Carcinoid Heart Disease: A Classic One, But Not Always Remembered

A 24-year-old female presented a dermatosis characterized by confluent erythematous spots on her face and neck and was diagnosed with presumed systemic lupus erythematosus. Five years later, she was admitted to our Cardiology Department due to symptoms of right heart failure (fatigue, dyspnea, edema in inferior limbs, and ascites). Thickened, stiff tricuspid and pulmonary valve leaflets that caused severe tricuspid and pulmonary regurgitation were seen on transthoracic echocardiography. Cardiac magnetic resonance showed right heart dilatation and right ventricular dysfunction. Multimodal imaging with contrast-enhanced computed tomography and dual positron emission tomography (PET/CT) with (¹⁸F) fluorodeoxyglucose and (¹⁸F) AlF-NOTA-octreotide showed liver lesions highly suggestive of metastasis. The liver biopsy confirmed a well-differentiated neuroendocrine tumor. A diagnosis of carcinoid heart disease (CHD) was made. She was treated with lanreotide (a long-acting somatostatin analog) and surgical replacement of the tricuspid and pulmonary valves.