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 left‐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.

Carcinoid Heart Disease – A Review of Pathophysiology, Clinical Manifestations, Diagnosis and Management

“…Interestingly, the number of valves replaced has not been found to be associated with mortality [50]. The choice of valve prosthesis is a matter of debate; however, bio-prosthetic valves are favored over mechanical valves for several reasons [8,51]. Bioprosthetic valves are associated with a lower risk of valve thrombosis, do not require long term anticoagulation and are associated with longer than expected survival in most patients with CHD [52].…”

What do we know about carcinoid heart disease in the present era?

“…CHD prevalence estimated in patients with carcinoid syndrome varies from 3% to 66% [8,[17][18][19][20], but in recent studies, has tended to be lower [17]. This might be due to an increase in somatostatin analogs usage and their impact on circulating serotonin levels, as well as improvements in diagnostic and surgical techniques [21][22][23].…”

Carcinoid Heart Disease Management: A Multi-Disciplinary Collaboration

“… 1 , 2 Of the approximately 180 000 patients with neuroendocrine tumors (NETs) in the US, 19% develop carcinoid syndrome (CS); among the patients who develop CS, 25%-65% may develop CaHD. 3-8 The exact estimates of CaHD incidence/prevalence are limited by the methodology of interrogating studies. Although the pathophysiology behind CaHD is not clearly defined, the majority of relevant published literature attributes its core mechanism to chronic overexposure to serotonin in cardiac cells.…”

“… 6 Echocardiographic findings in patients with CaHD demonstrate right-sided disease with tricuspid regurgitation is evident in all patients due primarily to thickened, retracted, immobile valve leaflets. 3 , 18 , 23 Concurrent pulmonary regurgitation has been reported in 59%-88% of cases, with stenosis of either the tricuspid or pulmonary valve less commonly reported in approximately one-third of patients with CaHD. 3 , 18 Left-sided disease is involved in <10% of cases, with regurgitation of the aortic and mitral valves prominent upon disease progression.…”

“… 3 , 18 , 23 Concurrent pulmonary regurgitation has been reported in 59%-88% of cases, with stenosis of either the tricuspid or pulmonary valve less commonly reported in approximately one-third of patients with CaHD. 3 , 18 Left-sided disease is involved in <10% of cases, with regurgitation of the aortic and mitral valves prominent upon disease progression. 3 , 24 CaHD diagnosis may be delayed 1.5 years or more, particularly if echocardiographic screening is not performed in patients with CS.…”