The path to surgery in carcinoid heart disease: a retrospective study and a multidisciplinary proposal of a new algorithm

Background: Development of carcinoid heart disease (CHD) is the major negative prognostic factor in patients with the carcinoid syndrome. The only effective treatment is valve replacement. However, the selection of candidates and determination of optimal timing remain unclear. Considerable variability in local screening and treatment strategies exist. Methods: In this single-centre study, we retrospectively analysed the diagnostic process and outcome of all CHD patients who underwent valve surgery between 2000 and 2016. We propose a new CHD screening and management algorithm. Results: All patients (n = 15), mean age 64 +/- 7, underwent tricuspid valve surgery. In 14 of them (93%) an additional valve was replaced. In only a minority of patients (27%) CHD diagnosis was established by screening. Survival after 1, 3, 12 and 24 months was 93%, 80%, 53% and 33%, respectively. Causes of death included infections and critical illness immediately postoperatively, and tumour progression and right heart failure in the longer term. There was a trend (p = .099) towards better preoperative right ventricular function in the patients who survived more than 12 months postoperatively (TAPSE 20mm +/- 4) compared to those who died between 3 to 12 months after surgery (TAPSE 16mm +/- 1). The former group had a shorter mean interval from diagnosis of the carcinoid syndrome to cardiac diagnosis than the latter (13 vs. 105 months, p = .014). Conclusion: Mortality after valve replacement for CHD remains high. A probably underestimated cause is late referral for cardiac surgery. We propose a systematic, multidisciplinary approach to all carcinoid syndrome patients.