Abstract Background Carcinoid heart disease (CHD) is a complication of neuroendocrine tumours (NETs) with significant cardiovascular morbidity and mortality. It causes fibrotic damage to predominantly the right-sided valves with subsequent tricuspid valve (TV) and/or pulmonary valve (PV) regurgitation leading to right ventricular (RV) dilatation and dysfunction. The definitive treatment is valve replacement commonly using bioprosthetic valves. Case studies have suggested that bioprosthetic valve degeneration may occur due to ongoing carcinoid plaque deposition, but serial data is lacking. Purpose To assess the trajectory of prosthetic valve function on serial transthoracic echocardiography (TTE) in patients with CHD and determine the effect on long-term outcomes. Methods A retrospective multicentre observational cohort study including 81 patients who had valve replacement for CHD between 2005-2022. TTE data assessing prosthetic valve function (peak velocity, mean gradient, para/transvalvular regurgitation) and any associations with outcome were identified. Results Eighty-one patients (64±8 years, 56% male) were followed up for a median of 24 months (IQR: 8-48), during which there were 52 deaths, an estimated median survival of 32 months and 1-, 3- and 5-year survival rates of 69%, 48% and 38%, respectively. The post-operative TTE was carried out at a median of 10 days (IQR: 7-47) after surgery. Of the 71 patients with available post-operative TTEs, 48 had >1 TTE. No significant trends were seen in TV parameters over time. PV peak velocity and mean gradient increased significantly over time (p=<0.001). Increasing TV peak velocities were significantly associated with shorter survival (p=0.008) and those with increases of >5% per year had 5-year survival of 0% compared to 70% in those with decreasing by <5% per year. Increasing PV mean gradients were associated with longer survival (p=0.014), with 5-year survival 35%, 55%, and 91% for patients with increases of <0, 0-2 and ≥2 mmHg per year, respectively. This may reflect RV remodelling and improved RV function. There were 5 cases of prosthetic valve dysfunction, with only 1 needing re-intervention with transcatheter valve-in-valve procedure. A cause of death was available for 39 patients, 46% were due to NET causes and 31% due to cardiac causes. Conclusions Our data suggests that bioprosthetic valve degeneration in CHD is uncommon. TV function did not change over time and PV velocities although increased with time did not lead to significant valve dysfunction. The main cause of death was due to advancing NET disease. This raises the question as to whether transcatheter replacement may be a viable alternative in patients with CHD, who are facing a median survival of less than 3 years.
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