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Indexed/Abstracted in: EMBASE, Scopus, Emerging Sources Citation Index
Online ISSN 1827-1782
Hattori R. 1, Uemura Y. 2, Okada T. 1, Johno H. 1, Enoki C. 1, Sumida T. 1, Nakao Y. 1, Imamura H. 1
1 Department of Thoracic and Cardiovascular Surgery Kansai Medical University, Hirakata, Osaka, Japan
2 Department of Pathology Kansai Medical University, Hirakata, Osaka, Japan
Pseudoxanthoma elasticum (PXE) is a genetic disorder in which the skin, eyes, and cardiovascular elastic fibers become systematically damaged. It is known that mitral regurgitation also occurs as part of this disorder, but there have so far been very few reports of cases that required surgical treatment. We encountered a patient with PXE who had undergone mitral valve replacement using a bioprosthetic valve and subsequently developed severe prosthetic valve failure within only 3 years. A 69-year-old female was taken to our hospital due to a severe respiratory disorder and she was immediately hospitalized in the CCU. Echocardiography showed prosthetic valve failure with a severe restriction of mobility. Rapid destructive heart failure thereafter progressed and cardiopulmonary resuscitation was needed for the occurrence of sudden cardiac arrest. An emergency re-valve replacement was not able to be performed due to the presence of severe brain damage and the patient died on Day 6 of hospitalization. In a histological specimen of the bioprosthetic valve which was obtained at autopsy, a significant degeneration of the elastic fibers and severe calcification of the tissue valve were detected. In addition, a protruded pannus throughout the area from the artificial valve ring to the middle of the valve cusp was also observed. Based on our experience, caution therefore needs to be exercised when using a bioprosthetic valve in a patient with PXE.