An adult case with an abnormal right ventricular structure causing intraventricular pressure gradient and a history of aphthous stomatitis and thrombophlebitis

We report a 50-year-old man with a right ventricular structure causing an i ntraventricular pressure gradient. He had been diagnosed as vasculo-Behcet with a history of aphthous stomatitis and thrombophlebitis. He had also bee n suffering from atrial flutter and mild right-side heart failure. Echocard iography showed that there was an abnormal structure attached to the right ventricular free wall and protruding into the cavity, and that it caused th e pressure gradient estimated to be approximately 19 mmHg. Chest X-ray comp uted tomography demonstrated that the structure was partially calcified. Ma gnetic resonance imaging depicted the structure separating the right ventri cle into two chambers. Angiographic study revealed a markedly enlarged righ t atrium and a filling defect at the mid-portion of the right ventricle, wh ich divided the right ventricular cavity into two parts. Hemodynamic study showed a slightly elevated right atrial pressure (mean 7 mmHg) and a peak-t o-peak intraventricular pressure difference of 18 mmHg in the right ventric le. The diastolic pressure tracing of the right ventricular low pressure ch amber showed a 'dip and plateau' pattern. Although the pathological feature s of the abnormal right ventricular structure in this case were not fully c larified, abnormal muscle bundle and/or endocardial fibrosis, which were re ported to be associated with Behcet's disease, may have contributed to its generation.