Aortic and Mitral Valve Replacement with Reconstruction of the Intervalvular Fibrous Body in Familial Hypercholesterolemia

Abstract

We report a case of a 22-year-old woman with a known history of homozygous familial hypercholesterolemia. The patient was admitted to our hospital with the chief complaints of chest discomfort and dyspnea on exertion. Preoperative echocardiography revealed highly echogenic aortic and mitral valves as well as a narrowed aortic root. A surgical procedure consisting of both aortic and mitral valve replacement with reconstruction of the intervalvular fibrous body was performed.

Key words

Heart valve surgery - heart disease - familial hypercholesterolemia

References

• 1 Rallidis L, Nihoyannopoulos P, Thompson G R et al. Aortic stenosis in homozygous familial hypercholesterolaemia.  Heart. 1996;  76 84-85
• 2 Rallidis L, Naoumova R P, Thompson G R et al. Extent and severity of atherosclerotic involvement of the aortic valve and root in familial hypercholesterolemia.  Heart. 1998;  80 583-590
• 3 Kawaguchi A, Miyatake K, Yutani C et al. Characteristic cardiovascular manifestation in homozygous and heterozygous familial hypercholesterolemia.  Am Heart J. 1999;  137 410-418
• 4 Yutani C, Go S, Imakita M et al. Autopsy findings in two patients with homozygous familial hypercholesterolemia: special references to apoprotein B localization and internalization defect of low density lipoprotein.  Acta Pathol Jpn. 1987;  39 1489-1504
• 5 David T E, Kuo J, Armstrong S. Aortic and mitral valve replacement with reconstruction of the intervalvular fibrous body.  J Thorac Cardiovasc Surg. 1997;  114 766-772

1 H. Jin and F. Yao contributed equally to this work.