A 61-year-old man with metastatic cancer was initially seen with a murmur that became increasingly louder. On examination, there was a right ventricular heave and a grade 2/6 holosystolic murmur along the left sternal edge at the fifth intercostal space that increased on inspiration. The liver was enlarged and pulsatile, and pedal edema was present.
What is your diagnosis?
Carcinoid syndrome is caused by the biologically active substances produced by metastatic carcinoid tumor tissue. The syndrome includes flushing of the skin, diarrhea, bronchospasm, and thickening of the endocardial structures of the right side of the heart. This latter feature is responsible for the abnormalities seen by echocardiography. The Figure shows thickened tricuspid leaflets that are retracted and do not meet properly during systolic closure.
Most primary carcinoid tumors are located in the intestinal tract, most frequently in the small intestine. The vast majority are found in the appendix, where they are usually