Carcinoid heart disease is a progressive right-sided valvulopathy caused by serotonin and other vasoactive mediators released by metastatic neuroendocrine tumours. As oncological therapies have extended survival, cardiac disease has become a leading determinant of mortality. Operative mortality has decreased to 5–6% in contemporary high-volume centres, and long-term survival appears increasingly determined by tumour biology rather than cardiac disease when surgery is appropriately timed. The principal determinant of operative outcome is preoperative right ventricular function; symptom-based referral alone is insufficient because many patients remain compensated until ventricular dysfunction is advanced. This review synthesises the evidence on surgical timing, operative strategy, prosthesis selection, perioperative endocrine management, and emerging transcatheter options. Tricuspid valve replacement is required in the majority of patients, with concomitant pulmonary valve replacement advocated where concurrent disease is present. Bioprosthetic valves are preferred. Continuous perioperative octreotide infusion has substantially reduced the incidence of carcinoid crisis. Structured multidisciplinary decision-making integrating echocardiographic surveillance, biomarker monitoring, and oncological status assessment is essential.