Objectives: Carcinoid heart disease (CaHD) is a rare manifestation of metastasic neuroendocrine tumor (NET) causing right valvular dysfunction and subsequent symptomatic right heart failure with reduced long-term survival. However, limited data exist regarding outcomes of right-sided heart valve surgery in such patients. The aim of this study was to analyze acute and mid-term outcomes in patients with CaHD undergoing surgery for tricuspid and pulmonary valve regurgitation

Methods: Between 06/2011 and 04/2022, 12 patients (75% male; mean age 62.0±12.4 years, STS-Score 2.5±1.6%) with symptomatic right heart failure and CaHD underwent elective valve surgery at our institution and were retrospectively included in our study. Procedural data, early clinical outcomes, survival and time of reintervention were analyzed.

Results: All patients received tricuspid biological valve replacement (TVR) and 4 patients received concomitant pulmonary valve replacement (PVR). Surgical approach was 3D-endoscopic via right anterolateral minithoracotomy in 33.3% (4/12) and median sternotomy in 66.7% (8/12) of cases. Surgery was performed in beating heart-technique in 58.3% (7/12) of patients. Intensive care unit and total hospital stay were 3.8±4.1 and 14.6±11.5 days. Rethoracotomy due to postoperative bleeding was necessary in 2 patients (16.7 %) and acute kidney injury occurred in one patient. No permanent pacemaker implantation and no stroke occurred in the study group. Post-procedure echocardiography showed competent TVR and PVR in all cases. Overall 30-day mortality was 8.3% (1/12) due to acute hepatic failure in one patient. During follow-up two patients deceased after 19 months and 131 months due to progression of NET and right ventricular failure. Consequently to progression of NET, five patients received reintervention for degeneration of the implanted heart valve in a period between 11 months and 44 months. Two patients underwent redo surgery (one re-TVR, one re-PVR). One patient initially underwent pulmonary balloon valvuloplasty followed by two valve-in-valve intervention (VIV) with balloon-expandable transcatheter heart valve (BE-THV). Two other patients received a VIV procedure for pulmonary and tricuspid valve with BE-THV.

Conclusions: Our single center results give insights in treatment options of a rare cardiovascular disease. Treatment of CaHD is technically feasible with acceptable early clinical and hemodynamic outcomes. However, treatment strategies involve reinterventions in a number of patients depending on NET progression. Therefore, a multidisciplinary team for adequate treatment of this seldom patient cohort is mandatory.