A 48-year-old woman presents with episodic flushing, diarrhoea, and wheezing. Urine 5-HIAA is markedly elevated at 78 mg/24h (normal <8 mg). CT abdomen shows a 2.3 cm ileal mass with liver metastases. Echocardiography shows tricuspid regurgitation and pulmonary stenosis. What complication of carcinoid syndrome is demonstrated by the echocardiographic findings?
- A Rheumatic heart disease
- B Marantic endocarditis from malignancy
- C Infective endocarditis with multivalvular involvement
- D Carcinoid heart disease (Hedinger syndrome) ✓
Explanation
Carcinoid heart disease (Hedinger syndrome) is a complication of carcinoid syndrome due to hepatic metastases, where serotonin and other vasoactive peptides bypass hepatic metabolism and reach the right heart, causing fibrous plaque deposition on the endocardium. This results in right-sided valvular lesions — tricuspid regurgitation and pulmonary stenosis — which are characteristic. Left-sided lesions occur rarely (only with bronchial carcinoids or patent foramen ovale). Elevated 5-HIAA confirms excessive serotonin production. Octreotide scintigraphy and PRRT are therapeutic options.
Reference: Harrison's Principles of Internal Medicine, 21st ed.
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Written and medically reviewed by the StethoPrep medical team.