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Introduction and Objective: Carcinoid Heart Disease (CaHD) develops from vasoactive substances released by neuroendocrine tumors causing significant patient morbidity and mortality necessitating surgical intervention. We performed a systematic review and meta-analysis to elucidate granular perioperative details and long-term outcomes in these patients.

Methods: Electronic search of Ovid, Scopus, Cumulative Index of Nursing and Allied Health Literature, and Cochrane Controlled Trials Register was performed. Nine articles comprising 416 patients who received surgery were selected. Primary outcomes investigated included patient characteristics, surgical characteristics and survival data. Study-level data were extracted and pooled for meta-analysis.

Results: Primary outcomes consisted of survival, length of stay and thirty-day mortality. Secondary outcomes included presence of right heart failure pre-operatively and type of valve replaced. Right heart failure was present in 48%. Moderate or severe regurgitation was present in 97% of tricuspid and 72% of pulmonary valves. 99% of tricuspid and 59% of pulmonary valves were replaced. Mean hospital length of stay was 16 days. Thirty-day mortality was 9%. Mean follow up was 25 months. Median survival was 3 years.

Conclusion: Surgical treatment of CaHD can be performed with acceptable short-term outcomes. However, overall survival appears to suffer from ongoing effects of the primary disease. Surgery is often performed after patients have extensive right-sided heart involvement. Overall, onset and duration of symptoms of carcinoid heart disease should be closely monitored to properly identify and refer patients who would most benefit from valvular surgery.