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Congenital unicuspid aortic valve stenosis in siblings

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Abstract

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.

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References

  1. Mookadam F, Thota VR, Lopez AM, Emani UR. Unicuspid aortic valve in children: a systematic review spanning four decades. J Heart Valve Dis. 2010;69:678–83.

    Google Scholar 

  2. Williams C, Jong M. Frequency by decades of unicuspid, bicuspid and tricuspid aortic valves in adults having isolated aortic valve replacement for aortic stenosis, with or without associated aortic regurgitation. Circulation. 2005;111:920–5.

    Article  Google Scholar 

  3. Schoher HJ, Aicher D, Riodionycheva S, Lindinger A, Radle-Hurst T, Langer F, Abdul-Khaliq H. Bicuspidization of the unicuspid aortic valve: a new reconstructive approach. Ann Thorac Surg. 2008;85:2012–8.

    Article  Google Scholar 

  4. Hunchington K, Hunter AGW, Chan KL. A prospective study to assess the frequency of familial clustering of congenital bicuspid valve. J Am Coll Cardiol. 1997;30:1809–12.

    Article  Google Scholar 

  5. Glick BN, Roberts CW. Congenitally bicuspid aortic valve in multiple family members. Am J Cardiol. 2004;44:138–43.

    Article  Google Scholar 

  6. Novaro GM, Mishra M, Griffin BP. Incidence and echocardiographic features of congenital unicuspid valve in an adult population. J Heart Valve Dis. 2003;12:670–3.

    Google Scholar 

  7. Tempe DK, Garg M, Tomar AS, Dutta DD, Dutta R, Singh AH. Unicuspid aortic valve: transesophageal echocardiographic features. J Cardiothorac Vasc Anesth. 2012;26:277–9.

    Article  PubMed  Google Scholar 

  8. Osman K, Nanda NC, Kim K-S, et al. Transesophageal echocardiographic features of unicuspid aortic valve. Echocardiography. 1994;11:469–73.

    Article  Google Scholar 

  9. Cripe L, Andelfinger G, Martin IJ, Shooner K, Benson DW. Bicuspid aortic valve is heritable. Am J Cardiol. 2004;44:138–43.

    Article  Google Scholar 

Download references

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Authors and Affiliations

  1. Yamagata Prefectural Central Hospital, 1800 Aoyagi, Yamagata, Yamagata, Japan

    Yu Kawahara, Kotarou Suzuki, Shingo Takahara & Manabu Fukasawa

Authors
  1. Yu Kawahara
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  2. Kotarou Suzuki
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  3. Shingo Takahara
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  4. Manabu Fukasawa
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Correspondence to Yu Kawahara.

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Kawahara, Y., Suzuki, K., Takahara, S. et al. Congenital unicuspid aortic valve stenosis in siblings. Gen Thorac Cardiovasc Surg 62, 567–569 (2014). https://doi.org/10.1007/s11748-013-0270-0

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  • DOI: https://doi.org/10.1007/s11748-013-0270-0

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