Metastatic carcinoid tumor with severe double valve disease at diagnosis: Case report and review of literature

Abstract

A 27-year-old woman with a history of anxiety presented with three months of diarrhea. Onphysical examination, she had a grade 3/6 systolic heart murmur at the lower left sternal borderwhich increased with inspiration, a grade 2/6 decrescendo diastolic murmur at the left sternalborder, and lower extremity edema. Stool studies were negative. The abdominal ultrasounddemonstrated multiple complex liver cysts. Transthoracic echocardiogram reported tricuspidvalvular thickening with stenosis and regurgitation, severe pulmonary stenosis, and mild aorticregurgitation with valvular thickening. 5-hydroxyindole acetic acid and chromogranin A levelswere elevated. The patient’s symptoms improved after octreotide therapy, and she underwenttricuspid 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 syndromeand is associated with poorer clinical outcomes. Left side cardiac involvement occurs in lessthan 10% of patients. The presence of both left and right sided valvular disease in the contextof gastrointestinal carcinoid is associated with severe and poorly controlled disease. Up to4% of these cases have metastatic disease on the valves, and 3.8% have direct myocardialinvolvement.