Progression of carcinoid heart disease in the modern management era

Abstract

BACKGROUND: The development of carcinoid heart disease (CaHD) is still relatively unclear. It is difficult to define an optimal follow-up for patients without any cardiac involvement at baseline. The aim of this study was to assess the prevalence and natural history of CaHD by annual echocardiographic examinations. METHODS AND RESULTS: We studied 137 consecutive patients (61±12 years, 53% men) with proven digestive endocrine tumor and carcinoid syndrome between 1997 and 2017. All patients underwent serial conventional transthoracic echocardiographic studies. Right-sided and lef t-sided CaHD were systematically assessed. We used a previous validated echocardiographic scoring system of severity for the assessment of CaHD. An increase of 25% of the score was considered to be significant. Mean follow-up was 54±45 months. Prevalence of CaHD was 27% at baseline and 32% at 5-year follow-up. Disease progression was reported in 28% of patients with initial CaHD followed up for >2 years (n=25). In patients without any cardiac involvement at baseline, occurrence of disease was 21%. CaHD occurred >5 years from the initial echocardiographic examination in 42% of our cases, especially in patients presenting with new recurrence of a digestive endocrine tumor. An increase of urinary 5-hydroxyindoleacetic acid by 25% during follow-up was identified as an independent predictor of CaHD occurrence during follow-up (hazard ratio [HR], 5.81; 95% CI, 1.19– 28.38; P=0.03), as well as a maximum value of urinary 5-hydroxyindoleacetic acid >205 mg/24 h during follow-up (HR, 8.41; 95% CI, 1.64–43.07; P=0.01). CONCLUSIONS: Our study demonstrates that in patients without initial CaHD, cardiac involvement may occur late and is related to serotonin. Our data emphasize the need for cardiologic follow-up in patients with recurrence of the tumor process.