An Adult Case with an Abnormal Right Ventricular Structure Causing Intraventricular Pressure Gradient and a History of Aphthous Stomatitis and Thrombophlebitis

Yasuyuki SUGISHITA; Shoko TAJIMA; Tatsuya SHIMIZU; Kazuro SUGISHITA; Kazumasa HARADA; Hiroshi MATSUI; Masashi INOUE; Yuji MURAKAWA; Jun-ichi SUZUKI; Katsu TAKENAKA; Masao OMATA; Toshiyuki TAKAHASHI

doi:10.1536/jhj.40.517

Yasuyuki SUGISHITA, Shoko TAJIMA, Tatsuya SHIMIZU, Kazuro SUGISHITA, Kazumasa HARADA, Hiroshi MATSUI, Masashi INOUE, Yuji MURAKAWA, Jun-ichi SUZUKI, Katsu TAKENAKA, Masao OMATA, Toshiyuki TAKAHASHI

Author information

Yasuyuki SUGISHITA
Department of Internal Medicine, Faculty of Medicine, University of Tokyo, Tokyo, Japan
Shoko TAJIMA
Department of Internal Medicine, Faculty of Medicine, University of Tokyo, Tokyo, Japan
Tatsuya SHIMIZU
Department of Internal Medicine, Faculty of Medicine, University of Tokyo, Tokyo, Japan
Kazuro SUGISHITA
Department of Internal Medicine, Faculty of Medicine, University of Tokyo, Tokyo, Japan
Kazumasa HARADA
Department of Internal Medicine, Faculty of Medicine, University of Tokyo, Tokyo, Japan
Hiroshi MATSUI
Department of Internal Medicine, Faculty of Medicine, University of Tokyo, Tokyo, Japan
Masashi INOUE
Department of Internal Medicine, Faculty of Medicine, University of Tokyo, Tokyo, Japan
Yuji MURAKAWA
Department of Internal Medicine, Faculty of Medicine, University of Tokyo, Tokyo, Japan
Jun-ichi SUZUKI
Department of Internal Medicine, Faculty of Medicine, University of Tokyo, Tokyo, Japan
Katsu TAKENAKA
Department of Internal Medicine, Faculty of Medicine, University of Tokyo, Tokyo, Japan
Masao OMATA
Department of Internal Medicine, Faculty of Medicine, University of Tokyo, Tokyo, Japan
Toshiyuki TAKAHASHI
Department of Internal Medicine, Faculty of Medicine, University of Tokyo, Tokyo, Japan

Keywords: Double-chambered right ventricle, Endocardial fibrosis, Atrial flutter, Vasculo-Behçet, Right-side heart failure

JOURNAL FREE ACCESS

1999 Volume 40 Issue 4 Pages 517-525

DOI https://doi.org/10.1536/jhj.40.517

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Abstract

We report a 50-year-old man with a right ventricular structure causing an intraventricular pressure gradient. He had been diagnosed as vasculo-Behçet with a history of aphthous stomatitis and thrombophlebitis. He had also been suffering from atrial flutter and mild right-side heart failure. Echocardiography showed that there was an abnormal structure attached to the right ventricular free wall and protruding into the cavity, and that it caused the pressure gradient estimated to be approximately 19 mmHg. Chest X-ray computed tomography demonstrated that the structure was partially calcified. Magnetic resonance imaging depicted the structure separating the right ventricle into two chambers. Angiographic study revealed a markedly enlarged right atrium and a filling defect at the mid-portion of the right ventricle, which divided the right ventricular cavity into two parts. Hemodynamic study showed a slightly elevated right atrial pressure (mean 7 mmHg) and a peak-to-peak intraventricular pressure difference of 18 mmHg in the right ventricle. The diastolic pressure tracing of the right ventricular low pressure chamber showed a ‹dip and plateau' pattern. Although the pathological features of the abnormal right ventricular structure in this case were not fully clarified, abnormal muscle bundle and/or endocardial fibrosis, which were reported to be associated with Behçet's disease, may have contributed to its generation.

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