A 33-year follow-up after valvular surgery for carcinoid heart disease

Abstract

Aims Valvular surgery has improved long-term prognosis in severe carcinoid heart disease (CaHD). Experience is limited and uncertainty remains about predictors for survival and strategy regarding single vs. double-valve surgery. The aim was to review survival and echocardiographic findings after valvular surgery for CaHD at our institution. Methods and results Between 1986 and 2019, 60 consecutive patients, median age 64 years, underwent valve surgery for severe CaHD. Operations involved combined tricuspid valve replacement (TVR) and pulmonary valve replacement (PVR) in 42 cases, and TVR-only or TVR with pulmonary valvotomy (no PVR) in 18 patients. All implanted valves were bioprosthetic. Preoperative echocardiography, creatinine, NT-pro-brain natriuretic peptide (NT-pro-BNP), and 24-h urinary 5-hydroxyindoleacetic acid (5-HIAA) were obtained. 30-Day mortality was 12% (n=7), and 8% for the most recent decade 2010–2019. Median survival was 2.2 years and maximum survival 21 years. Patients undergoing combined TVR and PVR had significantly higher survival compared with operations without PVR (median 3.0 vs. 0.9 years, P = 0.02). Preoperative levels of NT-pro-BNP and 5-HIAA in the top quartile predicted poor survival. On preoperative echocardiograms, pulmonary regurgitation was severe in 51% and indeterminate in 17%. Postoperative echocardiography confirmed relatively good durability of bioprostheses, relative to the patients’ limited oncological life expectancy. Conclusion Valvular surgery in CaHD has an acceptable perioperative risk. Survival for combined TVR and PVR was significantly higher compared with operations without PVR. Bioprosthetic valve replacement appears to have adequate durability. Preoperative echocardiography may underestimate pulmonary pathology. Combined TVR and PVR should be considered in most patients.

Arterial function, biomarkers, carcinoid syndrome and carcinoid heart disease in patients with small intestinal neuroendocrine tumours

“…They consist of tumour debulking surgery, medication to reduce serotonin concentrations (i.e., somatostatin analogues), PRRT, and treatments for right-sided heart failure, including valve surgery. The diagnosis of CHD is associated with a diminished prognosis when compared to SI-NET patients without CHD [ 20 ], and the mortality of CHD patients is also high following valve surgery [ 43 ]. Early recognition of SI-NET patients with high risk for CHD is thus important to improve their prognosis.…”

Carcinoid heart disease in patients with midgut neuroendocrine tumours

“…The improvement of CHD prognosis can be explained by the wider use of SSAs and introduction of modern antitumour therapies such as PRRT. Moreover, improvements in CHD screening and progresses in surgical techniques have yielded an increase in the number of patients operated on for CHD – and earlier timing of surgery, with postoperative mortality decreasing from 25% to <10% in 20 years 17,19,48,55 . Subsequently, the proportion of patients with CHD recurrence on bioprostheses requiring reintervention has gradually increased, accounting for 5%–10% of all patients in recent studies 19,48,67 …”

“…56 Overall, the pulmonary valve is replaced in 70%-90% of cases (Table 3). 48,[51][52][53][54][55] Should PFO be detected, its closure is usually performed during the same procedure, accounting for 10%-40% of patients.…”

“…Almost all reported cases of surgical valve replacement in patients with CHD involved tricuspid valve, but all four heart valves may be involved (Table 3). 48,[51][52][53][54][55] As the pulmonary valve is often poorly visualized on TTE, evaluation of pulmonary valve involvement during surgery is advised (Figure 6). In a series of 11 patients operated on for CHD with systematic intraoperative inspection of pulmonary valve, detection of severe pulmonary valve involvement resulted in unplanned replacement in 27% of patients.…”

“…Moreover, improvements in CHD screening and progresses in surgical techniques have yielded an increase in the number of patients operated on for CHDand earlier timing of surgery, with postoperative mortality decreasing from 25% to <10% in 20 years. 17,19,48,55 Subsequently, the proportion of patients with CHD recurrence on bioprostheses requiring reintervention has gradually increased, accounting for 5%-10% of all patients in recent studies. 19,48,67 The main pejorative prognostic factors of patients with CHDin addition to surgical replacementare older age, advanced New York Heart Association (NYHA) class, high NT-proBNP and 5-HIAA levels and right ventricular dilatation (Table 4).…”