Metastatic carcinoid tumor with severe double valve disease at diagnosis: Case report and review of literature
A 27-year-old woman with a history of anxiety presented with three months of diarrhea. On
physical examination, she had a grade 3/6 systolic heart murmur at the lower left sternal border
which increased with inspiration, a grade 2/6 decrescendo diastolic murmur at the left sternal
border, and lower extremity edema. Stool studies were negative. The abdominal ultrasound
demonstrated multiple complex liver cysts. Transthoracic echocardiogram reported tricuspid
valvular thickening with stenosis and regurgitation, severe pulmonary stenosis, and mild aortic
regurgitation with valvular thickening. 5-hydroxyindole acetic acid and chromogranin A levels
were elevated. The patient’s symptoms improved after octreotide therapy, and she underwent
tricuspid and pulmonary valve replacement.
The incidence of carcinoid tumors is 1.2 to 2.1 per 100,000 people in the general population.
Carcinoid heart disease occurs in one-half to two-thirds of patients with carcinoid syndrome
and is associated with poorer clinical outcomes. Left side cardiac involvement occurs in less
than 10% of patients. The presence of both left and right sided valvular disease in the context
of gastrointestinal carcinoid is associated with severe and poorly controlled disease. Up to
4% of these cases have metastatic disease on the valves, and 3.8% have direct myocardial
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