Survival trends in the management of carcinoid heart disease among patients with neuroendocrine neoplasms

BACKGROUND: Nearly 20% of patients with neuroendocrine tumors (NET) develop carcinoid syndrome (CS), and 20-25% of those with CS develop carcinoid heart disease (CHD). Contemporary risk factors for CHD and prognostic variables in NET management remain limited. This study aimed to characterize the clinical and imaging factors associated with the development, and prognostic impact of carcinoid heart disease in patients with neuroendocrine neoplasms. METHODS: A single-institution, retrospective analysis of well-differentiated NET patients from January 2010 to December 2024 was conducted. Cox proportional hazards regression assessed associations between baseline covariates and CHD development (primary endpoint) and overall survival (OS; secondary endpoint). CHD was defined as moderate or severe tricuspid regurgitation on echocardiogram with echocardiographic features consistent with carcinoid valvulopathy. Survival curves were estimated using Kaplan-Meier methods. A p-value < 0.05 was considered significant. RESULTS: We identified 270 patients with NET. Median age was 64.5 years; 52% were male; 5.6% developed CHD. CS was present in 10% (n = 27), and 41% of those with CS developed CHD. Fifteen patients (5.6%) developed carcinoid heart disease. Common primary sites were lung (26%), pancreas (17.4%), and small intestine (16.2%). CHD was associated with more hospitalizations and greater use of systemic or multimodal therapies. For OS, median OS for CHD patients was 90.8 months, while median OS was not reached for the full NET cohort. CHD independently predicted worse OS (HR 7.09; 95% CI: 1.84-27.40; p = 0.004). Systemic therapy use (HR 8.97; p = 0.0101) and high-grade tumors (HR 6.92; p < 0.001) were also associated with worse OS. CONCLUSION: In NET patients, CHD and high-grade tumors were associated with worse OS. Systemic therapy use was associated with worse overall survival, likely reflecting more advanced disease rather than a causal treatment effect. Although CHD incidence is low, early detection and close monitoring remain essential.