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KEY FEATURES

ESSENTIALS OF DIAGNOSIS

  • CT or other imaging of neuroendocrine tumor(s) in the gut, with possible liver or pulmonary metastases

  • Elevated 5-hydroxyindoleacetic acid (5-HIAA) in the urine or plasma

  • Cardiac examination evidence of tricuspid regurgitation or pulmonic stenosis

  • Echocardiographic evidence of thickened tricuspid or pulmonary valves

GENERAL CONSIDERATIONS

  • Carcinoid tumors are neuroendocrine in origin and often secrete serotonin or other vasoactive amines

  • About 15% of carcinoid patients develop symptoms of flushing and diarrhea due to hepatic metastases (carcinoid syndrome)

  • About 50% of carcinoid syndrome patients develop valvular heart disease, mainly of the right heart valves

  • The endothelial lesion consists of distinctive fibrotic plaques that adhere to the valves and chamber walls

  • Valvular plaques usually result in tricuspid regurgitation and pulmonic stenosis

  • Bronchial tumors can result in left heart valve disease

CLINICAL PRESENTATION

SYMPTOMS AND SIGNS

  • Symptoms range from flushing to diarrhea to profound hypotension

  • Symptoms of right heart failure may be present: ascites and edema

PHYSICAL EXAM FINDINGS

  • Jugular venous elevation with a large v wave may be present

  • The murmur of tricuspid regurgitation or pulmonic stenosis may be present

  • Signs of right heart failure may be present: ascites, edema

DIFFERENTIAL DIAGNOSIS

  • Other causes of pulmonic stenosis, tricuspid regurgitation, and right heart failure

  • Consumption of serotonin-rich foods can give false-positive 5-HIAA screening test results

DIAGNOSTIC EVALUATION

LABORATORY TESTS

  • Twenty-four-hour urine or plasma for elevated 5-HIAA levels

  • High N-terminal pro-B-type natriuretic peptide levels are of diagnostic and prognostic value

ELECTROCARDIOGRAPHY

  • Some patients demonstrate right ventricular hypertrophy or right atrial enlargement

IMAGING STUDIES

  • Abdominal CT is indicated to locate the primary tumor and hepatic or other metastases

  • Echocardiography shows the characteristic diffuse thickening of the right heart valves with an echogenic material

    • – Doppler echo shows tricuspid regurgitation and pulmonic stenosis

  • Right ventricular volume and systolic function are important for prognosis

  • Cardiac CT is useful adjunct for diagnosing cardiac sarcoid and assessing coronary disease

  • Cardiac MRI is also useful, especially in patients with poor echo windows

  • Late gadolinium enhancement of the endocardium and valves is characteristic

DIAGNOSTIC PROCEDURES

  • Cardiac catheterization may be required in selected patients

TREATMENT

CARDIOLOGY REFERRAL

  • Evidence of significant valvular disease

  • Right heart failure

  • Endocarditis

HOSPITALIZATION CRITERIA

  • Right heart failure

  • Endocarditis

  • Profound hypotension

MEDICATIONS

  • Somatostatin analog therapy to control symptoms:

    • – Octreotide 50–150 μg 2–4 times a day SC or IV; maximum dose 1500 μg/day

    • – Octreotide 50–500 μg IV prn carcinoid crisis or 250–500 μg IV preoperatively ...

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