Optimized Outcomes Using a Standardized Approach for the Treatment of Patients with Carcinoid Heart Disease

Abstract

Background: Carcinoid heart disease (CHD) is common in patients with carcinoid syndrome (CS). Surgical treatment improves the poor prognosis of CHD, although the reported peri-operative mortality is high (∼17%). We attempted to improve outcomes by implementation of a protocol for the management of patients with CHD at a UK Neuroendocrine Centre of Excellence and report our experience. Methods: All patients treated for CHD between 2008 and 2015 were included. Peri-operative treatment included surgical features such as invasive pulmonary valve (PV) inspection and preservation of the tricuspid subvalvular apparatus. Results: A total of 11 patients were treated; the median age was 63 years (IQR: 56-70). Ten patients underwent both pulmonary valve replacement (PVR) and tricuspid valve replacement (TVR); 1 patient underwent isolated TVR. One patient had additional aortic valve replacement (AVR), another one coronary artery bypass grafting. Bioprostheses (BP) were used in all patients, stented for TVR and AVR, stentless for PVR. Invasive PV inspection caused unplanned PVR in 3 cases (27.3%). All patients were discharged home. One patient (9.1%), who had had previous TVR by another surgeon, had right heart failure (RHF) during follow-up. One death occurred due to progression of CS (day 346). The carcinoids' primary was resected in 5 patients (45.5%) 10 months (4.5-19.5) after cardiac surgery. Conclusion: Excellent results were achieved in patients with CHD. PV stenosis can be underestimated by echocardiography; therefore, intraoperative inspection is recommended. Right ventricular geometry should be respected to prevent RHF. BP should be used, as these patients are likely to undergo future non-cardiac surgeries.

Carcinoid heart disease: a guide for screening and timing of surgical intervention

“…Due to the complexity and rarity of CaHD, patients should be treated in a specialised centre by a multidisciplinary team involving the oncologist, endocrinologist, gastroenterologist, cardiologist, and abdominal and cardiothoracic surgeons [ 14 , 51 , 52 ]. With regards to the indications of screening for CaHD, no consensus has been reached.…”

“…The UK and Ireland Neuroendocrine Tumour Society (UKINETS) guidelines recommend that all patients with midgut NETs and all patients with carcinoid syndrome should be screened for CaHD, which may include measuring NT-proBNP or echocardiography [ 52 ]. Others suggest echocardiography should only be performed in patients with carcinoid syndrome [ 52 ] or with elevated NT-proBNP-[ 51 ] or 5‑HIAA levels [ 52 ]. European Neuroendocrine Society (ENETS) guidelines recommend echocardiographic screening only in patients with carcinoid syndrome or if urinary 5‑HIAA and/or chromogranin A are elevated [ 19 ].…”

Nowotwory neuroendokrynne jelita cienkiego i wyrostka robaczkowego — zasady postępowania (rekomendowane przez Polską Sieć Guzów Neuroendokrynnych)

“…The gold standard in CHD diagnosis is echocardiography, which should be performed in all patients with carcinoid syndrome. Possible NET metastases to the pericardium should also be considered [3,[27][28][29].…”

Carcinoid Heart Disease From a Primary Liver Carcinoid Tumor

“…Somatostatin analogues such as octreotide improve symptoms and survival in patients with CaHD by reducing the levels of vasoactive peptides that cause the symptoms. 3 …”