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Coexistence of Marfan-like Connective Tissue Disease with Morphologic Left Ventricular Non-compaction |
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| Authors: | Satomi Yashima Hiroyuki Takaoka Togo Iwahana Manami Takahashi Yusuke Kondo Hideki Ueda Aya Saito Yuya Ito Noboru Motomura Nobuyuki Hiruta Jun-ichiro Ikeda Goro Matsumiya Yoshio Kobayashi |
| Affiliation: | 1.Department of Cardiovascular Medicine, Chiba University Graduate School of Medicine, Japan; 2.Department of Cardiovascular Surgery, Chiba University Graduate School of Medicine, Japan; 3.Department of Cardiovascular Surgery, Toho University Sakura Medical Center, Japan; 4.Department of Surgical Pathology, Toho University Sakura Medical Center, Japan; 5.Department of Diagnostic Pathology, Chiba University Graduate School of Medicine, Japan |
| Abstract: | We treated a man with co-incident Marfan-like connective tissue disease with morphologic left ventricular non-compaction (LVNC). He underwent valve-sparing aortic root replacement because of aortic root dilation at 43 years old. Pathological findings of the aorta revealed cystic medio-necrosis, consistent with Marfan syndrome. He developed congestive heart failure caused by LVNC at 47 years old. His daughter had scoliosis, and he had several physical characteristics suggestive of Marfan syndrome. We herein report a rare case of a patient who had Marfan-like connective disease with an LVNC appearance. |
| Keywords: | connective tissue disease left ventricular non-compaction Marfan syndrome |