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

Edna Juarez MD, Avinash Adiga MD, Paloma Sanchez MD, Hilal Fanasch MD


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 involvement.

Keywords: carcinoid heart disease, carcinoid tumor, carcinoid syndrome

Article citation: Juarez E, Adiga A, Sanchez P, Fanasch H. Metastatic carcinoid tumor with severe double valve disease at diagnosis: case report and review of literature. The Southwest Respiratory and Critical Care Chronicles 2017; 21:26–31
From: Department of Internal Medicine, Texas Tech University Health Sciences Center, Lubbock, TX
Submitted: 5/12/2017
Accepted: 9/30/2017
Reviewer: Kenneth Nugent MD
Conflicts of interest: none