Valvular Disorders in Carcinoid Heart Disease

Yuan, Shi‐Min

doi:10.5935/1678-9741.20160079

Cited by 17 publications

(23 citation statements)

References 29 publications

(61 reference statements)

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“…10 Up to 80% of patients have had PV regurgitation or stenosis. 10,13,26 These structural and valvular lesions eventually lead to right-sided heart failure with symptoms such as lower-extremity edema and early satiety. 12,26 New York Heart Association functional class III/IV symptoms and right ventricular (RV) dysfunction were important predictors of outcome in these patients.…”

Section: Manifestationsmentioning

confidence: 99%

Carcinoid Heart Disease: Review of Current Knowledge

Ram¹,

Peñalver²,

Lo³

et al. 2019

Texas Heart Institute Journal

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Carcinoid heart disease is the collective term for all cardiac manifestations in patients who have carcinoid syndrome. Carcinoid heart disease has a multifactorial pathophysiology, and the right side of the heart is usually involved. Symptoms and signs vary depending upon the affected cardiac components; most typical is right-sided heart failure secondary to diseased tricuspid and pulmonary valves. Despite no single ideal diagnostic test, strong suspicion, coupled with serologic and imaging results, usually enables diagnosis. Advances in imaging, such as speckle-tracking echocardiography and cardiac magnetic resonance, have improved the diagnostic yield. Treatment is challenging, warrants a multidisciplinary approach, and can be medical or surgical depending on the cardiac manifestations. Investigators are exploring the therapeutic use of monoclonal antibodies and new somatostatin analogues. In this review, we cover current knowledge about the pathophysiology, diagnosis, and treatment of carcinoid heart disease.

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Section: Manifestationsmentioning

confidence: 99%

Carcinoid Heart Disease: Review of Current Knowledge

Ram¹,

Peñalver²,

Lo³

et al. 2019

Texas Heart Institute Journal

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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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“… 1 Carcinoid heart disease, also known as Hedinger syndrome is present in approximately 20% of patients with carcinoid syndrome. 7 Patients with carcinoid heart disease present with early symptoms of right-sided valvular heart disease, including fatigue and dyspnoea on exertion, secondary to tricuspid regurgitation and pulmonic valve stenosis. As the disease progresses, right-sided heart failure occurs in a majority of patients causing worsening dyspnoea, oedema, and ascites owing to severe dysfunction of the tricuspid and pulmonary valves.…”

Section: Discussionmentioning

confidence: 99%

Flow-driven right-to-left cardiac shunting in a patient with carcinoid heart disease and patent foramen ovale without elevated right atrial pressure: a case report and literature review

Dherange

Telles

Modi

2020

European Heart Journal - Case Reports

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Background Carcinoid heart disease is present in approximately 20% of the patients with carcinoid syndrome and is associated with poor prognosis. It usually manifests with right-sided valvular involvement including tricuspid insufficiency and pulmonary stenosis. Patent foramen ovale (PFO) is present in approximately 50% of the patients with carcinoid heart disease which is twice higher than the general population. Right-to-left shunting through a PFO can occur either due to higher right atrial pressure than left (pressure-driven) or when the venous flow is directed towards the PFO (flow-driven) in the setting of normal intracardiac pressures. We report a rare case of flow-driven right-to-left atrial shunting via PFO in a patient with carcinoid heart disease. Case summary A 54-year-old male with a metastatic neuroendocrine tumour to liver presented with progressive shortness of breath for 5 months. Patient was found to be hypoxic with oxygen saturation of 78% and examination revealed a holosystolic murmur. Arterial blood gas showed oxygen tension of 43 mmHg. A transthoracic and transoesophageal echocardiogram showed aneurysmal inter-atrial septum with a PFO, severe tricuspid regurgitation directed anteriorly towards the inter-atrial septum leading to a marked right-to-left shunt. Right heart catheterization showed right atrial pressure of 8 mmHg, mean pulmonary artery pressure of 12 mmHg, and normal oxygen saturations in the right atrium, right ventricle, and pulmonary arteries. The patient then underwent closure of the PFO along with tricuspid valve and pulmonary valve replacement at an experienced cardiovascular surgical centre and has been asymptomatic since. Conclusion Right-to-left shunting through a PFO in patients with normal right atrial pressure can be successfully treated with closure of the PFO. Thus, understanding the mechanism of intracardiac shunts is important to accurately diagnose and treat this rare and fatal condition.

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Valvular Disorders in Carcinoid Heart Disease

Cited by 17 publications

References 29 publications

Carcinoid Heart Disease: Review of Current Knowledge

Carcinoid Heart Disease: Review of Current Knowledge

Carcinoid Syndrome: A Review

Flow-driven right-to-left cardiac shunting in a patient with carcinoid heart disease and patent foramen ovale without elevated right atrial pressure: a case report and literature review

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