Acute and midterm outcomes of patients undergoing right-sided heart valve surgery for carcinoid heart valve disease

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Objectives: Carcinoid heart syndrome is a rare manifestation of metastatic neuroendocrine tumor that causes right heart valve dysfunction and subsequent symptomatic right heart failure, reducing long-term survival. In addition to symptomatic treatment, therapeutic strategies include surgical or interventional valve replacement for tricuspid and pulmonary valve regurgitation. Methods: Between 2011 and 2022, 12 patients with symptomatic right heart failure and carcinoid heart syndrome underwent elective valve surgery at our institution and were retrospectively included in this study. Procedural data, early clinical outcomes, survival, and time to reintervention were analyzed. The median follow-up period was 33 months, ranging from 2 to 131 months. Results: All patients underwent tricuspid bioprosthetic valve replacement with concomitant pulmonary valve replacement in 4 patients. A 3-dimensional endoscopic approach via right anterolateral minithoracotomy was conducted in 33.3% (4 out of 12) of cases, whereas a full median sternotomy was performed in 66.7% (8 out of 12). Beating-heart technique without crossclamping of the aorta was used in 58.3% (7 out of 12) of cases. Postoperative rethoracotomy for bleeding was required in 2 patients, and 1 patient developed acute kidney injury. No patients experienced permanent pacemaker implantation or stroke. One patient died during hospitalization due to acute hepatic failure in the presence of a preoperatively elevated Model for End-Stage Liver Disease score of 13, resulting in an in-hospital mortality rate of 8.3%. At final follow-up, overall survival was 41.7% (5 out of 12). Five patients required valve reintervention due to bioprosthetic degeneration associated with progression of the underlying neuroendocrine tumor, occurring 11 to 44 months after the index procedure. Two patients underwent surgical redo procedures (1 repeat tricuspid valve replacement and 1 repeat pulmonary valve replacement). One patient underwent pulmonary balloon valvuloplasty followed by 2 valve-in-valve procedures using balloon-expandable transcatheter heart valves. In addition, 2 patients underwent combined valve-in-valve interventions of both the tricuspid and pulmonary valves. Four of the 5 patients undergoing reintervention died during subsequent follow-up. Conclusions: The results from our single-center study provide descriptive information on current treatment approaches for this rare cardiovascular disease. Surgical valve replacement for carcinoid heart syndrome is technically feasible and associated with satisfactory early clinical outcomes. However, reinterventions are frequently required, largely driven by progression of neuroendocrine tumor disease. Consequently, the influence of valve intervention on long-term survival remains uncertain, and valve durability appears limited, as reflected by the high rate of repeat procedures. These findings underscore the necessity for close follow-up with dedicated heart valve units, enabling early detection of disease progression and bioprosthetic valve degeneration and ensuring optimized care through a multidisciplinary treatment approach.

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