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http://dx.doi.org/10.1111/echo.15365DOI Listing

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A unicuspid aortic valve (UAV) in adults is a very rare form of aortic valve (AV) malformation. UAV has two distinct subtypes, acommissural UAV and unicommissural, and can be differentiated by anatomical features, imaging modalities, and clinical presentation. With the development of significant AV lesion (s), surgical or transcatheter intervention will be required.

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Unicuspid aortic valve repair in a neonate.

Multimed Man Cardiothorac Surg

October 2024

Department of Cardiothoracic Surgery, Royal Children's Hospital, Melbourne, Victoria, Australia.

The patient had a unicuspid aortic valve with severe aortic stenosis and a mildly dilated and hypertrophied left ventricle with moderately impaired systolic function. Herein we demonstrate the technique of severely dysplastic unicuspid aortic valve repair in the neonatal period.

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We describe a case of a 30-year-old woman with gradually increasing dyspnoea on exertion where CT angiography revealed a unicuspid unicommissural morphology of the aortic valve. The present report highlights the anatomical and embryological aspects of this rare anatomical variant as well as the associated cardiovascular abnormalities.

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Article Synopsis
  • Unicuspid aortic valve (UAV) is a rare heart condition commonly confused with the more common bicuspid aortic valve (BAV), affecting young adults primarily around 36 years old.
  • This study involved a systematic review and meta-analysis of 2953 studies, focusing on the demographic and clinical characteristics of UAV, which often includes associations with conditions like aortic coarctation and Turner syndrome.
  • Surgical intervention, primarily aortic valve replacement (AVR), showed high survival rates (95-98%) over ten years, highlighting the need for further research into UAV as a distinct condition apart from BAV.
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Article Synopsis
  • Bicuspidization is a surgical method for repairing a unicuspid aortic valve, emphasizing the need for symmetrical commissural orientation to enhance patient outcomes.* -
  • The presence of the right coronary orifice can complicate achieving this symmetry, as it often obstructs proper placement of the neocommissure.* -
  • In a pediatric case without aortic root dilation, surgeons rotated the right coronary orifice and created a neocommissure by manipulating the surrounding sinus walls, ensuring symmetry through specific measurements.*
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