This protocol covers carcinoid heart disease arising in the setting of carcinoid syndrome with severe carcinoid involvement of the mitral or aortic valve, in patients with an expected survival of 12 months or longer.
Clinical scenario: Carcinoid syndrome with severe carcinoid valvular heart disease of the mitral or aortic valve, and expected survival ≥ 12 months. The combination of systemic neuroendocrine disease and advanced valvular pathology creates a high-stakes peri-operative environment requiring coordinated surgical and medical management.
Approach overview: Valve surgery — replacement or repair — is central to this protocol, together with specific intravenous somatostatin analogue prophylaxis administered around the time of the procedure. Additional structural considerations may also apply. The full sequence, agent selection, timing, and complete surgical criteria are detailed in the structured protocol.
DOI: 10.1093/eurheartj/ehac244
Valve replacement or repair surgery is recommended in symptomatic patients with severe carcinoid mitral or aortic VHD and an expected survival ≥12 months.
Administration of i.v. somatostatin analogues (e.g. octreotide) is recommended to avoid a peri-operative carcinoid crisis.
The infusion should be started on the morning of the procedure (up to 12 h pre-operatively), continued throughout the procedure (surgery, pre-operative coronary angiography, pacemaker implantation), and post-operatively for at least 48 h following valve surgery or until stable if a carcinoid crisis is triggered post-operatively.
In patients with left-sided carcinoid valvular involvement, closure of interatrial shunts should be considered, although only sparse data exist for this approach.
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