Carcinoid heart disease
- Heidi M Connolly, MD, FASE
Heidi M Connolly, MD, FASE
- Section Editor — Congenital Heart Disease
- Professor of Medicine
- Mayo Medical School
Carcinoid tumors are rare, arising in 1.2 to 2.1 per 100,000 people in the general population per year . They can arise anywhere in the body, but are most commonly found in the gastrointestinal tract (midgut carcinoids) and bronchus (foregut carcinoids) (table 1). Primary midgut carcinoid tumors metastasize to the liver or regional lymph nodes and may present with bowel obstruction. In 20 to 30 percent of patients, the initial presentation occurs as a result of hormone production, called the carcinoid syndrome. (See "Clinical characteristics of carcinoid tumors".)
The most common manifestations of the carcinoid syndrome are vasomotor changes (flushing) (picture 1), gastrointestinal hypermotility (secretory diarrhea), bronchospasm, and hypotension (table 2). These symptoms are caused by the release of vasoactive substances, including serotonin (5-hydroxytryptamine), 5-hydroxytryptophan, histamine, bradykinin, tachykinins, and prostaglandins (table 3). The diagnosis of carcinoid syndrome is usually suspected by the clinical features and confirmed by identification of the primary tumor, localization of metastatic lesions, and detection of increased urinary excretion of the by-product of serotonin metabolism, 5-hydroxyindoleacetic acid (5-HIAA) (figure 1). (See "Clinical features of the carcinoid syndrome" and "Diagnosis of the carcinoid syndrome and tumor localization".)
Progress in the medical and surgical management of patients with carcinoid disease has resulted in improved symptoms and survival. (See "Staging, treatment, and posttreatment surveillance of non-metastatic well-differentiated neuroendocrine (carcinoid) tumors".)
However, carcinoid heart disease, which eventually occurs in over 50 percent of patients with carcinoid syndrome and may be the initial presentation of carcinoid disease in as many as 20 percent of patients [2,3], remains a major cause of morbidity and mortality among patients with carcinoid syndrome [4,5].
Pathologic findings — Carcinoid heart disease is characterized by pathognomonic plaque-like deposits of fibrous tissue. These deposits occur most commonly on the endocardium of valvular cusps, leaflets, papillary muscles and cords, cardiac chambers, and occasionally on the intima of the pulmonary arteries or aorta (figure 2) [6,7].
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- Pathologic findings
- Role of serotonin
- CLINICAL MANIFESTATIONS
- Physical examination
- Initial test findings
- - Electrocardiogram
- - Chest radiograph
- DIAGNOSIS AND EVALUATION
- When and how to initially test for carcinoid heart disease
- - Patients with carcinoid syndrome
- Role of biomarkers
- - Patients with tricuspid and/or pulmonic valve disease
- Approach to diagnosis and evaluation
- - Echocardiography
- - CMR and CT
- Approach to management
- Diuretic therapy
- Cardiac surgery
- - Choice of valve prosthesis
- - Anesthesia management
- Treatment of carcinoid syndrome
- SUMMARY AND RECOMMENDATIONS