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Risk Factors for the Development and Progression of Carcinoid Heart Disease

https://doi.org/10.1016/j.amjcard.2010.12.025Get rights and content

The development of valvular heart disease in patients with carcinoid syndrome is thought to be related to the secretion of vasoactive substances by a tumor. We sought to identify modifiable risk factors for the development of carcinoid heart disease because this may help define strategies to attenuate the disease process. Two hundred fifty-two patients with carcinoid syndrome were prospectively followed with serial echocardiograms at 6-month intervals. Clinical characteristics, biochemical markers, and radiologic markers were measured at set intervals. An echocardiographic scoring system was applied. Patients were defined as having progression of carcinoid heart disease if the echocardiographic score increased by ≥25%. After a median follow-up of 29 months, 44 patients developed carcinoid heart disease or had progression of existing valvular dysfunction. At time of progression of carcinoid heart disease compared to the previous 6 months, there was a significant increase in median levels of 5-hydroxyindoleacetic acid (5-HIAA; 791 vs 460.5 μmol/24 hours) and flushing episodes (4.5 vs 2 episodes per day). Independent predictors of the development or progression of carcinoid heart disease were a 5-HIAA level ≥300 μmol/24 hours and ≥3 episodes of flushing per day. 5-HIAA levels of ≥300 to 599, 600 to 899, and >900 μmol/24 hours conferred 2.74, 3.16, and 3.40 times the risk of progression of carcinoid heart disease, respectively. In conclusion, a 5-HIAA level ≥300 μmol/24 hours and ≥3 flushing episodes per day are predictors of the development or progression of carcinoid heart disease.

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Methods

We conducted an observational cohort study. Patients with histologically proved metastatic carcinoid tumor of midgut origin and carcinoid syndrome were consecutively and prospectively recruited from the neuroendocrine tumor clinic at the Royal Free Hospital (London, United Kingdom) from April 2006 through January 2010. All patients gave written informed consent for the study. The study was approved by the institution's local ethics committee.

All patients were evaluated at 4- to 6-month

Results

Two hundred fifty-two patients were recruited. Median follow-up was 29 months (interquartile range 24 to 36). At baseline 41 patients had carcinoid heart disease. Forty-four patients developed carcinoid heart disease during the study period or had progression of existing carcinoid heart disease (15 patients developed and 29 patients had progression). Thirty-four patients died during the study period (13 patients had carcinoid heart disease). Twenty-two patients underwent cardiac valve

Discussion

The development of carcinoid heart disease and subsequent development of right-sided heart failure is a major cause of morbidity in patients with carcinoid syndrome. Risk factors for development and progression of carcinoid heart disease are poorly defined.

In a retrospective analysis Moller et al6 demonstrated that progression of carcinoid heart disease is related to increased levels of urinary 5-HIAA and use of chemotherapy. Denney et al13 performed a prospective study, although only 23

Acknowledgment

We are grateful for statistical advice provided by Colette Smith, PhD, Department of Infection and Population Health, UCL Medical School.

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