Right ventricular endomyocardial fibrosis mimicking Ebstein anomaly in a patient with Behçet’s disease: case report and review of the literature

Abstract

Behçet’s disease is a multisystemic, chronic inflammatory disorder with diffuse clinical manifestations including the cardiovascular system. Endomyocardial fibrosis is a rarely seen complication of Behçet’s disease leading to progressive heart failure. We report a case of right ventricular endomyocardial fibrosis mimicking Ebstein anomaly in a 26-year-old male Turkish patient with Behçet’s disease, who had heart failure symptoms. In addition, the previously reported cases of endomyocardial fibrosis complicating Behçet’s disease are reviewed in this article.

Keywords:

• Behçet’s disease
• Endomyocardial fibrosis

Acknowledgments

We would like to thank Prof. Dr. Handan Zeren from Acıbadem University pathology department for her great help in examination of the histopathological specimens and Dr. Selçuk Görmez for his great job in perioperative transesophageal echocardiography.

Conflict of interest

None.