When the Owl Blinks
A 47-year-old man presented to our hospital with dyspnea on exertion and lower extremity edema of 1 year's duration. Transthoracic echocardiography showed mild left ventricular systolic dysfunction; a D-shaped left ventricle during diastole; severe right ventricular enlargement with moderate systolic dysfunction; thickened, retracted, and malcoapted tricuspid valve leaflets resulting in severe tricuspid regurgitation; thickened and retracted pulmonary valve leaflets with mild-to-moderate pulmonary stenosis and moderate pulmonary insufficiency; a thickened mitral valve with mild mitral regurgitation; a thickened aortic valve with mild aortic insufficiency; and small size patent foramen ovale. Data acquired via transthoracic two-dimensional and real-time three-dimensional echocardiography, magnetic resonance imaging, urine analysis, and immunohistochemistry confirmed the carcinoid syndrome.
Carcinoid tumors are uncommon, slow-growing, neuroendocrine neoplasms arising from enterochromaffin cells, propagated throughout the bronchopulmonary and gastrointestinal systems.1 The release of serotonin and other vasoactive substances into the systemic circulation, in the case of liver metastasis, is deemed to cause the carcinoid syndrome, the manifestations of which are episodic flushing, diarrhea, bronchoconstriction, and disease of right-sided heart valves.2 Nearly 40% of patients exhibiting the carcinoid syndrome will develop carcinoid heart disease with fibrotic endocardial plaques and associated heart valve dysfunction, which classically involves the right-sided valves, and left-side valves are affected through an intracardiac shunt such as patent foramen ovale or atrial septal defect, as in our case.3
- Pinchot SN, Holen K, Sippel RS, Chen H. Carcinoid Tumors. Oncologist 2008;13:1255-69.
- Ha J, Tan WA. Gastrointestinal Carcinoid Tumours: A Review. J Gastrointest Dig Syst 2012;2:107.
- Gustafsson BI, Hauso O, Drozdov I, Kidd M, Modlin IM. Carcinoid heart disease. Int J Cardiol 2008;129:318-24.
Date: August 25, 2020
Clinical Topics: Noninvasive Imaging
Keywords: Cardiac Imaging Techniques