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

Hart, Einar A.; Meijs, Timion A; Meijer, Rudy; Dreijerink, Koen M.A.; Tesselaar, Margot E. T.; Groot, C. A. de; Valk, Gerlof D.; Chamuleau, Steven A.J.

doi:10.1007/s12471-017-1011-2

Cited by 24 publications

(32 citation statements)

References 55 publications

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“…CaHD in the left side of the heart is less severe than in the right side of the heart. Left-sided heart involvement in carcinoid syndrome happens in patients with persistent foramen ovale or bronchial carcinoid [ 16 ].…”

Section: Discussionmentioning

confidence: 99%

“…Surgical resection of the primary tumor and resection of liver metastases appear to decrease the cardiac progression in CaHD and improve prognosis [ 16 ]. Since hepatic surgery exposes a patient to the risk of perioperative bleeding, hepatic intra-arterial treatment (transarterial chemoembolization, selective internal radiotherapy) is well suited to patients with hepatic metastases [ 21 ].…”

Section: Discussionmentioning

confidence: 99%

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Carcinoid heart disease revealed by cyanosis with both right and left valvular involvement: a case report

Khay¹,

Arous²,

Bentaoune³

et al. 2018

J Med Case Reports

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BackgroundCarcinoid heart disease is a frequent complication of carcinoid syndrome. It is related to the release by the carcinoid tumor and/or its metastases of bioactive substances such as serotonin. It is characterized by right-sided valvular involvement and can lead to right-sided heart failure. It is a prognostic factor of carcinoid syndrome.Case presentationWe report the case of a 53-year-old African woman with an endocrine tumor of her small intestine complicated by carcinoid heart disease, revealed by right-sided heart failure. The diagnosis was confirmed by transthoracic echocardiography, which showed a severe tricuspid regurgitation with a patent foramen ovale, and by increased serum chromogranin A and urinary 5-hydroxyindoleacetic acid.Initially she was treated with medical therapy (furosemide and injection of somatostatin). Afterwards she was proposed for surgery. The evolution of her treatment was good.ConclusionsCarcinoid syndrome is complicated in 60% of the cases of a heart disease, and is responsible for an important morbidity and mortality. The prognosis of patients with carcinoid heart disease has improved in recent years through somatostatin analogues and the cardiac surgery.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Carcinoid heart disease revealed by cyanosis with both right and left valvular involvement: a case report

Khay¹,

Arous²,

Bentaoune³

et al. 2018

J Med Case Reports

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“…This involves secretion of vasoactive substances, leading to plaque-like deposits of fibrous tissue on cardiac valve cusps and leaflets. Our patient had a nonfunctioning NET, negative for all biomarkers including 5-hydroxyindoleacetic acid (5-HIAA) and chromogranin [ 4 - 5 ]. Congestive heart failure was due to intra-cardiac metastasis by the tumor.…”

Section: Discussionmentioning

confidence: 99%

Right Ventricular Outflow Obstruction Due to Metastatic Neuroendocrine Tumor

Sood

Chiadika

Everett

et al. 2018

Cureus

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Neuroendocrine tumors (NETs) are rare malignant tumors that arise from neuroendocrine cells of the gastrointestinal tract and often metastasize to the liver, lung, and bone. Cardiac metastasis of NETs is uncommon. We report a patient with a past medical history of a neuroendocrine tumor of the left femur presenting with signs and symptoms of new onset heart failure. Transthoracic echocardiogram and cardiac magnetic resonance showed a large mass within the right ventricle causing right ventricular outflow obstruction. A positron emission tomographic/computed tomographic scan (PET-CT) revealed increased uptake of fluorodeoxyglucose (FDG) activity within the right ventricle consistent with metastasis. Cardiac biopsy of the right ventricular mass revealed metastatic nonfunctioning neuroendocrine tumor. In view of the fact that it was a tumor that caused the right ventricular obstruction, the patient was started on chemotherapy with improvement of symptoms. This case highlights that in patients with a history of neuroendocrine tumor presenting with heart failure, cardiac metastasis should be included in the differential.

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“…Valve replacement surgery or valvuloplasty is the only definitive treatment and should be planned carefully and early in the course of the disease [76,85]. e choice of valve prosthesis is still under debate: bioprosthesis is preferred to mechanical valves, due to the absence of need for anticoagulation and the risk of bleeding complications, but these valves may be less durable and more vulnerable for the recurrence of disease.…”

Section: Complications: Carcinoid Heart Diseasementioning

confidence: 99%

Update on Pathophysiology, Treatment, and Complications of Carcinoid Syndrome

Clement

Ramage

Srirajaskanthan

2020

Journal of Oncology

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Carcinoid syndrome (CS) develops in patients with hormone-producing neuroendocrine neoplasms (NENs) when hormones reach a significant level in the systemic circulation. The classical symptoms of carcinoid syndrome are flushing, diarrhoea, abdominal pain, and wheezing. Neuroendocrine neoplasms can produce multiple hormones: 5-hydroxytryptamine (serotonin) is the most well-known one, but histamine, catecholamines, and brady/tachykinins are also released. Serotonin overproduction can lead to symptoms and also stimulates fibrosis formation which can result in development of carcinoid syndrome-associated complications such as carcinoid heart disease (CaHD) and mesenteric fibrosis. Transforming growth factor beta (TGF-β) is one of the main factors in developing fibrosis, but platelet-derived growth factor (PDGF), basic fibroblast growth factor (FGF2), and connective tissue growth factor (CTGF or CCN2) are also related to fibrosis development. Treatment of CS focuses on reducing serotonin levels with somatostatin analogues (SSA’s). Telotristat ethyl and peptide receptor radionuclide therapy (PRRT) have recently become available for patients with symptoms despite being established on SSA’s. Screening for CaHD is advised, and early intervention prolongs survival. Mesenteric fibrosis is often present and associated with poorer survival, but the role for prophylactic surgery of this is unclear. Depression, anxiety, and cognitive impairment are frequently present symptoms in patients with CS but not always part of their care plan. The role of antidepressants, mainly SSRIs, is debatable, but recent retrospective studies show evidence for safe use in patients with CS. Carcinoid crisis is a life-threatening complication of CS which can appear spontaneously but mostly described during surgery, anaesthesia, chemotherapy, PRRT, and radiological procedures and may be prevented by octreotide administration.

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

Cited by 24 publications

References 55 publications

Carcinoid heart disease revealed by cyanosis with both right and left valvular involvement: a case report

Carcinoid heart disease revealed by cyanosis with both right and left valvular involvement: a case report

Right Ventricular Outflow Obstruction Due to Metastatic Neuroendocrine Tumor

Update on Pathophysiology, Treatment, and Complications of Carcinoid Syndrome

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