J Cardiovasc Ultrasound.  2011 Mar;19(1):45-49. 10.4250/jcu.2011.19.1.45.

Ovarian Tumor-Associated Carcinoid Heart Disease Presenting as Severe Tricuspid Regurgitation

• Shin HW
• Kim H
• Yoon HJ
• Park HS
• Cho YK
• Nam CW
• Hur SH
• Kim YN
• Kim KB

• KMID: 2135434
• DOI: http://doi.org/10.4250/jcu.2011.19.1.45

Abstract

Carcinoid heart disease is characterized by heart valve dysfunction as well as carcinoid symptomatology. We report a case of carcinoid heart disease associated with a primary ovarian tumor. A 60-year-old woman presented for dyspnea evaluation with a history of facial flushing, telangiectatic skin changes, and pitting edema of both lower extremities. Chest radiography showed cardiomegaly, and echocardiography revealed an isolated, severe tricuspid regurgitation without left-sided valvular dysfunction. The tricuspid leaflets were severely retracted and shortened, resulting in poor coaptation. Furthermore, mild pulmonary valve stenosis and moderate regurgitation were found along with this deformation. The 24-hour urine analysis revealed an increased level of 5-hydroxyindoleacetic acid, and an ovarian tumor was apparent on computed tomography images. The mass was surgically removed, and the patient was diagnosed as having a primary ovarian carcinoid tumor. She was treated with chemotherapy and regularly followed-up with supportive treatments, deferring surgical correction.

Keyword

Carcinoid; Tricuspid regurgitation; Echocardiography; Ovarian tumor

MeSH Terms

Carcinoid Heart Disease
Carcinoid Tumor
Cardiomegaly
Dyspnea
Echocardiography
Edema
Female
Flushing
Heart Valves
Humans
Lower Extremity
Middle Aged
Pulmonary Valve Stenosis
Skin
Thorax
Tricuspid Valve Insufficiency

Figure

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  Fig. 1 Echocardiographic imagings showing a deformed tricuspid valve with regurgitation. The echocardiogram on apical four-chamber view shows an enlarged right atrium and right ventricle. Both anterior and septal leaflets (arrows) reveal thickened and retracted (A). On the right ventricular inflow view, both anterior and posterior leaflets also show no mobility, shortening, and coaptation failure (B). Severe tricuspid regurgitation is noted on color Doppler imaging (C). Continuous-wave Doppler signal of tricuspid regurgitation shows an early peak velocity and a rapid decline, shaping dagger-shaped "V" wave (velocity, 2.7 m/s) (D). RV: right ventricle, RA: right atrium, LA: left atrium, TV: tricuspid valve, TR: tricuspid regurgitation.

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  Fig. 2 Echocardiographic imagings showing a deformed pulmonary valve with regurgitation and stenosis. Parasternal short axis view of the pulmonary valve which has thickened and shortened leaflets (arrows); in addition, pulmonary annular contraction is present in zoomed imaging (A and D). On color Doppler imaging, moderate pulmonary regurgitation is shown and its peak velocity is at 1.5 m/s (B and C). Simultaneously, color flow is accelerated in systolic phase due to the stiffened and constricted leaflets (peak velocity, 1.8 m/s) (E and F). RV: right ventricle, Ao: aorta, PV: pulmonary valve, PR: pulmonary regurgitation, PS: pulmonary stenosis.

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  Fig. 3 Computed tomographic findings of carcinoid tumor. A large mass with inhomogenous density occupies the right pelvic cavity. The mass (white arrow) consists of multiple thick-enhancing septa and enhancing central soft tissue density (A). The right ovary is grossly enlarged; it is measured to be 80 × 124 × 78 mm in size and to weigh 461 g. The ovarian surface is smooth. The cut surface of the mass is bright yellow and solid, and it shows several foci of hemorrhage (B).

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  Fig. 4 Microscopic appearance of ovarian carcinoid tumor. Photomicrograph showing solid nests and broad trabeculae of tumor by cellular proliferation. These patterns were lined with one to two layers of cells (hematoxylin-eosin, 100×) (A). The regular round tumor cells have uniform rounded nuclei and abundant fibrovascular stroma (hematoxylin-eosin, 400×) (B). Immnuohistochemistry stains for neuroendocrine marker of the tumor cells reveals diffuse, strong, granular, and cytoplasmic staining (brown color) with chromogranin A (chromogranin, 100×) (C).

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