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- W2040357175 abstract "A 59-yr-old-woman with an 8-yr history of carcinoid syndrome with hepatic metastasis presented with progressive shortness of breath. Transesophageal echocardiographic examination (TEE) revealed severe tricuspid regurgitation, tricuspid stenosis, mild mitral stenosis, moderate mitral regurgitation, and a patent foramen ovale (PFO). She was referred for a tricuspid and mitral valve replacement. Intraoperative TEE confirmed the preoperative diagnosis of mild tricuspid stenosis with leaflet and chordal thickening, severe tricuspid regurgitation by extent of color flow jet area and hepatic vein flow reversal by pulse wave Doppler, severe right atrial and ventricular enlargement Video Clip 1; please see video clips available at www.anesthesia-analgesia.org), mild mitral stenosis, and moderate mitral regurgitation with mitral leaflet thickening and posterior leaflet restriction (Figs. 1 and 2; Video Clip 1). Agitated saline contrast injection confirmed a PFO. Mild pulmonary regurgitation was also noted but the aortic valve was uninvolved. Both the mitral and tricuspid valves were replaced with pericardial bioprosthetic valves, and the PFO was repaired (Video Clip 2). The excised tricuspid valve was diffusely thickened and fibrotic and the mitral valve leaflets appeared white and smooth, consistent with carcinoid valvular heart disease. The patient’s hospital course was complicated by recurrent carcinoid crises occurring within the first postoperative day. Her subsequent hospital course was uneventful and she was discharged on postoperative day 10.Figure 1.: Mid-esophageal transesophageal echocardiographic four-chamber view shows the tricuspid carcinoid valve. Figure 1A shows the systolic frame and Figure 1B the diastolic frame. Note the thickened, rigid carcinoid tricuspid valve leaflets that appear to be in a semi-open, “funnel-shaped” position. A comparison between the systolic and diastolic frames emphasizes the immobility of the leaflets: they appear to be the same in systolic and diastolic frames.Figure 2.: Mid-esophageal transesophageal echocardiographic four-chamber view shows thickened mitral valve leaflets. ME = midesophageal; TEE = transesophageal.Carcinoid tumors arise from the gastrointestinal tract. Malignant carcinoid syndrome occurs when serotonin and other vasoactive substances are released from carcinoid tumor cells and result in one or more of a constellation of symptoms which include flushing, hypotension, diarrhea and bronchospasm. More than half of patients with carcinoid syndrome develop carcinoid heart disease as a late complication and it is associated with progressive disease.1 Although the exact mechanism of carcinoid heart disease is unknown, the role of serotonin is supported by the observation that serotonin-like drugs, such as ergotamine and methysergide, or drugs that promote increased serotonin release, such as the appetite suppressant, fenfluramine, cause valvulopathy resembling carcinoid heart disease.1 The most common manifestation of carcinoid heart disease is valvular heart disease. It typically affects the right heart and generally occurs in patients with hepatic metastases where large amounts of serotonin and other vasoactive hormones can reach the heart directly before being inactivated by monoamine oxidases in the lungs.1 Therefore, echocardiographic evaluation of a patient with carcinoid heart disease typically reveals involvement of the tricuspid and pulmonic valves with sparing of the mitral and aortic valves. Left-sided involvement can be seen in the setting of intracardiac shunts, extensive primary bronchial carcinoid or when high overall tumor secretions or poorly controlled disease overwhelm the metabolic capacity of the lungs.1–3 It is usually seen in conjunction with right-sided involvement. As noted in this case, left-sided valve disease was characterized by valvular regurgitation rather than stenosis, even though valve leaflets appear thickened with mild reduction of excursion. Although there was no carcinoid involvement of the aortic valve, a case series by Connolly et al. notes that the aortic valve is affected as frequently as the mitral valve in patients with tricuspid carcinoid valve disease.4 Histologically, carcinoid heart disease is characterized by diffuse collections of thick, pearly white plaques composed of myofibroblasts that are deposited on the endocardium of valvular cusps, leaflets, and cardiac chambers.1 In the carcinoid tricuspid valve, regurgitation is more prominent than stenosis and septal and anterior leaflets are predominantly affected.1 Severity of tricuspid regurgitation can be assessed by evaluating color flow Doppler jet area, vena contracta width, proximal isovelocity surface area, and hepatic venous flow reversal with pulse wave Doppler. Continuous-wave Doppler spectral profile of severe tricuspid regurgitation typically appears dagger-shaped, with an early peak velocity and steep decline, indicating rapid equalization of right atrial and ventricular pressures, as opposed to a rounded envelope seen in patients with mild to moderate tricuspid regurgitation.1 Because the valve leaflets are rigid and fixed in the semi-open “funnel shaped” position, some degree of tricuspid stenosis is commonly present in conjunction with tricuspid regurgitation. Severity of stenosis can be evaluated by 2-dimensional imaging and quantified by calculating the mean pressure gradient across the valve. The carcinoid pulmonary valve commonly manifests as a combination of regurgitation and stenosis. However, pulmonic stenosis is seen more frequently than tricuspid stenosis because the pulmonic valve orifice area is much smaller than the tricuspid.1 A mean transpulmonic gradient of >10 mmHg is considered an indication for pulmonary valve surgery.1 As with the tricuspid valve, the carcinoid pulmonic valve is characterized by leaflet thickening and retraction of valve cusps. Unfortunately, with severely retracted pulmonary valve leaflets, visualization of the pulmonic valve with TEE is often difficult. Although 3-dimensional imaging is emerging as a diagnostic tool, visualization of the pulmonary valve with real-time 3-dimensional TEE imaging is limited.5 Although medical treatment may slow disease progression, surgical treatment may need to be considered.3 The optimal timing of cardiac surgery for carcinoid heart disease is debated. Many consider surgery when symptoms of right-sided failure develop. The decision regarding the type of valve to implant is difficult. Although mechanical valve replacement is associated with decreased prosthesis degeneration in carcinoid patients, many surgeons favor the use of bioprosthetic valves because the thrombotic risk is reduced and systemic anticoagulation is not required.1,3 Valve repair is usually not considered because of severe leaflet retraction and fixation. A recent case series of 150 patients with carcinoid syndrome noted 5 patients with left-sided valvular involvement, all of whom had concurrent PFOs.6 Our case emphasizes the need to thoroughly assess for intracardiac shunts in patients with carcinoid heart disease. Given the risk of future left-sided lesions, echocardiographic interrogation of the interatrial septum is mandatory and the presence of intracardiac shunts needs to be addressed during the primary surgical procedure in order to avoid leaving a passage for mediators, which can later develop into left-sided carcinoid disease." @default.
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- W2040357175 date "2008-12-01" @default.
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- W2040357175 title "Tricuspid and Mitral Valve Carcinoid Disease in the Setting of a Patent Foramen Ovale" @default.
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- W2040357175 doi "https://doi.org/10.1213/ane.0b013e318185cc63" @default.
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