Carcinoid Heart Disease: Starting From Heart Failure

Bertin, Nicole; Favretto, Serena; Pelizzo, Francesco; Mos, Lucio; Pertoldi, Franco; Vriz, Olga

doi:10.1177/2324709617713511

Cited by 10 publications

(16 citation statements)

References 40 publications

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“…Complications of mesenteric fibrosis may include intra-abdominal vessel ischemia and intestinal obstruction as well as ureteral obstruction and renal failure [1]. CS may cause fibrotic lesions of the endocardium, particularly on the right side of the heart, resulting in an insufficiency of the tricuspid valve and, less frequently, the pulmonary valve [12][13]. In many patients, the cause of death is attributed directly to cardiac disease.…”

Section: Pathophysiologymentioning

confidence: 99%

Carcinoid Syndrome: A Review

Gade¹,

Olariu²,

Douthit³

2020

Cureus

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Carcinoid syndrome (CS) is a paraneoplastic syndrome caused by the release of serotonin and other substances from well-differentiated neuroendocrine tumors (NETs). The hallmark symptoms of carcinoid syndrome are flushing and diarrhea; atypical signs and symptoms can include wheezing, abdominal pain, valvular heart disease, telangiectasias, pellagra, and the complications of mesenteric fibrosis, including ureteral obstruction, bowel obstruction, and bowel ischemia. These symptoms are mediated by the release of serotonin (5-HT), histamine, kallikrein, prostaglandins, and tachykinins. The diagnosis of CS requires these symptoms and corresponding elevations in lab tests. Treatment options include surgery and medical management with somatostatin analogs.

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Section: Pathophysiologymentioning

confidence: 99%

Carcinoid Syndrome: A Review

Gade¹,

Olariu²,

Douthit³

2020

Cureus

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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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“…The characteristic symptoms are diarrhoea, cutaneous flushing and bronchoconstriction. Carcinoid tumours are rare, with an estimated incidence of 1–2 per 100,000 in the United States of America ( 1 , 2 ).…”

Section: Discussionmentioning

confidence: 99%

An unusual cause of hypoxia: getting to the heart of the matter

Hammersley¹,

Shamsi²,

Zaman³

et al. 2018

Echo Res Pract

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A 63-year-old female presented to hospital with progressive exertional dyspnoea over a 6-month period. In the year preceding her admission, she reported an intercurrent history of abdominal pain, diarrhoea and weight loss. She was found to be hypoxic, the cause for which was initially unclear. A ventilation–perfusion scan identified a right-to-left shunt. Transoesophageal echocardiography (TOE) demonstrated a significant right-to-left intracardiac shunt through a patent foramen ovale (PFO); additionally severe tricuspid regurgitation was noted through a highly abnormal tricuspid valve. The findings were consistent with carcinoid heart disease with a haemodynamically significant shunt, resulting in profound systemic hypoxia. 24-h urinary 5-hydroxyindoleacetic acid (5-HIAA) and imaging were consistent with a terminal ileal primary carcinoid cancer with hepatic metastasis. Liver biopsy confirmed a tissue diagnosis. The patient was commenced on medical therapy for carcinoid syndrome. She subsequently passed away while undergoing anaesthetic induction for valvular surgery to treat her carcinoid heart disease and PFO.Learning points:Carcinoid syndrome is a rare condition, which presents a significant diagnostic challenge due to its insidious presentation and symptoms. This frequently results in a marked delay in diagnosis.Carcinoid heart disease is characterised by distortion and fixation of right-sided heart valves, which cause valvular regurgitation, stenosis or both. Valvular abnormalities are often found in association with right ventricular failure.In the case described, carcinoid heart disease was found in association with a significant right-to-left intracardiac shunt, created through a PFO due to right atrial volume overload. This prevented right ventricular failure at the expense of creating a state of severe induced systemic hypoxia.This physiological adaptation resulted in an unusual presentation of this condition, due to symptoms resulting from hypoxia, rather than the classical symptoms of carcinoid syndrome or right ventricular failure.

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Carcinoid Heart Disease: Starting From Heart Failure

Cited by 10 publications

References 40 publications

Carcinoid Syndrome: A Review

Carcinoid Syndrome: A Review

Right Ventricular Outflow Obstruction Due to Metastatic Neuroendocrine Tumor

An unusual cause of hypoxia: getting to the heart of the matter

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