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Standardized Technique of Aortic Valve Re-implantation for Valve-sparing Aortic Root Replacement
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Transcatheter Aortic Valve Replacement for Alkaptonuria-Associated Aortic Stenosis
Jacqueline K Olive1, Ahmed Alnajar2, Swaminadhan Gnanashanmugam2
1Division of Cardiothoracic Surgery, Department of Surgery, Baylor College of Medicine, Houston, Texas.
The Annals of Thoracic Surgery
|June 11, 2019
Summary
Alkaptonuria can cause aortic stenosis. Transfemoral transcatheter aortic valve replacement successfully treated a patient with this rare condition when surgery was not feasible.
Area of Science:
- Cardiology
- Metabolic Disorders
- Medical Case Reports
Background:
- Alkaptonuria is a rare inherited metabolic disorder.
- It involves homogentisic acid metabolism, leading to ochronosis and tissue deposition.
- Aortic valvular calcification and stenosis are known complications.
Observation:
- A 71-year-old woman presented with alkaptonuria-associated aortic stenosis.
- Minimally invasive surgical aortic valve replacement was attempted but could not be completed due to extensive aortic root calcification.
- The patient subsequently underwent transfemoral transcatheter aortic valve replacement.
Findings:
- Transfemoral transcatheter aortic valve replacement using an Edwards SAPIEN 3 valve was technically successful.
- This approach provided a viable treatment option for severe aortic stenosis in the context of alkaptonuria.
Implications:
- This case demonstrates the feasibility of transcatheter aortic valve replacement for alkaptonuria-associated aortic stenosis.
- It expands treatment options for patients with this rare condition and complex aortic root anatomy.
- Further research may explore long-term outcomes of TAVR in alkaptonuria patients.
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