One of the most common functioning syndromes associated with neuroendocrine tumors (NET) is the carcinoid syndrome (CS). By releasing vasoactive substances, these tumors can cause fibrotic complications, including right-sided valve heart fibrosis, named carcinoid heart disease (CHD). Factors associated with the onset and progression of CHD are poorly understood. We aimed to investigate prognostic factors associated with CHD.
Retrospective study of consecutive patients (pts) with advanced NET and CS and/or elevated 24h-urinary 5HIAA who performed an echocardiogram to screen for CHD. CHD was defined as echocardiographic evidence of moderate to severe tricuspid or pulmonar regurgitation.
From 2009 to 2017 42 pts were included: Median age was 54.4 (19 – 85) years, 24 were female, 69% had midgut NET. The frequency of CHD was 38% (16 pts) CHD was not associated with age (p = 0.79), sex (p = 0.38), bone metastasis (p = 0.66), flushing (p = 0.14) or diarrhea (p = 0.53); the median urinary level of 5HIAA at diagnosis of CHD was numerically higher, albeit not significant, among CHD pts (p = 0.20). CHD was significantly associated with higher volume (>50% of parenchyma) of liver metastases [OR 13.86 (2.57 – 74.68), p = 0.002]. Time from symptoms to diagnosis of NET was borderline significant (p = 0.08). When CHD was defined as at least mild valve regurgitation, the frequency of CHD was 45% (19 patients) and we observed a significant association between the presence of cardiovascular comorbidities and CHD [OR 6.58 (1.09; 39.78), p = 0.040].
CHD is highly frequent among pts with CS. We found that high liver tumor burden and possibly, longer time of symptoms until diagnosis of NET were associated with CHD. Such findings probably imply that a delayed diagnosis negatively affects CS patients, increasing the risk of CHD. Interestingly, we found that concurrent cardiovascular disease was associated with CHD, as a potential predisposing factor.
Clinical trial identification
Legal entity responsible for the study
Instituto do Câncer do Estado de São Paulo
All authors have declared no conflicts of interest.