, 45%, and 31%. Between 1985 and 1999, short-acting somatostatins were used to control systemic carcinoid symptoms and prevent carcinoid crisis, and in this era, early mortality occurred in 12/71 (17%). Since 2000, long-acting somatostatins, which avoid rebound hypersecretion, have been preferred for most patients, and early mortality was 10/161 (6%). Conclusions - Survival in patients with carcinoid heart disease is improving, and operation on patients with valvular disease has acceptable short-term mortality and complication rates. The clear decrease in early mortality in the recent era appears to be due to better perioperative management of carcinoid syndrome with long-acting somatostatins, as well as increased surgical experience and patient selection.
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