Introduction: Up to half of patients with carcinoid syndrome develop carcinoid heart disease; this is thought to be mediated by serotonin from disordered tryptophan metabolism. Valve degeneration can lead to right heart failure which drives morbidity and mortality in this patient population. Surgical replacement of affected valves is an effective therapy. Peptide receptor radiotargeted therapy (PRRT) has demonstrated better progression-free survival compared to high dose sandostatin LAR.
Methods: We reviewed the clinical records of consecutive patients with carcinoid heart disease who underwent heart valve replacement surgery at a large single centre between 2003 and 2019. Patients were followed up for up to nine years.
Results: 26 patients with carcinoid heart disease underwent valve replacement surgery. Mean (S.D.) age was 61 (11) years, 54% female. All had liver metastasis, 31% lymph node, 23% bone and 3.9% lung. CGA at diagnosis mean (S.D.) 600 (1055), HIAA at diagnosis mean (S.D.) 317 (555). NYHA before surgery mean (S.D.) 2.0 (0.7); after surgery mean 1.2; at follow up mean (S.D.) 1.6 (0.8). Mean difference in NYHA score from before to after surgery 0.71 (P=0.002). 88.5% had two valves replaced (PR & TR), 3.9% one valve, 3.9% three valves and 3.9% four valves replaced. 13 of 26 patients (50%) received Lutathera PRRT therapy; 27% completed four cycles. All received somatostatin analogues. Mortality at 1, 2, 3, 4, 5 years of follow up was 42%, 50%, 47%, 46%, 50% respectively. In a Cox proportional-hazards model of survival from surgery, adjusting for age (Hazard ratio (HR) 0.96 [0.89, 1.03] (P=0.25)), four cycles of Lutathera demonstrated HR 0.087 [0.0079, 0.95] (P=0.045) indicating improved survival.
Conclusions: Patients who underwent surgery had relatively early carcinoid heart disease (NYHA II): patients were screened with NT-P-BNP and echocardiography. NYHA symptom scores fell after surgery. All patients received SST analogues, and many received PRRT. Completion of four cycles of Lutathera had a beneficial effect on survival from surgery, although the confidence interval was wide; a randomised controlled trial would be necessary to further determine a treatment effect.