Background: Carcinoid Heart Disease (CHD) is a well-documented but devastating complication of metastatic neuroendocrine tumours (mNETs). Occurring in approximately 20% of patients with Carcinoid Syndrome (CS) the prognosis is poor, with a 3-year survival of 31% versus 68% in patients with mNETs without CHD1. Management of these patients requires a multidisciplinary approach to manage tumour load via medical and/or surgical options, alongside control of hormonal excesses, heart failure (HF) management and consideration of valve surgery2. Typically, medical management of HF due to CHD has been considered a palliative measure with symptomatic benefit only, rather than a life-prolonging treatment. Valve surgery conversely has been shown to improve life expectancy in these patients from 11 months to 58 months3. Unfortunately, the peri-operative mortality of these patients is high with a 17% 30-day mortality3, therefore appropriate patient selection is crucial.
Case Presentation: We present a review of 4 patients with CHD who were deemed not suitable for surgery and have been subsequently medically managed. Aged between 60-83 years, these patients presented between 2019-2022 with symptoms of CS and were subsequently diagnosed with mNETs and CHD. All were deemed unfit for surgery and were managed with somatostatin analogues alongside diuretic therapy.
Discussion: To date the survival of these patients has exceeded the previously quoted life expectancy of 11 months (1 death at 47 months, others 11-34 months and ongoing).
Conclusion: We demonstrate that with excellent medical management patients could expect some survival benefit as well as improvement to symptoms and quality of life.
References: 1. Clement D, Ramage J, Srirajaskanthan R. Update on Pathophysiology, Treatment, and Complications of Carcinoid Syndrome. J Oncol. 2020 Jan 21;2020:8341426. doi: 10.1155/2020/8341426. PMID: 32322270; PMCID: PMC7160731. 2. Steeds R, Sagar V, Shetty S, Oelofse T, Singh H, Ahmad R, Bradley E, Moore R, Vickrage S, Smith S, Yim I, Elhassan YS, Venkataraman H, Ayuk J, Rooney S, Shah T. Multidisciplinary team management of carcinoid heart disease. Endocr Connect. 2019 Dec;8(12):R184-R199. doi: 10.1530/EC-19-0413. PMID: 31751305; PMCID: PMC6933832. 3. Chengyue Jin, Ajay Nair Sharma, Balasingam Thevakumar, Muhammad Majid, Shahad Al Chalaby, Nene Takahashi, Ashraf Tanious, Aro Daniela Arockiam, Neil Beri, Ezra A. Amsterdam; Carcinoid Heart Disease: Pathophysiology, Pathology, Clinical Manifestations, and Management. Cardiology 20 January 2021; 146 (1): 6573.