Pulmonary valve in carcinoid disease: be suspicious of functional assessment
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Pulmonary valve in carcinoid disease: be suspicious of functional assessment Alvaro Luis Gamarra1 · Alberto Cecconi1 · Antonio Rojas‑Gonzalez1 · Maria Jose Olivera2 · Emilio Monguio3 · Luis Jesus Jimenez‑Borreguero4 · Fernando Alfonso1 Received: 20 August 2020 / Accepted: 24 August 2020 © Springer Nature B.V. 2020
Neuroendocrine tumours (NET), previously known as carcinoid tumours, are a rare cause of neoplastic disease. NET are predominantly found in gastrointestinal tract and bronchi, only 0.5% are primarily ovarian [1]. Carcinoid syndrome is mainly associated to NET located in the gastrointestinal tract or liver, with direct access to the systemic bloodstream [2]. Still, the presence of carcinoid syndrome associated to ovarian NET has been well recorded in the past [1]. Carcinoid heart disease occurs in 50% of patients with carcinoid syndrome, especially on serotonin-secreting NET, although it can be the first manifestation of the carcinoid syndrome in up to a 20% of the patients [2]. Carcinoid heart disease’s typical findings are carcinoid plaques, composed of smooth muscle, myofibroblasts and endothelium. These plaques, most frequently localized on the right side valvular cusps and leaflets, produce fixation and retraction, causing valvular regurgitation and/or stenosis. Carcinoid plaques may also develop in the intima of great vessels, whereas leftsided heart disease is rare (
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