Abstract Background Neuroendocrine tumours (NETs) are uncommon cancers that can secrete vasoactive hormones, causing a range of symptoms. Approximately 50% of metastatic small bowel NETs develop carcinoid syndrome, with up to 50% historically developing carcinoid heart disease (CHD). With advances in medical therapy, contemporary characteristics and prognosis of CHD are unclear. Purpose To evaluate clinical characteristics, echocardiographic features, and outcomes of patients with NETs and CHD in a contemporary Canadian cohort and identify factors associated with CHD development and mortality. Methods This retrospective cohort study analyzed patients undergoing echocardiograms at a tertiary cancer centre-affiliated laboratory (2013-2023) with references to NETs or CHD in reports. Patients were classified based on whether they did or did not develop CHD in the study period. Baseline clinical characteristics, treatments, and outcomes were compared between the two groups. Baseline echocardiographic data (≤6 months from NET diagnosis) were reviewed to characterize features associated with CHD development. Interventions were compared between patients who survived 2.5 years after CHD diagnosis and those who survived ≥2.5 years or were alive by the end of the study period. Results In this 10-year period, 87 patients with NETs were identified 42 (48%) female; median age 61 (IQR 53-69) years and 18 (21%) developed CHD 8 (44%) female; median age 66 (IQR 59-71) years. Primary NETs of small bowel origin were more common in patients with CHD than those without CHD (83% vs. 55%, p=0.032). CHD patients exclusively had right-sided valve involvement: 100% tricuspid and 61% pulmonary; 2 (11%) had a patent foramen ovale. Compared to non-CHD patients at baseline echo, CHD patients demonstrated higher mean right atrial volume index (41mL/m2 vs 24mL/m2, p=0.001), higher mean right ventricular (RV) basal diameter (40mm vs. 34mm, p=0.006), and more frequent RV dysfunction (22% vs. 0%, p0.001). Surgical valve replacement was performed in 9 (50%) CHD patients and was indicated but deferred in 6 (33%) due to competing comorbidities. The median survival of CHD patients was 2.4 vs. 13.4 years without CHD (p0.001). NET progression and refractory heart failure were main reasons for mortality. Among CHD patients, primary NET resection was significantly associated with improved survival (p=0.009). Tyrosine kinase inhibitors and radiation therapy showed trends toward improved survival. Conclusions In our cohort of patients with NETs, CHD prevalence was lower than previously reported with all cases affecting only right-sided valves. Over half developed an indication for valve replacement though many were ineligible. Contemporary therapeutics, including medical and radiation therapies, showed trends toward improved survival. Further studies are needed to determine reasons behind differences in CHD prevalence, valvular involvement, and outcomes in the current era.Study design and findings Table 1.Clinical and echo data
Yoon et al. (Sat,) studied this question.