Background: Carcinoid heart disease (CHD) is a rare carcinoid syndrome (CS) manifestation, often linked to liver metastasis releasing vasoactive amines. CHD can also result from ovarian neuroendocrine tumours (NETs), an uncommon association.
Cases Presentations: We reviewed three female patients at our NET centre, averaging 63years of age (59-67years). They all had primary ovarian NETs causing CS and CHD.
Discussion: In all patients, investigations identified a diagnosis of CS and CHD secondary to primary ovarian NETs, evident in Tables 1&2. Table2 showed a shared pattern of dilated right ventricles and severe Pulmonary and Tricuspid valves regurgitations, emphasising the cardiac burden. Treatment encompassed both valves replacements alongside ovarian tumour removal, with receiving preoperative somatostatin analogues.One initially declined cardiac surgery but later accepted. Postoperative results uniformly demonstrated disease remission clinically, biochemically and radiologically, reflecting successful tumour resection and cardiac intervention.
|Case1||CgA: 24nmol/L 5HIAA: 857umol/L BNP: 565ng/L||CgA: 6nmol/L 5HIAA: 17umol/L|
|Case2||CgA: 2504pmol/L 5HIAA: 604umol/L BNP: 1615ng/L||CgA: 27pmol/L 5HIAA: awaiting|
|Case3||CgA: 39.7nmol/L 5HIAA: 536umol/L BNP: 477ng/L||CgA: 17.5nmol/L 5HIAA: 150umol/L|
|Case1||ECHO: EDD 50mm Impaired RV||Awaiting|
|Case2||MRI: RVEDV 240ml RVEF 63%||Normal RV|
|Case3||ECHO: RVEDarea 14.8cm2/m2 EDV 36.7ml||RVEF 56+/-6%|
Conclusion: Tumour excision and cardiac interventions provide optimistic possibilities for curing CS and CHD linked to primary ovarian NETs. Early diagnosis and multidisciplinary involvement improve clinical outcomes.
References: 1. Preda VA, Chitoni M, Talbot D, et al Primary Ovarian Carcinoid: Extensive Clinical Experience With an Underrecognized Uncommon Entity International Journal of Gynecologic Cancer 2018;28:466-47. 2. Mansour S, Anaka MR, Al-Agha R. A Case of Primary Insular Ovarian Carcinoid Tumor with Hyperandrogenism and Carcinoid Heart Disease. Am J Case Rep. 2022 Oct 1;23:e937403. doi: 10.12659/AJCR.937403. PMID: 36181247; PMCID: PMC9536143. 3. Simões-Pereira J, Wang LM, Kardos A, Grossman A. Carcinoid Syndrome and Carcinoid Heart Disease as Manifestations of Non-Metastatic Ovarian Neuroendocrine Tumour. Acta Med Port. 2017 May 31;30(5):421-425. doi: 10.20344/amp.7713. Epub 2017 May 31. PMID: 28865508.