A natural history of carcinoid heart disease in the modern management era

Abstract

The development of carcinoid heart disease (CaHD) is still relatively unknown at present. It is difficult to define an optimal follow-up for patients initially free from cardiac involvement. The aim of this study was to assess the prevalence and the evolution of CaHD using annual echocardiographic follow-up. We reviewed from our database 137 patients (61 ± 12 years, 53% men) with histologically proven neuroendocrine tumor between 1997 and 2017. All patients underwent serial conventional transthoracic echocardiographic studies. Right-sided and left-sided CaHD were systematically assessed. We used a previous validated echocardiographic scoring system of severity for the assessment of CHD. An increase of 25% of the score was considered as significant. Mean follow-up was 2.6 ± 3.5 years [0;16]. Prevalence of CaHD was 27% (37 pts) at baseline and 36% (49 pts) at the end of follow-up. Among patients with initial CaHD followed for more than one year, disease progression was observed in 28% of cases. Among the patients free from initial cardiac involvement, an onset of the disease was observed during follow-up in 21% of cases. The onset of CHD could be very late, more than 5 years from the initial echocardiographic examination in 42% of our cases ( Figure 1 ). This late occurrence of CaHD was only observed in patients presenting with new resumption of neuroendocrine tumor (symptoms, increased of 5-HIAA, occurrence of new metastasis). Our study demonstrated that in patients without initial CaHD, cardiac involvement may occur tardily after a normal initial assessment. Our data suggest the need for prolonged echocardiographic follow-up in patients presenting with a resumption of tumor process ( Figure 1 ).