Congenital unicuspid aortic valve stenosis in siblings.

We reported the case of unicuspid aortic valve in sibling, suggesting the familial incidence and genetic relation. A 41-year-old man (elder brother) with 79 mmHg of aortic valvular peak pressure gradient (peak PG) underwent aortic valve replacement with mechanical prosthetic valve (ATS 21 mm). The intraoperative finding showed the unicuspid aortic valve with one rudimentary commissure. A 37-year-old woman (sister) had been followed by echocardiography as bicuspid aortic valve since 10 years old. After 27 years, the peak PG had increased to 176 mmHg on preoperative echocardiography. The aortic valve replacement was performed. The bioprosthetic valve (MOSAIC 21 mm) was implanted supra-annularly because the patient required pregnancy. The intraoperative finding showed the unicuspid aortic valve with two rudimentary commissures and one opening of LCC-RCC commissure. The histopathological study of both patients confirmed as congenitally unicuspid aortic valve. In our best knowledge, this is a first report of unicuspid aortic valve in sibling.