Carcinoid Heart Disease
A 57-year-old man presented with peripheral edema, fatigue, and dyspnea on exertion. He also had diarrhea and occasional flushing for 4 months. On physical examination he had elevated neck veins at 9 cm with a dominant v-wave, sternal lift with 2/6 systolic ejection murmur at the left upper sternal border and holosystolic murmur at the left lower sternal border, and a moderate lower extremity edema. His echocardiogram is shown (Figure 1). In a 24-hour urine collection, 5-hydroxyindolacetic acid was measured as 182 mg (normal, 2 to 8 mg). A computed tomography scan of the abdomen (Figure 2) showed multiple liver lesions consistent with metastatic carcinoid. Treatment with octreotide, a somatostatin analogue, was initiated. Both the tricuspid and pulmonary valves (Figure 3 and Figure 4) were replaced with bioprosthetic valves. The pathology of his native valves showed fibrous intimal thickenings on the inside surfaces of the valvular leaflets consistent with carcinoid heart disease (Figure 5 and Figure 6). The patient recovered well from surgery, and his symptoms of heart failure improved.
The online-only Data Supplement, which contains Movie I through Movie III, is available at http://cir.ahajournals.org/cgi/content/full/e160/DC1.