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Transcatheter Aortic Valve Replacement for Alkaptonuria-Associated Aortic Stenosis.

08:00 EDT 7th June 2019 | BioPortfolio

Summary of "Transcatheter Aortic Valve Replacement for Alkaptonuria-Associated Aortic Stenosis."

Alkaptonuria, a rare disorder of homogentisic acid metabolism, can lead to aortic valvular calcification and stenosis. We present the case of a 71-year-old woman with alkaptonuria-associated aortic stenosis in whom a minimally invasive surgical aortic valve replacement was attempted but abandoned because of extensive root calcification. She subsequently underwent transfemoral transcatheter aortic valve replacement with an Edwards SAPIEN 3 valve (Edwards Lifesciences, Irvine, CA). To our knowledge, this is the first report of transcatheter aortic valve replacement for treating alkaptonuria-associated aortic stenosis, expanding potential treatment options for these patients.

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This article was published in the following journal.

Name: The Annals of thoracic surgery
ISSN: 1552-6259
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Medical and Biotech [MESH] Definitions

Surgical treatment for severe AORTIC VALVE STENOSIS. Transcatheter aortic valve replacement (TAVR) is used as an alternative option in patients who are deemed at high risk or inoperable for traditional open-heart surgery.

A pathological constriction that can occur above (supravalvular stenosis), below (subvalvular stenosis), or at the AORTIC VALVE. It is characterized by restricted outflow from the LEFT VENTRICLE into the AORTA.

A type of constriction that is caused by the presence of a fibrous ring (discrete type) below the AORTIC VALVE, anywhere between the aortic valve and the MITRAL VALVE. It is characterized by restricted outflow from the LEFT VENTRICLE into the AORTA.

The downward displacement of the cuspal or pointed end of the trileaflet AORTIC VALVE causing misalignment of the cusps. Severe valve distortion can cause leakage and allow the backflow of blood from the ASCENDING AORTA back into the LEFT VENTRICLE, leading to aortic regurgitation.

A pathological constriction occurring in the region below the AORTIC VALVE. It is characterized by restricted outflow from the LEFT VENTRICLE into the AORTA.

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