A53-year-old Indian man developed watery diarrhea. He was treated for inflammatory bowel disease without improvement. Three months before admission, he developed lower extremity edema, which progressed to include massive scrotal edema as well as dyspnea on exertion, orthopnea, and paroxysmal nocturnal dyspnea. An ECG (Fig 1⇓) and chest radiograph (Fig 2⇓) were performed. An upper gastrointestinal series and small bowel series were unrevealing. An abdominal computed tomography scan demonstrated the presence of multiple liver masses (Fig 3⇓). A percutaneous fine-needle liver biopsy was performed and yielded the cells seen in Fig 4⇓. A 24-hour urine collection for 5-hydroxyindoleacetic acid was markedly elevated at 871 μmol/d (normal, <200 μmol/d). Echocardiographic findings are summarized in Figs 5⇓ and 6⇓.
Based on our findings (Figs 1 through 6⇓⇓⇓⇓⇓⇓), the suspected diagnosis of carcinoid syndrome with cardiac involvement was confirmed. Therapy with the long-acting somatostatin analogue octreotide resulted in marked improvement in the patient’s diarrhea. Heart failure improved but did not resolve with medical therapy. A recent report1 suggests that in the current era of improved therapy for metastatic carcinoid disease, progressive right heart failure has become a major cause of morbidity and mortality. Despite high perioperative mortality, valve surgery has been proposed as definitive therapy, resulting in a significant improvement in functional class and prolonged survival.1 Valve replacement surgery was therefore recommended.
We gratefully acknowledge the contribution of Louis M. Cohen, MD.
The editor of Images in Cardiovascular Medicine is Hugh A. McAllister, Jr, MD, Chief, Department of Pathology, St Luke’s Hospital and Texas Heart Institute, and Clinical Professor of Pathology, University of Texas Medical School and Baylor College of Medicine
Circulation encourages readers to submit cardiovascular images to Dr Hugh A. McAllister, Jr, St Luke’s Episcopal Hospital and Texas Heart Institute, 6720 Bertner, MC 4-265, Houston, TX 77030.
- Copyright © 1996 by American Heart Association
1. Connolly HM, Nishimura RA, Smith HC, Pellikka PA, Mullany CJ, Kvols LK. Outcome of cardiac surgery for carcinoid heart disease. J Am Coll Cardiol. 1995;2:410-416.