959 AN UNEXPECTED DIAGNOSIS BY ECHOCARDIOGRAPHY: APPLICATION IN CLINICAL PRACTICE

Abstract A carcinoid tumore is a rare neuroendocrinologic malignancy that commonly origins from enterochromaffin or Kulchitsky cells in the gastrointestinal tract and secretes bioactive molecules in blood. Up to 60% of patients with carcinoid tumors have cardiac involvement, often on the right-sided heart valves (typically the tricuspid valve and the pulmonic valve). A 66 year old woman was admitted to hospital with congestive heart failure, chronic diarrhea and weight loss. A contrast-enhanced computed tomography scan of the liver showed multiple masses (the largest 70 mm×45mm) compatible with metastases. A transthoracic echocardiogram showed dilatation of right sections of the heart with severe tricuspid regurgitation (RV 64 ml/beat, ERO 1 cmq) and mild pulmonary valve stenosis (Peak gradient 13 mmHg). Patient's aortic and mitral valves were normal in structure and function. The EKG revealed low-voltage QRS complexes of all leads, unspecific ST segment changes and sinusal rhythm. The blood tests showed a significant increase in chromogranin A (17670 ng/ml) and enolase; a 24-hour urine collection showed a total output of 5-hydroxyindoleacetic acid eleveted. A biopsy of the liver with immunostaining for chromogranin and synaptophysin were strongly positive and confirmed the diagnosis of metastatic carcinoid syndrome (grade 1, cells CDX2 positive). The echocardiographic findings of carcinoid heart disease is drumstick-like, rigid, and shortened leaflets with a visible regurgitant orifice, causing torrential tricuspid regurgitation. The pulmonic valve presents more frequently valvular stenosis. A combination of biomarkers and cardiac imaging is useful in screening and diagnosis of carcinoid heart involvement in patients with carcinoid syndrome. The management of carcinoid heart disease is hardful and requires a multidisciplinary approach, medical and surgical treatment, for optimized care.